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Hu R, Gui R, Nie X, Duan H. Case report: Clinical analysis and literature review of five cases of metastatic solid pseudopapillary tumor of the pancreas. Front Oncol 2024; 14:1386987. [PMID: 39450257 PMCID: PMC11499064 DOI: 10.3389/fonc.2024.1386987] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2024] [Accepted: 09/18/2024] [Indexed: 10/26/2024] Open
Abstract
Background Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare and low-grade malignant tumor. It mainly occurs in women of reproductive age, accounting for approximately 1-3% of all pancreatic tumors. SPN has a low incidence rate and is difficult to diagnose before surgery. Some cases may show local infiltration, but distant metastasis rarely occurs. Currently, there is no standardized treatment protocol for SPN. Patient and methods We have collected clinical data from 5 patients with solid pseudopapillary neoplasm (SPN) of the pancreas who presented with distant metastasis at our hospital. This study retrospectively analyzes their clinical manifestations, imaging characteristics, pathological findings, and treatment outcomes. The aim is to summarize the clinical features of SPN with distant metastasis, thereby improving the diagnosis, treatment, and prognosis prediction of this disease. This study also reviews relevant literature. Results The median age of the 5 patients was 32 years old, with a male-to-female ratio of 1:4. All patients underwent enhanced CT scans and were diagnosed with SPN through biopsy or surgical pathology. All 5 patients had liver metastases, and one patient had clavicular lymph node metastasis. Another patient had both lung and clavicular lymph node metastases. Three patients underwent curative surgery, one patient received chemotherapy combined with targeted immunotherapy and subsequently underwent TACE(Transcatheter arterial chemoembolization) and HAIC (Hepatic artery infusion chemotherapy) treatments due to progression. One patient received internal radiation therapy but experienced multiple relapses and eventually died due to complications. The follow-up period ranged from 7 to 53 months, with 2 patients succumbing to the disease. Conclusion As a low-grade tumor, SPN has a low rate of distant metastasis, typically occurring in only 5%-15% of cases. These metastases often lack characteristic clinical symptoms. Diagnosis can only be confirmed after exclusion of other lesions through imaging and pathological examination. The primary treatment for metastatic SPN is curative surgery, which can lead to a favorable prognosis.
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Affiliation(s)
- Run Hu
- Department of Oncology, Hunan Provincial People’s Hospital, The First Affiliated Hospital of Hunan Normal University, Hunan Normal University, Changsha, Hunan, China
- Key Laboratory of Study and Discovery of Small Targeted Molecules of Hunan Province, Hunan Normal University, Changsha, Hunan, China
| | - Renjie Gui
- Department of Oncology, Hunan Provincial People’s Hospital, The First Affiliated Hospital of Hunan Normal University, Hunan Normal University, Changsha, Hunan, China
- Key Laboratory of Study and Discovery of Small Targeted Molecules of Hunan Province, Hunan Normal University, Changsha, Hunan, China
| | - Xi Nie
- Department of Oncology, Hunan Provincial People’s Hospital, The First Affiliated Hospital of Hunan Normal University, Hunan Normal University, Changsha, Hunan, China
- Key Laboratory of Study and Discovery of Small Targeted Molecules of Hunan Province, Hunan Normal University, Changsha, Hunan, China
| | - Huaxin Duan
- Department of Oncology, Hunan Provincial People’s Hospital, The First Affiliated Hospital of Hunan Normal University, Hunan Normal University, Changsha, Hunan, China
- Key Laboratory of Study and Discovery of Small Targeted Molecules of Hunan Province, Hunan Normal University, Changsha, Hunan, China
- Hunan Provincial Clinical Medical Research Center for Hepatobiliary and Pancreatic Tumors, Changsha, Hunan, China
- Changsha City Comprehensive Diagnosis and Treatment Technology Innovation Center for Hepatobiliary Tumors, Changsha, Hunan, China
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Mazzarella G, Muttillo EM, Coletta D, Picardi B, Rossi S, Rossi Del Monte S, Gomes V, Muttillo IA. Solid pseudopapillary tumor of the pancreas: A systematic review of clinical, surgical and oncological characteristics of 1384 patients underwent pancreatic surgery. Hepatobiliary Pancreat Dis Int 2024; 23:331-338. [PMID: 37236826 DOI: 10.1016/j.hbpd.2023.05.004] [Citation(s) in RCA: 8] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2022] [Accepted: 05/09/2023] [Indexed: 05/28/2023]
Abstract
BACKGROUND Pancreatic solid pseudopapillary tumors (SPTs) are rare clinical entity, with low malignancy and still unclear pathogenesis. They account for less than 2% of exocrine pancreatic neoplasms. This study aimed to perform a systematic review of the main clinical, surgical and oncological characteristics of pancreatic SPTs. DATA SOURCES MEDLINE/PubMed, Web of Science and Scopus databases were systematically searched for the main clinical, surgical and oncological characteristics of pancreatic SPTs up to April 2021, in accordance with the preferred reporting items for systematic reviews and meta-analyses (PRISMA) standards. Primary endpoints were to analyze treatments and oncological outcomes. RESULTS A total of 823 studies were recorded, 86 studies underwent full-text reviews and 28 met inclusion criteria. Overall, 1384 patients underwent pancreatic surgery. Mean age was 30 years and 1181 patients (85.3%) were female. The most common clinical presentation was non-specific abdominal pain (52.6% of cases). Mean overall survival was 98.1%. Mean recurrence rate was 2.8%. Mean follow-up was 4.2 years. CONCLUSIONS Pancreatic SPTs are rare, and predominantly affect young women with unclear pathogenesis. Radical resection is the gold standard of treatment achieving good oncological impact and a favorable prognosis in a yearly life-long follow-up.
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Affiliation(s)
- Gennaro Mazzarella
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy; Department of Surgery "Pietro Valdoni", Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
| | - Edoardo Maria Muttillo
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy; Surgical and Medical Department of Translational Medicine, Sant'Andrea Hospital, Sapienza University of Rome, 00185 Rome, Italy
| | - Diego Coletta
- Department of Surgical Sciences, Umberto I University Hospital, Sapienza University of Rome, 00136 Rome, Italy; Department of General Surgery, Ospedali Riuniti Marche Nord, 61121 Pesaro, Italy
| | - Biagio Picardi
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy
| | - Stefano Rossi
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy
| | | | - Vito Gomes
- Department of Pathology, San Filippo Neri Hospital, 00136 Rome, Italy
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de Jesus VHF, Donadio MDS, de Brito ÂBC, Gentilli AC. A narrative review on rare types of pancreatic cancer: should they be treated as pancreatic ductal adenocarcinomas? Ther Adv Med Oncol 2024; 16:17588359241265213. [PMID: 39072242 PMCID: PMC11282540 DOI: 10.1177/17588359241265213] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2024] [Accepted: 06/13/2024] [Indexed: 07/30/2024] Open
Abstract
Pancreatic cancer is one of the deadliest malignancies in humans and it is expected to play a bigger part in cancer burden in the years to come. Pancreatic ductal adenocarcinoma (PDAC) represents 85% of all primary pancreatic malignancies. Recently, much attention has been given to PDAC, with significant advances in the understanding of the mechanisms underpinning disease initiation and progression, along with noticeable improvements in overall survival in both localized and metastatic settings. However, given their rarity, rare histological subtypes of pancreatic cancer have been underappreciated and are frequently treated as PDAC, even though they might present non-overlapping molecular alterations and clinical behavior. While some of these rare histological subtypes are true variants of PDAC that should be treated likewise, others represent separate clinicopathological entities, warranting a different therapeutic approach. In this review, we highlight clinical, pathological, and molecular aspects of rare histological types of pancreatic cancer, along with the currently available data to guide treatment decisions.
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Affiliation(s)
- Victor Hugo Fonseca de Jesus
- Oncoclínicas, Department of Gastrointestinal Medical Oncology, Santos Dumont St. 182, 4 floor, Florianópolis, Santa Catarina 88015-020, Brazil
- Department of Medical Oncology, Centro de Pesquisas Oncológicas, Florianópolis, Santa Catarina, Brazil
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Li X, Ren J, Ke J, Jiang P, Guo L, Zhang L, Han W, Liu Y, Ji B. Solid pseudopapillary neoplasm of the pancreas with hepatic metastases: problems and strategies. Front Oncol 2024; 14:1410888. [PMID: 39099687 PMCID: PMC11294175 DOI: 10.3389/fonc.2024.1410888] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2024] [Accepted: 07/05/2024] [Indexed: 08/06/2024] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasms of the pancreas with hepatic metastases are infrequent and difficult to diagnose, and treatment is uncertain. METHODS A retrospective analysis of clinical data from patients with pancreatic solid pseudopapillary neoplasm (SPN) hepatic metastases who underwent surgery at the First Hospital of Jilin University from January 2005 to December 2021 was conducted. A total of 287 patients with SPN were included in the study, of which 8 (3%) developed liver metastases, all of whom were treated surgically and recovered well after surgery. The clinical presentation, imaging features, surgical treatment, histopathological examination, and postoperative follow-up data (mean 70 months; range 28-138 months) of the patients were recorded and analyzed. Clinical response strategies can be derived by reviewing previous studies on hepatic metastases of SPNs. RESULTS For resectable hepatic metastases from pancreatic solid pseudopapillary neoplasms, early surgery with total resection of the primary tumor and metastasis has shown great efficiency and is associated with patient good prognosis. In patients presenting unresectable hepatic metastases, aggressive tumor reduction surgery resulted in the alleviation of clinical symptoms and reduction of tumor burden while potentially achieving long-term survival. CONCLUSION For hepatic metastases of SPNs, a preoperative liver tissue biopsy is beneficial for a definitive diagnosis. Surgery demonstrates excellent therapeutic efficacy and is considered the preferred curative treatment approach. This paper presents clinical experiences with SPN-related hepatic metastases at the Affiliated Hospital of Jilin University, which can be used to guide patient counseling in clinical practice.
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Affiliation(s)
- Xiaocheng Li
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, the First Hospital of Jilin University, Changchun, China
| | - Jiaxin Ren
- Department of Neurology, the First Hospital of Jilin University, Changchun, China
| | - Jianji Ke
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, the First Hospital of Jilin University, Changchun, China
| | - Peng Jiang
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, the First Hospital of Jilin University, Changchun, China
| | - Liang Guo
- Department of Pathology, the First Hospital of Jilin University, Changchun, China
| | - Li Zhang
- Department of Radiology, the First Hospital of Jilin University, Changchun, China
| | - Wei Han
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, the First Hospital of Jilin University, Changchun, China
| | - Yahui Liu
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, the First Hospital of Jilin University, Changchun, China
| | - Bai Ji
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, the First Hospital of Jilin University, Changchun, China
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Bauschke A, Altendorf-Hofmann A, Ali-Deeb A, Ardelt M, Dondorf F, Rauchfuss F, Rohland O, Tekbaș A, Settmacher U. Rare tumours of the pancreas: monocentric study. J Cancer Res Clin Oncol 2024; 150:349. [PMID: 39002034 PMCID: PMC11246325 DOI: 10.1007/s00432-024-05884-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2024] [Accepted: 07/07/2024] [Indexed: 07/15/2024]
Abstract
PURPOSE The biology of rare pancreatic tumours, which differs from that of ductal pancreatic cancer, requires increased attention. Although the majority of rare pancreatic tumours are benign, it is difficult to decide whether an invasive component exists without complete removal of the lesion, despite considerable progress in diagnosis. We are investigating a large cohort of patients with histologically confirmed epithelial non-ductal non-neuroendocrine neoplasms of the pancreas. METHODS Here we analyze long-term survival from patients, who underwent resection of histologically confirmed epithelial non-ductal non-neuroendocrine neoplasms of the pancreas. At our department between Jan 1st, 1999, and Dec 31st, 2019. The median follow-up was 61 (range 0-168) month. All statistical analyses were performed using SPSS 26.0 (IBM, Chicago, IL, USA) software. RESULTS 46 patients (48%) were followed up for more than 5 years, 18 patients (19%) for more than 10 years. The 5-year and 10-year survival rates for rare non-invasive pancreatic tumours were 72% and 55% respectively. The proportion of rare tumour entities (non-ductal and non-neuroendocrine) increased continuously and statistically significantly (p = 0.004) from 4.2 to 12.3% in our clinic between 1999 and 2019. If there is no invasive growth yet, there is a varying risk of malignant degeneration in the course of the disease. Therefore, the indication for pancreatic resection is still the subject of discussion. CONCLUSION The long-term prognosis of rare epithelial pancreatic tumours after R0 resection-even if they are already malignant-is much better than that of ductal pancreatic cancer.
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Affiliation(s)
- Astrid Bauschke
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany.
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany.
| | - Annelore Altendorf-Hofmann
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
| | - Aladdin Ali-Deeb
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany
| | - Michael Ardelt
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany
| | - Felix Dondorf
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany
| | - Falk Rauchfuss
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany
| | - Oliver Rohland
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany
| | - Aysun Tekbaș
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany
| | - Utz Settmacher
- Department of General, Visceral and Vascular Surgery, University Hospital Jena, Am Klinikum 1, 07740, Jena, Germany
- Comprehensive Cancer Center Central Germany (CCCG), 04103, Leipzig, Germany
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Codjia T, Marique L, Aussilhou B, Ftériche FS, de Mestier L, Rebours V, Cros J, Ruszniewski P, Lévy P, Lesurtel M, Sauvanet A, Dokmak S. Outcome and survival were similar with laparoscopic and open pancreatectomy in 102 solid pseudopapillary neoplasms. Surg Endosc 2024; 38:2169-2179. [PMID: 38448620 DOI: 10.1007/s00464-024-10708-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2023] [Accepted: 01/21/2024] [Indexed: 03/08/2024]
Abstract
BACKGROUND Solid pseudopapillary neoplasms of the pancreas (SPNP) are rare tumors predominantly in young women. We report the largest single-center cohort study comparing resection of SPNP by laparoscopic approach (LA) and the open approach (OA). METHOD Between 2001 and 2021, 102 patients (84% women, median age: 30) underwent pancreatectomy for SPNP and were retrospectively studied. Demographic, perioperative, pathological, early and the long-term results were evaluated between patients operated by LA and those by OA. RESULTS Population included 40 LA and 62 OA. There were no significant differences in demographics data between the groups. A preoperative biopsy by endoscopic ultrasound was performed in 45 patients (44%) with no difference between the groups. Pancreatoduodenectomy (PD) was less frequently performed by LA (25 vs 53%, p = 0.004) and distal pancreatectomy (DP) was more frequently performed by LA (40 vs 16%, p = 0.003). In the subgroup analysis by surgical procedure, LA-PD was associated with one mortality, less median blood loss (180 vs 200 ml, p = 0.034) and fewer harvested lymph nodes (11 vs 15, p = 0.02). LA-DP was associated with smaller median tumor size on imaging (40 vs 80mm, p = 0.048), shorter surgery (135 vs 190 min, p = 0.028), and fewer complications according to the median comprehensive complication index score (0 vs 8.7, p = 0.048). LA-Central pancreatectomy was associated with shorter surgery (160 vs 240, p = 0.037), less median blood loss (60 vs 200, p = 0.043), and less harvested lymph nodes (5 vs 2, p = 0.025). After a median follow-up of 60 months, two recurrences (2%) were observed and were unrelated to the approach. CONCLUSIONS The LA for SPNP appears to be safe, should be applied cautiously in case of PD for large lesion, and was not associated with recurrence.
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Affiliation(s)
- Tatiana Codjia
- AP-HP, Hôpital Beaujon, Department of HPB Surgery and Liver Transplantation, DMU DIGEST, University Paris Cité, Clichy, France
| | - Lancelot Marique
- AP-HP, Hôpital Beaujon, Department of HPB Surgery and Liver Transplantation, DMU DIGEST, University Paris Cité, Clichy, France
| | - Béatrice Aussilhou
- AP-HP, Hôpital Beaujon, Department of HPB Surgery and Liver Transplantation, DMU DIGEST, University Paris Cité, Clichy, France
| | - Fadhel Samir Ftériche
- AP-HP, Hôpital Beaujon, Department of HPB Surgery and Liver Transplantation, DMU DIGEST, University Paris Cité, Clichy, France
| | - Louis de Mestier
- AP-HP, Hôpital Beaujon, Department of Gastroenterology and Pancreatic Diseases, DMU DIGEST, University Paris Cité, Clichy, France
| | - Vinciane Rebours
- AP-HP, Hôpital Beaujon, Department of Gastroenterology and Pancreatic Diseases, DMU DIGEST, University Paris Cité, Clichy, France
| | - Jérome Cros
- AP-HP, Hôpital Beaujon, Department of Pathology, University Paris Cité, Clichy, France
| | - Philippe Ruszniewski
- AP-HP, Hôpital Beaujon, Department of Gastroenterology and Pancreatic Diseases, DMU DIGEST, University Paris Cité, Clichy, France
| | - Philippe Lévy
- AP-HP, Hôpital Beaujon, Department of Gastroenterology and Pancreatic Diseases, DMU DIGEST, University Paris Cité, Clichy, France
| | - Mickael Lesurtel
- AP-HP, Hôpital Beaujon, Department of HPB Surgery and Liver Transplantation, DMU DIGEST, University Paris Cité, Clichy, France
| | - Alain Sauvanet
- AP-HP, Hôpital Beaujon, Department of HPB Surgery and Liver Transplantation, DMU DIGEST, University Paris Cité, Clichy, France
| | - Safi Dokmak
- AP-HP, Hôpital Beaujon, Department of HPB Surgery and Liver Transplantation, DMU DIGEST, University Paris Cité, Clichy, France.
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Honda S, Yamaguchi H, Aimono E, Hara S, Minamiguchi S, Norose T, Ohike N, Yamochi T, Yasuda M, Moriya T, Shiko Y, Nishihara H, Nagao T. High-grade Solid Pseudopapillary Neoplasms of the Pancreas: Distinct Clinicopathological Malignant Features With Intriguing Gene Alterations through a Comparison With the Conventional Type. Am J Surg Pathol 2024; 48:353-363. [PMID: 38189381 DOI: 10.1097/pas.0000000000002177] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2024]
Abstract
Pancreatic solid pseudopapillary neoplasm (SPN) is a low-grade malignant neoplasm with a good prognosis. Clinically aggressive SPNs have rarely been reported but have not been analyzed in detail. In this study, we referred to this highly malignant type of SPN as high-grade SPN (HG-SPN) and compared its clinicopathological and genetic characteristics with conventional SPN (C-SPN) using immunohistochemistry and gene panel analyses. Five HG-SPNs and 15 C-SPNs were evaluated in this study. HG-SPNs share many pathologic characteristics: macroscopically, solid/cystic appearances, microscopically, pseudopapillary/pseudorosette pattern (100%), tumor cell loose cohesiveness (100%), thin/delicate vasculature (100%), tumor cell cytoplasmic vacuolization (100%), immunohistochemical positivity for β-catenin (nuclear expression) (100%), CD10 (80%), CD56 (80%), and vimentin (100%). Conversely, HG-SPNs showed distinct malignant features compared with C-SPNs: mean tumor size (11.7 vs. 2.9 cm, P <0.001); true necrosis (100% vs. 0%, P <0.001); high-grade nuclear atypia (100% vs. 0%, P <0.001); lymphatic and/or venous invasion (100% vs. 20%, P =0.004); mean mitotic count (4.38 vs. 0.05/high-power field, P <0.001); and mean Ki-67 labeling index (33.9% vs. 3.4%, P <0.001). All HG-SPN patients died of primary disease 3 to 36 months after surgery, while all C-SPN patients were alive without disease. Genetic studies have shown that all analyzed HG-SPNs have CTNNB1 mutations. Two HG-SPN cases showed RB1 mutations with altered immunohistochemical findings for RB1 and p16. Two HG-SPN cases had TP53 mutation and/or p53 overexpression. In conclusion, HG-SPNs show distinct malignant features and some genetic alterations that differ from C-SPNs, indicating the importance of differentiating between these 2 subtypes.
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Affiliation(s)
- Shogo Honda
- Department of Anatomic Pathology, Tokyo Medical University
- Department of Pathology, NHO Sagamihara National Hospital
| | - Hiroshi Yamaguchi
- Department of Anatomic Pathology, Tokyo Medical University
- Department of Pathology, Saitama Medical University
| | - Eriko Aimono
- Genomics Unit, Keio Cancer Center, Keio University School of Medicine
| | - Shigeo Hara
- Department of Diagnostic Pathology, Kobe City Medical Center General Hospital, Kobe
| | | | - Tomoko Norose
- Department of Pathology, Showa University School of Medicine, Tokyo
- Department of Pathology, Division of Molecular Pathology, St. Marianna University School of Medicine, Kanagawa
| | - Nobuyuki Ohike
- Department of Pathology, Showa University School of Medicine, Tokyo
- Department of Pathology, Division of Molecular Pathology, St. Marianna University School of Medicine, Kanagawa
| | - Toshiko Yamochi
- Department of Pathology, Showa University School of Medicine, Tokyo
| | - Masanori Yasuda
- Department of Pathology, Saitama Medical University, International Medical Center, Saitama
| | - Takuya Moriya
- Department of Pathology, Kawasaki Medical School, Okayama
| | - Yuki Shiko
- Biostatistics Section, Clinical Research Center, Chiba University Hospital, Chiba, Japan
| | - Hiroshi Nishihara
- Genomics Unit, Keio Cancer Center, Keio University School of Medicine
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ALGhabra Y, Hamdi M, Alhomsi M, Alusef A, Qatleesh S, Ousta MA. Solid pseudopapillary neoplasm of the pancreas: A case report with a brief literature review. Int J Surg Case Rep 2024; 115:109234. [PMID: 38245948 PMCID: PMC10831808 DOI: 10.1016/j.ijscr.2024.109234] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2023] [Revised: 01/03/2024] [Accepted: 01/05/2024] [Indexed: 01/23/2024] Open
Abstract
INTRODUCTION Solid pseudopapillary neoplasm (SPN) of the pancreas, representing only 1 % of pancreatic cancers, was identified by Virginia Frantz in 1959. Predominantly affecting young females, it often remains asymptomatic, posing diagnostic challenges due to slow growth. This paper emphasizes SPN's rarity and associated diagnostic complexities. CASE PRESENTATION In a specific case, a 17-year-old female with post-traumatic right flank pain underwent an enhanced CT scan, revealing a well-defined, hypodense mass in the pancreatic head. With normal laboratory results, a diagnostic laparotomy exposed a sizable solid cystic mass. A Whipple procedure unveiled a predominantly cystic mass enveloped by a well-developed capsule. DISCUSSION SPN appears as a distinct mixed solid and cystic lesion on imaging, necessitating confirmation through core biopsy. Surgical resection, the primary treatment, ensures a positive overall prognosis, despite rare recurrence and metastases. Microscopic examination reveals pseudopapillae, and immunohistochemistry aids diagnosis with positive staining for estrogen receptor, progesterone receptor, CD10, and CD99. CONCLUSION SPN, a rare pancreatic neoplasm predominantly affecting young females, may present with abdominal pain or palpable mass despite its usual asymptomatic nature. Diagnosis involves imaging and biopsy confirmation, with surgical resection as the curative treatment. While prognosis is generally favorable, comprehensive understanding and improved management require further research for this uncommon pancreatic neoplasm.
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Affiliation(s)
- Yasser ALGhabra
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Mohammad Hamdi
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria; M.D, Faculty of Medicine, Aleppo University, Aleppo, Syria.
| | - Malath Alhomsi
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Ahmad Alusef
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria; M.D, Faculty of Medicine, Aleppo University, Aleppo, Syria
| | - Safaa Qatleesh
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Mhd Ali Ousta
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
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Abi-Saab T, Cunningham AM, Rush PS, Matkowskyj KA. Pathologic Features of Primary Pancreatic Malignancies. Cancer Treat Res 2024; 192:89-117. [PMID: 39212917 DOI: 10.1007/978-3-031-61238-1_5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/04/2024]
Abstract
This chapter explores the pathologic features of benign and malignant lesions of the pancreas. As pathologic classifications evolve particularly for cystic lesions and neuroendocrine tumors, it is important for physicians who treat patients with gastrointestinal malignance to fully evaluate these pathologic classifications.
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Affiliation(s)
- Tarek Abi-Saab
- School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI, USA
| | | | - Patrick S Rush
- Virginia Tech Carilion School of Medicine, Roanoke, VA, USA
- Dominion Pathology Associates, Roanoke, VA, USA
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Chen J, Zong L, Wang P, Liu Y, Zhang H, Chang X, Lu Z, Li W, Ma Y, Yu S, Chen J. Solid Pseudopapillary Neoplasms of the Pancreas: Clinicopathologic Analysis and a Predictive Model. Mod Pathol 2023; 36:100141. [PMID: 36813115 DOI: 10.1016/j.modpat.2023.100141] [Citation(s) in RCA: 15] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2022] [Revised: 01/27/2023] [Accepted: 02/14/2023] [Indexed: 02/22/2023]
Abstract
Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare. They are considered low-grade malignancies, and a small percentage of patients experience recurrence or metastasis. It is critical to investigate associated biological behavior and identify patients at a risk of relapse. This was a retrospective study of 486 patients with SPNs who were diagnosed between 2000 and 2021. Their clinicopathologic features, including 23 parameters and prognoses were analyzed. Six patients (1.2%) presented with synchronous liver metastasis. A total of 21 patients experienced recurrence or metastasis postoperatively. The overall and disease-specific survival rates were 99.8% and 100%, respectively. The 5- and 10-year relapse-free survival (RFS) rates were 97.4% and 90.2%, respectively. Tumor size, lymphovascular invasion, and the Ki-67 index were independent predictors of relapse. Furthermore, a Peking Union Medical College Hospital-SPN risk model was built to evaluate the risk of relapse and compared it with the American Joint Committee on Cancer tumor staging system (eighth edition, 2017). Risk factors included 3 parameters: tumor size (>9 cm), lymphovascular invasion status (presence), and Ki-67 index (>1%). Risk grades were available for 345 patients, who were divided into 2 groups: (1) low risk (n = 124) and (2) high risk (n = 221). The group with no risk factors was designated as low risk and had a 10-year RFS of 100%. The group associated with 1 to 3 factors was designated as high risk, with a 10-year RFS of 75.3%. Receiver operating characteristic curves were generated, and the area under the curve was 0.791 for our model and 0.630 for the American Joint Committee on Cancer with respect to the cancer staging system. We validated our model in independent cohorts and demonstrated a sensitivity of 98.3%. In conclusion, SPNs are low-grade malignant neoplasms that rarely metastasize, and the 3 selected pathologic parameters can be used to predict their behavior. A novel Peking Union Medical College Hospital-SPN risk model was proposed for routine application to guide the patient counseling in clinical practice.
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Affiliation(s)
- Jingci Chen
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Liju Zong
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Pengyan Wang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yilin Liu
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Hongkai Zhang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiaoyan Chang
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Zhaohui Lu
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Wencai Li
- Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yihui Ma
- Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Shuangni Yu
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
| | - Jie Chen
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
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11
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Pawlak KM, Tehami N, Maher B, Asif S, Rawal KK, Balaban DV, Tag-Adeen M, Ghalim F, Abbas WA, Ghoneem E, Ragab K, El-Ansary M, Kadir S, Amin S, Siau K, Wiechowska-Kozlowska A, Mönkemüller K, Abdelfatah D, Abdellatef A, Lakhtakia S, Okasha HH. Role of endoscopic ultrasound in the characterization of solid pseudopapillary neoplasm of the pancreas. World J Gastrointest Endosc 2023; 15:273-284. [PMID: 37138939 PMCID: PMC10150282 DOI: 10.4253/wjge.v15.i4.273] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/22/2022] [Revised: 02/17/2023] [Accepted: 03/29/2023] [Indexed: 04/14/2023] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasm (SPN) is an uncommon pathology of the pancreas with unpredictable malignant potential. Endoscopic ultrasound (EUS) assessment plays a vital role in lesion characterization and confirmation of the tissue diagnosis. However, there is a paucity of data regarding the imaging assessment of these lesions. AIM To determine the characteristic EUS features of SPN and define its role in preoperative assessment. METHODS This was an international, multicenter, retrospective, observational study of prospective cohorts from 7 large hepatopancreaticobiliary centers. All cases with postoperative histology of SPN were included in the study. Data collected included clinical, biochemical, histological and EUS characteristics. RESULTS One hundred and six patients with the diagnosis of SPN were included. The mean age was 26 years (range 9 to 70 years), with female predominance (89.6%). The most frequent clinical presentation was abdominal pain (80/106; 75.5%). The mean diameter of the lesion was 53.7 mm (range 15 to 130 mm), with the slight predominant location in the head of the pancreas (44/106; 41.5%). The majority of lesions presented with solid imaging features (59/106; 55.7%) although 33.0% (35/106) had mixed solid/cystic characteristics and 11.3% (12/106) had cystic morphology. Calcification was observed in only 4 (3.8%) cases. Main pancreatic duct dilation was uncommon, evident in only 2 cases (1.9%), whilst common bile duct dilation was observed in 5 (11.3%) cases. One patient demonstrated a double duct sign at presentation. Elastography and Doppler evaluation demonstrated inconsistent appearances with no emergence of a predictable pattern. EUS guided biopsy was performed using three different types of needles: Fine needle aspiration (67/106; 63.2%), fine needle biopsy (37/106; 34.9%), and Sonar Trucut (2/106; 1.9%). The diagnosis was conclusive in 103 (97.2%) cases. Ninety-seven patients were treated surgically (91.5%) and the post-surgical SPN diagnosis was confirmed in all cases. During the 2-year follow-up period, no recurrence was observed. CONCLUSION SPN presented primarily as a solid lesion on endosonographic assessment. The lesion tended to be located in the head or body of the pancreas. There was no consistent characteristic pattern apparent on either elastography or Doppler assessment. Similarly SPN did not frequently cause stricture of the pancreatic duct or common bile duct. Importantly, we confirmed that EUS-guided biopsy was an efficient and safe diagnostic tool. The needle type used does not appear to have a significant impact on the diagnostic yield. Overall SPN remains a challenging diagnosis based on EUS imaging with no pathognomonic features. EUS guided biopsy remains the gold standard in establishing the diagnosis.
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Affiliation(s)
- Katarzyna M Pawlak
- Endoscopy Unit, Department of Gastroenterology, Hospital of The Ministry of Interior and Administration, Szczecin 01-218, Poland
| | - Nadeem Tehami
- Interventional Endoscopy Unit, University Hospital Southampton NHS Foundation Trust, 3AX PO, United Kingdom
| | - Ben Maher
- Interventional Endoscopy Unit, University Hospital Southampton NHS Foundation Trust, 3AX PO, United Kingdom
| | - Shujaath Asif
- Consultant Gastroenterologist, AIG Hospitals, Hyderabad 500032, India
| | - Krishn Kant Rawal
- Department of Gastroenterology, Prime Institute of Digestive Sciences, Rajkot 360001, India
| | - Daniel Vasile Balaban
- Department of Gastroenterology, Central Military Emergency University Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest 010011, Romania
| | - Mohammed Tag-Adeen
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Qena Faculty of Medicine, South Valley University, Qena 83511, Egypt
- Department of Endoscopy, Shefa Al-Orman Hospital, Luxor 85951, Egypt
| | - Fahd Ghalim
- Interventional Endoscopy Unit, Sainte Marie Hospital, OSNY 95520, France
| | - Wael A Abbas
- Department of Internal Medicine, Gastroenterology Unit, Faculty of Medicine, Assuit University, Assuit 71511, Egypt
| | - Elsayed Ghoneem
- Department of Gastroenterology and Hepatology, Faculty of Medicine, Mansoura University, Mansoura 35511, Egypt
| | - Khaled Ragab
- Department of Hepatology and Gastroenterology, Theodor Bilharz Research Institute, Giza 12511, Egypt
| | - Mahmoud El-Ansary
- Department of Hepatology & Gastroenterology, Theodor Bilharz Research Institute, Giza 12511, Egypt
| | - Shanil Kadir
- Department of Gastroenterology, Liaquat National Hospital & Medical College, Karachi 75900, Pakistan
| | - Sunil Amin
- Division of Digestive and Liver Diseases, Department of Medicine, University of Miami Miller School of Medicine, Miami, FL 32118, United States
| | - Keith Siau
- Department of Gastroenterology, Royal Cornwall Hospitals NHS Trust, Truro, Cornwall, United Kingdom
| | | | - Klaus Mönkemüller
- Department of Gastroenterology, Virginia Tech Carilion School of Medicine, Roanoke, Virginia, AL 35294, United States
| | - Dalia Abdelfatah
- Department of Cancer Epidemiology and Biostatistics, National Cancer Institute, Cairo University, Cairo 11511, Egypt
| | - Abeer Abdellatef
- Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kasr Al-Aini School of Medicine, Faculty of Medicine, Cairo University, Cairo 11511, Egypt
| | - Sundeep Lakhtakia
- Consultant Gastroenterologist, AIG Hospitals, Hyderabad 500032, India
| | - Hussein Hassan Okasha
- Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kasr Al-Aini School of Medicine, Faculty of Medicine, Cairo University, Cairo 11451, Egypt
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12
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Ogawa D, Okabe H, Masuda T, Sugita H. Long-term survivor of giant pancreatic solid pseudopapillary neoplasm with splenic infiltration and lymph node metastasis. BMJ Case Rep 2023; 16:e253554. [PMID: 36731944 PMCID: PMC9896223 DOI: 10.1136/bcr-2022-253554] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/21/2022] [Indexed: 02/04/2023] Open
Abstract
A man in his 70s with a 10 cm abdominal mass in the tail of the pancreas was diagnosed with pancreatic tail cancer. Distal pancreatectomy with curative intent was performed. Since tumour invasion of the spleen and transverse colon was suspected, pancreatectomy with splenectomy, left adrenalectomy and partial transverse colectomy was performed. Pathological examination of the resected specimen showed a giant pancreatic tumour, and a diagnosis of locally invasive solid pseudopapillary neoplasm (SPN) of the pancreas was made. The patient achieved 8-year survival without any recurrences. We herein report a very rare case of a giant pancreatic SPN with splenic infiltration and lymph node metastasis that was cured by resection.
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Affiliation(s)
- Daisuke Ogawa
- Department of Surgery, Kumamoto Regional Medical Center, Kumamoto, Japan
| | - Hirohisa Okabe
- Department of Surgery, Kumamoto Regional Medical Center, Kumamoto, Japan
| | - Toshiro Masuda
- Department of Surgery, Kumamoto Regional Medical Center, Kumamoto, Japan
| | - Hiroki Sugita
- Department of Surgery, Kumamoto Regional Medical Center, Kumamoto, Japan
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13
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Sun G, Fang K, Fu X, Peng L, Shu J, Tu Y, Li Y, Xiao W. Solid Pseudopapillary Neoplasm of the Pancreas: A Multi-Institution Study of 118 Cases. Pancreas 2023; 52:e121-e126. [PMID: 37523603 DOI: 10.1097/mpa.0000000000002219] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 08/02/2023]
Abstract
OBJECTIVES The aim of the study is to summary the clinicopathological characteristics and surgical outcomes of solid pseudopapillary neoplasm (SPN) of the pancreas. METHODS In this retrospective study, the information of 118 patients with SPN from 3 hospitals were analyzed. RESULTS A total of 118 patients. The mean age was 30.8 (standard deviation, 14.7) years and the majority were female (n = 95, 80.5%). Sixty-seven patients (56.8%) had clinical symptoms, of which the most common symptom was abdominal pain (49.6%). The mean tumor size was 5.9 (standard deviation, 2.9) cm. Pseudopapillary architecture was the commonest histologic feature, and β-catenin, CD56, vimentin, neuron-specific enolase, CD10, a1-antitrypsin, cytokeratins showed different degrees of positive expression in immunohistochemical staining. Fourteen patients (11.9%) presented aggressive pathologic behavior, which was correlated to the incomplete tumor capsule. At a median follow-up of 59.2 months, the recurrence rate was 1.8% and the overall 5-year survival rate was 97.7%. CONCLUSIONS Solid pseudopapillary neoplasm of the pancreas is a potentially low-grade malignant tumor that most frequently found in young females. Its clinical manifestations are nonspecific and the diagnosis mostly depends on pathological examination. Surgical resection is the first choice of treatment for SPN with a good prognosis.
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Affiliation(s)
- Gen Sun
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Kang Fang
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Xiaowei Fu
- Department of General Surgery, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Long Peng
- Department of General Surgery, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Jiaming Shu
- Department of Oncology, Jiangxi Provincial People's Hospital, Nanchang, China
| | - Yi Tu
- Department of Pathology, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Yong Li
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Weidong Xiao
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
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14
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Shang Y, Zhang Y, Pan E, Yang P, Xu L, Sun J. A refractory liver metastatic solid pseudopapillary neoplasm pancreas harbored CTNNB1 mutation showed good response to celecoxib: A case report. Front Oncol 2022; 12:1022290. [PMID: 36387184 PMCID: PMC9650638 DOI: 10.3389/fonc.2022.1022290] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2022] [Accepted: 10/11/2022] [Indexed: 01/11/2025] Open
Abstract
Solid pseudopapillary neoplasm (SPN) of the pancreas is rare relatively low-grade malignant neoplasm and metastasis rarely. Surgical resection is the primary treatment option for primary and metastatic lesions of SPN, and chemotherapy is often ineffective in non-operable SPNs. SPNs are characterized by the presence of somatic CTNNB1 exon 3 mutations, leading to the activation of Wnt/β-catenin/Cox-2 signal pathway. Here, we firstly report that a refractory liver metastatic pancreatic SPN patient after the failure of multi-line chemotherapies benefited from the Cox-2 selective inhibitor (Celecoxib) based on CTNNB1 D32V mutation detected by next-generation sequencing (NGS), achieving a more than 22-month progression-free survival without any adverse events. Our case provides a potential treatment option for liver metastatic SPN patients with CTNNB1 mutations and highlights the application of NGS for the better treatment decision making.
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Affiliation(s)
- Yu Shang
- Department of Medical Oncology, The Second Hospital of Dalian Medical University, Dalian, Liaoning, China
| | - Yunkun Zhang
- Department of Medical Oncology, The Second Hospital of Dalian Medical University, Dalian, Liaoning, China
| | - Evenki Pan
- Department of Medical, Nanjing Geneseeq Technology Inc., Nanjing, Jiangsu, China
| | - Peng Yang
- Department of Medical, Nanjing Geneseeq Technology Inc., Nanjing, Jiangsu, China
| | - Lingling Xu
- Department of Medical Oncology, The Second Hospital of Dalian Medical University, Dalian, Liaoning, China
| | - Jinghua Sun
- Department of Medical Oncology, The Second Hospital of Dalian Medical University, Dalian, Liaoning, China
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15
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Tasar P, Kilicturgay SA. Solid pseudopapillary neoplasms of the pancreas: Is there a factor determining the prognosis? Experience of a single institution. Medicine (Baltimore) 2022; 101:e30101. [PMID: 36042645 PMCID: PMC9410631 DOI: 10.1097/md.0000000000030101] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/03/2022] Open
Abstract
Solid pseudopapillary neoplasms (SPNs) are frequently seen in young women. Although the behavior pattern varies, these rare lesions generally have a low malignant potential. In this study, the aim was to investigate the effect of clinicopathological features of lesions on the recurrence in and survival of patients. In this study, patients of our clinic who were pathologically diagnosed with SPN after pancreatic surgery between July 2008 and December 2020 were evaluated retrospectively. Patients' age, gender, comorbidities, symptoms at the time of application, preoperative CA 19-9, CEA value, preoperative cross-sectional diagnostic imaging method and lesion characteristics, surgery, postoperative complications, length of hospital stay, and histopathological features were evaluated. Early and late mortality, overall survival, disease-free survival, and recurrence rate were determined. Four of the 23 patients diagnosed with SPN were male and the median age was 29 (23-47) years. Of the study patients, 69.56% experienced pain symptoms and 30.43% were asymptomatic. The median tumor size was 4 cm (1.5-15). The most common surgical procedure was distal pancreatectomy (56.5%). The median length of hospital stays was 5 (3-120) days and morbidity was observed in 9 cases (39.13%). The mortality rate was 4.35%. The mean follow-up period in the series was 53 (8-132) months and none of the patients developed recurrence. In this study, no significant difference was found regarding recurrence in patients with SPN with histopathologically aggressive biological behavior. The overall survival rate was 95.7%. SPNs are rare lesions with low malignant potential. SPNs are associated with longer-term survival after surgical resection.
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Affiliation(s)
- Pinar Tasar
- Department of General Surgery, School of Medicine, Uludag University, Gorukle, Bursa, Turkey
| | - Sadik Ayhan Kilicturgay
- Department of General Surgery, School of Medicine, Uludag University, Gorukle, Bursa, Turkey
- *Correspondence: Sadik Ayhan Kilicturgay, Department of General Surgery, Uludag University, Gorukle, Bursa/Turkey (e-mail: )
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16
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Mattiolo P, Mafficini A, Lawlor RT, Marchegiani G, Malleo G, Pea A, Salvia R, Piccoli P, Sciammarella C, Santonicco N, Parisi A, Silvestris N, Milella M, Adsay V, Scarpa A, Luchini C. "Pure" hepatoid tumors of the pancreas harboring CTNNB1 somatic mutations: a new entity among solid pseudopapillary neoplasms. Virchows Arch 2022; 481:41-47. [PMID: 35359182 PMCID: PMC9226109 DOI: 10.1007/s00428-022-03317-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2021] [Revised: 03/04/2022] [Accepted: 03/26/2022] [Indexed: 02/06/2023]
Abstract
Hepatoid tumors (HTs) represent a rare group of neoplasms that are histologically similar to hepatocellular carcinoma but arise outside the liver. The current World Health Organization classification recognizes the hepatoid morphology of pancreatic tumors only as a possible variant of pancreatic ductal adenocarcinoma (PDAC). Here, we describe two cases of "pure" HT of the pancreas showing common features and characterized by indolent biological behavior. These tumors were roundish nodules with pushing borders, hyaline globules, and pure hepatoid histology; they were diffusely positive for β-catenin and LEF1 on immunohistochemistry. At next-generation sequencing, both neoplasms harbored only one pathogenic somatic mutation that affected the CTNNB1 gene at exon 3 and showed a loss of heterozygosity on chromosomes 18 and 21. By integrating macroscopic and microscopic features, along with their molecular profiles, we advocate that such tumors represent a distinct entity from PDAC and should be considered a new variant of solid pseudopapillary neoplasms. The recognition of this new neoplastic category may have immediate implications not only for tumor taxonomy but also for clinical practice.
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Affiliation(s)
- Paola Mattiolo
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Piazzale Scuro, 10, 37134, Verona, Italy
| | - Andrea Mafficini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Piazzale Scuro, 10, 37134, Verona, Italy
| | - Rita T Lawlor
- ARC-Net Research Center for Applied Research On Cancer, University of Verona, 37134, Verona, Italy
| | - Giovanni Marchegiani
- Department of Surgery, The Pancreas Institute, University and Hospital Trust of Verona, 37134, Verona, Italy
| | - Giuseppe Malleo
- Department of Surgery, The Pancreas Institute, University and Hospital Trust of Verona, 37134, Verona, Italy
| | - Antonio Pea
- Department of Surgery, The Pancreas Institute, University and Hospital Trust of Verona, 37134, Verona, Italy
| | - Roberto Salvia
- Department of Surgery, The Pancreas Institute, University and Hospital Trust of Verona, 37134, Verona, Italy
| | - Paola Piccoli
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Piazzale Scuro, 10, 37134, Verona, Italy
| | - Concetta Sciammarella
- ARC-Net Research Center for Applied Research On Cancer, University of Verona, 37134, Verona, Italy
| | - Nicola Santonicco
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Piazzale Scuro, 10, 37134, Verona, Italy
| | - Alice Parisi
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Piazzale Scuro, 10, 37134, Verona, Italy
| | - Nicola Silvestris
- Medical Oncology Unit, Department of Human Pathology "G. Barresi", University of Messina, 98125, Messina, Italy
| | - Michele Milella
- Department of Medicine, Section of Medical Oncology, University and Hospital Trust of Verona, 37134, Verona, Italy
| | - Volkan Adsay
- Department of Pathology, Koç University Hospital and Koç University Research Center for Translational Medicine (KUTTAM), 34010, Istanbul, Turkey
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Piazzale Scuro, 10, 37134, Verona, Italy
- ARC-Net Research Center for Applied Research On Cancer, University of Verona, 37134, Verona, Italy
| | - Claudio Luchini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Piazzale Scuro, 10, 37134, Verona, Italy.
- ARC-Net Research Center for Applied Research On Cancer, University of Verona, 37134, Verona, Italy.
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17
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Cabrit N, Labiad C, Aussilhou B, Sartoris R, Sauvanet A, Dokmak S. Laparoscopic Pancreatoduodenectomy with Resection of the Inferior Vena Cava and Reconstruction with a Peritoneal Patch. Ann Surg Oncol 2022; 29:10.1245/s10434-022-11550-z. [PMID: 35325375 DOI: 10.1245/s10434-022-11550-z] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2021] [Accepted: 02/21/2022] [Indexed: 11/18/2022]
Abstract
BACKGROUND Laparoscopic resection of the inferior vena cava (IVC) during laparoscopic pancreatoduodenectomy (LPD) has never been described. A 32-year-old male with large solid pseudopapillary neoplasm underwent LPD with resection of the IVC and reconstruction by a peritoneal patch (PP). METHODS In this indication, the dissection is achieved by resection of the IVC. Kocher maneuver is difficult owing to the caval invasion, and section of the retroportal lamina tissue, before Kocher maneuver, is needed to control the left side of the IVC. Extended lymphadenectomy is not needed because the risk of lymph node invasion is low, and venous resection may be required for severe tumor adhesions without necessary histological invasion, to avoid tumor rupture at high risk of recurrence.1,2 The IVC was clamped by a laparoscopic vascular clamp and reconstructed (5-6 cm) with a PP. RESULTS The operative duration was 430 min, including IVC clamping for 27 min. The outcome was marked by biliary fistula and 24 days of hospital stay. Histology showed 6 cm tumor without histological invasion of the IVC wall. After 15 months of follow-up, there was no recurrence and no stenosis of the IVC. In our experience, reconstruction of the IVC with a PP is a safe procedure, with no PP-related complications and high patency rate (> 90%).3 CONCLUSION: Laparoscopic resection of the IVC is feasible in highly selected centers. The harvesting of the PP is easier than that of other autologous venous grafts, especially when done by the laparoscopic approach.
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Affiliation(s)
- Nicolas Cabrit
- Department of HPB Surgery and Liver Transplantation, DMU DIGEST, AP-HP, Hôpital Beaujon, Clichy, France
| | - Camélia Labiad
- Department of HPB Surgery and Liver Transplantation, DMU DIGEST, AP-HP, Hôpital Beaujon, Clichy, France
| | - Béatrice Aussilhou
- Department of HPB Surgery and Liver Transplantation, DMU DIGEST, AP-HP, Hôpital Beaujon, Clichy, France
| | | | - Alain Sauvanet
- Department of HPB Surgery and Liver Transplantation, DMU DIGEST, AP-HP, Hôpital Beaujon, Clichy, France
| | - Safi Dokmak
- Department of HPB Surgery and Liver Transplantation, DMU DIGEST, AP-HP, Hôpital Beaujon, Clichy, France.
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18
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Singhal S, Vashistha N, Singhal D. Solid Pseudopapillary Tumor of Pancreas with Sinistral Portal Hypertension: A Rare Presentation. J Gastrointest Cancer 2022; 54:297-299. [PMID: 35211852 DOI: 10.1007/s12029-022-00814-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/16/2022] [Indexed: 10/19/2022]
Abstract
An elderly male presented with complaint of dull aching pain in left upper abdomen of 2 months' duration. He was well built, and on abdominal examination, a large lump was palpable in left lumbar region. His biochemical, hematological parameters and tumor markers including CA 19.9 were within prescribed normal limits. Contrast enhanced computed tomography (CT) of the abdomen revealed large, well-defined, mixed solid, and cystic lesion arising from distal body-tail of the pancreas extending up to splenic hilum. Chronic thrombosis involving retro pancreatic splenic vein with multiple perisplenic, peripancreatic, and perigastric collaterals were noted. Based on characteristic CT abdomen findings, a diagnosis of solid pseudopapillary tumor (SPT) of the pancreas with sinistral portal hypertension (SPH) was made. The patient was planned for open distal pancreatectomy with splenectomy. At surgery, splenic flexure of colon was densely adhered to the tumor, and hence en bloc resection of colon was also performed. Postoperative period was uneventful, and he was discharged from the hospital on postoperative day 6. Histopathology reported solid pseudopapillary tumor of the pancreas, 22 cm in the largest dimension. SPT of the pancreas rarely present with SPH. At 22-cm size, this may be one of the largest SPTs reported in the English literature to date.
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Affiliation(s)
- Siddharth Singhal
- Department of Surgical Gastroenterology, Max Superspeciality Hospital, Saket, New Delhi, India
| | - Nitin Vashistha
- Department of Surgical Gastroenterology, Max Superspeciality Hospital, Saket, New Delhi, India
| | - Dinesh Singhal
- Department of Surgical Gastroenterology, Max Superspeciality Hospital, Saket, New Delhi, India.
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19
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Application of MSCT characteristic nomogram model in predicting invasion of pancreatic solid pseudopapillary neoplasms. Eur J Radiol 2022; 149:110201. [PMID: 35180581 DOI: 10.1016/j.ejrad.2022.110201] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/05/2021] [Revised: 01/24/2022] [Accepted: 02/07/2022] [Indexed: 12/12/2022]
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Sibio S, Di Carlo S. Current highlights on solid pseudopapillary neoplasm of the pancreas. World J Hepatol 2022; 14:300-303. [PMID: 35126857 PMCID: PMC8790393 DOI: 10.4254/wjh.v14.i1.300] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/04/2021] [Revised: 10/25/2021] [Accepted: 01/05/2022] [Indexed: 02/06/2023] Open
Abstract
Solid pseudopapillary neoplasm of the pancreas is a low-grade malignant tumor that predominantly affects young women in their third and fourth decade. Etiology and risk factors are unknown. Clinical symptoms are aspecific and most commonly due to mass effect. Diagnosis is made by computed tomography scan or magnetic resonance imaging and histological characterization is obtained by endoscopic ultrasound-guided fine needle biopsy. Microscopically, these lesions are composed by both solid and pseudopapillary structures with necrotic and hemorrhagic areas. Occasionally, the biological behavior is aggressive with tumor recurrence and distant metastasis. Usually, curative R0 surgical resection is the best option able to provide long term survival even in advanced disease. Unresectable disease is the main predictor of poor prognosis. Chemotherapy and radiotherapy regimens are not well standardized. However, they could be effective in reducing tumor size as neoadjuvant treatment or disease control in palliative setting. Although complete surgical resection provides a cure rate of > 95%, considering young age of the patients and morbidity associated to pancreatic surgery, further studies are needed to better investigate risk factors and responsiveness to hormones in order to allow early diagnosis and follow up strategies that could avoid unnecessary surgery in less aggressive disease.
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Affiliation(s)
- Simone Sibio
- Department of Surgery Pietro Valdoni, Sapienza University of Rome, Umberto I Hospital, Rome 00161, Italy
| | - Sara Di Carlo
- Minimally Invasive Surgery Unit, Tor Vergata Hospital, Tor Vergata University of Rome, Rome 00133, Italy
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Paredes O, Paredes K, Kawaguchi Y, Luque-Vasquez C, Chavez I, Celis J, Payet E, Ruiz E, Berrospi F. Clinicopathological factors associated with recurrence in patients undergoing resection of pancreatic solid pseudopapillary neoplasm. Discov Oncol 2021; 12:53. [PMID: 35201506 PMCID: PMC8777513 DOI: 10.1007/s12672-021-00451-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/11/2021] [Accepted: 11/17/2021] [Indexed: 11/06/2022] Open
Abstract
PURPOSE Solid pseudopapillary neoplasm (SPN) is an uncommon pathology with a low-grade malignancy. Surgery is the milestone treatment. Nevertheless, despite appropriate management, some patients present recurrence. Risk factors associated with recurrence are unclear. The objective was to identify the clinicopathological factors associated with recurrence in patients with SPN treated with pancreatic resection. METHODS Medical records of patients treated with pancreatic resection during 2006-2020 were evaluated. Patients with histological diagnosis of SPN were included. Survival analysis was performed to identify the clinicopathological factors related to recurrence. RESULTS Seventy-four patients were diagnosed with SPN; 70 (94.6%) patients were female, and the median age was 20 years old. The median tumor diameter was 7.9 cm. Multivisceral resection was performed in 9 (12.2%) patients. Four (5.4%) patients presented lymph node metastasis.R0 resection was achieved in all cases. Six (8%) patients presented recurrence and the liver was the most frequent recurrence site (n = 5).After a median follow-up of 40.2 months, 9 (12%) patients died. Five (6.8%) patients died of disease progression. The 1-3- and 5-year overall survival (OS) was 97.1%, 90.2% and 79.9%, respectively. The 1-3-and-5-year recurrence-free survival (RFS) was 98.4%, 89.9% and 87%, respectively. In the univariate Cox-regression analysis, age ≥ 28 years(HR = 8.61, 95% CI 1.1-73.8),tumor diameter ≥ 10 cm(HR = 9.3, 95% CI 1.12-79.6),invasion of adjacent organs (HR = 7.45, 95% CI 1.5-36.9), lymph node metastasis (pN +) (HR = 16.8, 95% CI 2.96-94.9) and, AJCC Stage III (HR = 10.1, 95% CI 1.2-90.9) were identified as predictors for recurrence. CONCLUSIONS SPN is more frequently diagnosed in young women with a good overall prognosis after an R0 surgical resection even with disease recurrence. Age ≥ 28 years, larger tumors ≥ 10 cm, invasion of adjacent organs, lymph node metastasis(pN +) and, AJCC Stage III were predictors factors of recurrence in resected SPN.
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Affiliation(s)
- Oscar Paredes
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru.
- , Montesquieu Street 277, Lima, Perú.
| | - Kori Paredes
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Yoshikuni Kawaguchi
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Carlos Luque-Vasquez
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Iván Chavez
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Juan Celis
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Eduardo Payet
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Eloy Ruiz
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Francisco Berrospi
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
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Wang X, Zhu D, Bao W, Li M, Wang S, Shen R. Prognostic Enigma of Pancreatic Solid Pseudopapillary Neoplasm: A Single-Center Experience of 63 Patients. Front Surg 2021; 8:771587. [PMID: 34881287 PMCID: PMC8645639 DOI: 10.3389/fsurg.2021.771587] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2021] [Accepted: 10/20/2021] [Indexed: 11/29/2022] Open
Abstract
Background: Studies investigating prognostic factors of solid pseudopapillary neoplasm (SPN) have been published with conflicting findings. Methods: Retrospective analysis of 63 consecutive cases of SPN in our institution from January 2010 to December 2019 was carried out. The clinicopathological features, treatment practices along with survival associations were collected and analyzed. Results: Fifteen patients (23.8%) were male, and 48 (76.2%) were female, with a median age of 34.0 ± 14.5 years. The larger tumor size was correlated with the more mixed components (p = 0.000) and the higher Ki-67 index (p = 0.042). No recurrence was found in the nine patients whose tumors fulfilled the WHO criteria for malignancy due to the presence of at least perineural invasion (6.4%), angiovascular invasion (2.3%), and/or adjacent organ invasion (6.4%). Microscopic infiltrative growth was detected in 9 (14.3%) tumors, which was correlated significantly with the WHO criteria (p = 0.002), capsule invasion (p = 0.005), and pancreatic parenchyma invasion (p = 0.001), but not with disease-free survival (p = 0.13). CD99 was found to be positively expressed in 88.9% (40/45) of tumors and more likely to have depressed Ki-67 index (p = 0.016). After a median follow-up of 58 months, only two patients (3.2%) had a recurrence after their first operation outside of our hospital. No patient died due to tumor progression. Conclusions: Although survival is favorable with aggressive surgery, it is actually difficult to assess the prognostic factors of resected SPNs. Future investigations into the role of clinicopathological evaluation will unveil the prognostic enigma of pancreatic SPN after resection.
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Affiliation(s)
- Xinbo Wang
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Daojun Zhu
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Wei Bao
- Department of Pathology, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Min Li
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Sizhen Wang
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Rongxi Shen
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
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Wang X, Zhu D, Bao W, Li M, Wang S, Shen R. Case Report: Targeted Therapy for Metastatic Solid Pseudopapillary Neoplasm of the Pancreas With CTNNB1 and PTEN Mutations. Front Oncol 2021; 11:729151. [PMID: 34733780 PMCID: PMC8558400 DOI: 10.3389/fonc.2021.729151] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Accepted: 10/04/2021] [Indexed: 12/24/2022] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasm (SPN) of the pancreas shows an indolent clinical behavior in cases undergoing surgical resection. The efficacy of combination therapy in the metastatic extrapancreatic SPN treatment remains largely unknown and a clinical challenge. CASE PRESENTATION We report a case of a metastatic pancreatic SPN in a 45-year-old woman who presented with an aggressive peritoneal dissemination and hepatic metastases and still showed an indolent clinical course with combination therapy with repeated surgery and targeted therapy. Although the follow-up effect remains to be seen, this is the first report of practical experience of the targeted agents sunitinib and everolimus in metastatic SPN tumors based on the mutation status of PTEN (c.379G>A; p.G127R) and CTNNB1 (c.98C>G; p.S33C). To our knowledge, the PTEN variant identified in this case has not been previously reported in SPN. CONCLUSION Evidence on variant genetics indicates that future molecular studies may not only help to explain the mechanism of SPN occurrence and development but are also more likely to direct to future precision treatments.
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Affiliation(s)
- Xinbo Wang
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Daojun Zhu
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Wei Bao
- Department of Clinical Pathology, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Min Li
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Sizhen Wang
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Rongxi Shen
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
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Rathi J, Anuragi G, J R LJ, R P, C S, O L NB. Prediction of Recurrence Risk in Solid Pseudopapillary Neoplasm of the Pancreas: Single-Institution Experience. Cureus 2021; 13:e17541. [PMID: 34646598 PMCID: PMC8478690 DOI: 10.7759/cureus.17541] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/29/2021] [Indexed: 01/13/2023] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasm (SPN) of the pancreas is a low-grade malignant neoplasm with unpredictable behavior. Factors associated with recurrence were not conclusively identified. The aim of this study is to define the clinicopathological criteria for recurrence risk prediction in SPNs based on the most recent scientific evidence and to present our experience with SPNs. METHODS A retrospective review of patients with SPNs operated on in our institution from June 2012 to June 2018 was completed. Patient characteristics and clinical outcomes were analyzed. A detailed literature review was performed to evaluate the factors associated with the recurrence of SPNs. RESULTS The cohort consisted of 13 female patients with a median age of 24 years and a mean tumor size of 7.7 cm. Body and tail (53.8%) were the most common location, and distal pancreatectomy with splenectomy was the prevalent surgical procedure. One patient of SPN operated on for local recurrence after 11 years which had high-grade malignant histological features on the previously resected tumor. At a median follow-up of 42 months (range 36 to 108), all patients were disease free and alive. The proposed criteria for predicting recurrence in SPNs include tumor size >8 cm, synchronous metastasis, malignant SPN (according to 2000 or 2010 World Health Organization [WHO] criteria), lymphovascular invasion, pancreatic parenchymal invasion, and high Ki-67 index (>4%). All these are worse prognostic factors and should be considered as high-risk factors for postoperative relapse. CONCLUSION The above-mentioned criteria can better predict SPN recurrence. Patients with high-risk features should undergo an extended follow-up.
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Affiliation(s)
- Jalaj Rathi
- Surgical Gastroenterology, Madras Medical College, Chennai, IND
| | | | - Livin Jose J R
- Surgical Gastroenterology, Madras Medical College, Chennai, IND
| | - Prabhakaran R
- Surgical Gastroenterology, Madras Medical College, Chennai, IND
| | - Sugumar C
- Surgical Gastroenterology, Madras Medical College, Chennai, IND
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Kotecha K, Pandya A, Gill AJ, Mittal A, Samra J. Pancreatic solid pseudopapillary neoplasm: a single-institution study. ANZ J Surg 2021; 91:2453-2458. [PMID: 34427035 DOI: 10.1111/ans.17142] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/01/2021] [Revised: 08/04/2021] [Accepted: 08/06/2021] [Indexed: 12/11/2022]
Abstract
BACKGROUNDS Solid pseudopapillary neoplasms (SPNs) are a distinct but rare form of low-grade pancreatic neoplasia, accounting for 0.3%-2.7% of all pancreatic tumours. They are most common in young females. Local recurrence and distant metastasis are reported but extremely rare, and are usually resectable with curative intent. We report the clinicopathological features and long-term outcomes of SPNs following surgical resection from a single institution. METHODS A total of 1296 patients undergoing pancreatic resection during the 30 years period from 1991 to 2020 were retrospectively reviewed, and those with a confirmed pathological diagnosis of pancreatic SPN on review were included. RESULTS Twenty-two patients (1.7% of all patients undergoing resection), were identified. Twenty patients (91%) were female. Unlike previous studies, most patients (91%) were symptomatic at diagnosis. On diagnostic CT, cystic components were visible in 16 patients (73%), calcifications were found in two patients (9%), haemorrhage in one patient (5%) and a defined capsule was seen in four patients (18%). Surgical resection was undertaken on all cases, with distal pancreatectomy the most commonly performed (n = 11, 50%). One patient (4.7%) had nodal involvement, nine patients had an incomplete tumour capsule (41%) and seven patients (32%) had tumour extension into the pancreatic parenchyma. Despite this, no patients had disease recurrence at 10 years. One patient died within 5 years of heart failure unrelated to the SPN process; no patients died within 10 years of the disease. CONCLUSION We confirm a high proportion of female patients. Interestingly, a high proportion of our cohort was investigated for symptomatic disease. Despite a high proportion of tumours with an incomplete capsule, and extension into the pancreatic parenchyma, our findings indicated that SPN patients have excellent survival after margin-negative surgical resection.
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Affiliation(s)
- Krishna Kotecha
- Department of Upper Gastrointestinal Surgery, Royal North Shore Hospital, St Leonards, New South Wales, Australia
| | - Advait Pandya
- Northern Clinical School, University of Sydney, Sydney, New South Wales, Australia
| | - Anthony J Gill
- Northern Clinical School, University of Sydney, Sydney, New South Wales, Australia.,NSW Health Pathology, Department of Anatomical Pathology, Royal North Shore Hospital, St Leonards, New South Wales, Australia.,Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research, Royal North Shore Hospital, St Leonards, New South Wales, Australia
| | - Anubhav Mittal
- Department of Upper Gastrointestinal Surgery, Royal North Shore Hospital, St Leonards, New South Wales, Australia.,Northern Clinical School, University of Sydney, Sydney, New South Wales, Australia
| | - Jaswinder Samra
- Department of Upper Gastrointestinal Surgery, Royal North Shore Hospital, St Leonards, New South Wales, Australia.,Northern Clinical School, University of Sydney, Sydney, New South Wales, Australia
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Prediction of Recurrence of Completely Resected Pancreatic Solid Pseudopapillary Neoplasms in Pediatric Patients: A Single Center Analysis. CHILDREN-BASEL 2021; 8:children8080632. [PMID: 34438523 PMCID: PMC8391822 DOI: 10.3390/children8080632] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 06/28/2021] [Revised: 07/12/2021] [Accepted: 07/23/2021] [Indexed: 12/14/2022]
Abstract
Background: Many previous studies have investigated the risk factors for the recurrence of pancreatic solid pseudopapillary neoplasms (SPNs), although a consensus has not yet been reached, despite this effort. We aimed to identify the predictive factors for recurrence in patients with SPNs who underwent complete surgical resection of the tumor. Methods: We retrospectively analyzed the records of pediatric patients with SPNs who underwent surgical resection at a single center between 2001 and 2018. Results: During the study period, 47 patients with SPNs underwent radical resection of the tumor. The median age of the patients was 14 (8–18) years. R0 resection was confirmed in every case and none of the patients presented with systemic metastasis at the time of diagnosis. The median follow-up period was 53.1 (30.8–150.8) months. Of the 47 patients, only two (4.2%) experienced recurrence. Using comparative analysis, we found that some factors such as a large tumor size, peripancreatic tissue invasion, and capsule invasion did not increase the risk of recurrence of SPNs. Lymph node metastasis was the only significant factor for recurrence in our study (p = 0.043). Conclusion: During our single center analysis, we found that only lymph node metastasis was a predictive factor for recurrence of SPNs among patients who underwent complete tumor resection. Long-term follow-up is required to determine whether SPNs will recur if lymph node metastasis is observed after surgery. Furthermore, therapeutic benefits of routine lymphadenectomy or sentinel lymph node biopsy should be investigated in future studies to reduce the risk of recurrence in patients with SPNs.
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Allam M, Hidalgo Salinas C, Machairas N, Kostakis ID, Watkins J, Fusai GK. Solid Pseudopapillary Neoplasms of the Pancreas: a Single-Center Experience and Review of the Literature. J Gastrointest Cancer 2021; 53:460-465. [PMID: 33877570 DOI: 10.1007/s12029-021-00638-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/11/2021] [Indexed: 12/15/2022]
Abstract
PURPOSE Pancreatic solid pseudopapillary neoplasms (SPNs) are rare borderline tumours mainly affecting young female patients. The number of patients diagnosed with SPNs has increased significantly in the last decades owing to the increased use of cross-sectional imaging investigating different abdominal symptoms, whilst a significant proportion are incidentally discovered during the process of evaluating other pathologies. We herein present our institutional experience of patients with SPN who underwent curative resection focusing on clinical, pathological features, and the long-term outcomes. METHODS All patients undergoing pancreatectomy in our institution for SPN from January 2010 until December 2018 were included. Clinical, perioperative, histological, and long-term outcomes were collected and analysed. RESULTS During the inclusion period, a total of 19 patients had a pathological diagnosis of SPNs after surgical resection. Sixteen of them were female (84%), while the median patient age was 30 (range 16-66) years. Nine patients (47%) underwent distal pancreatectomy and splenectomy, 2 (11%) underwent spleen preserving distal pancreatectomy, 6 (32%) underwent pancreatoduodenectomy, one (5%) underwent total pancreatectomy, and one (5%) central pancreatectomy. Seventeen patients underwent R0 resection. During a median follow-up of 23 months, no tumour recurrence or death was recorded. CONCLUSION In our experience, SPNs are rare tumours with low malignant potentials. Surgical resection remains the gold standard treatment and is associated with good prognosis.
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Affiliation(s)
- Moustafa Allam
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
| | - Camila Hidalgo Salinas
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
| | - Nikolaos Machairas
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK.
| | - Ioannis D Kostakis
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
| | - Jennifer Watkins
- Department of Cellular Pathology, Royal Free London NHS Foundation Trust, London, UK
| | - Giuseppe Kito Fusai
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
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Mafficini A, Lawlor RT, Ghimenton C, Antonello D, Cantù C, Paolino G, Nottegar A, Piredda ML, Salvia R, Milella M, Dei Tos AP, Fassan M, Scarpa A, Luchini C. Solid Pseudopapillary Neoplasm of the Pancreas and Abdominal Desmoid Tumor in a Patient Carrying Two Different BRCA2 Germline Mutations: New Horizons from Tumor Molecular Profiling. Genes (Basel) 2021; 12:genes12040481. [PMID: 33810291 PMCID: PMC8065547 DOI: 10.3390/genes12040481] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2021] [Revised: 03/19/2021] [Accepted: 03/25/2021] [Indexed: 02/08/2023] Open
Abstract
This case report describes the history of a 41 year-old woman with a solid pseudopapillary neoplasm (SPN) of the pancreas and a metachronous abdominal desmoid tumor (DT) that occurred two years after the SPN surgical resection. At next-generation sequencing of 174 cancer-related genes, both neoplasms harbored a CTNNB1 somatic mutation which was different in each tumor. Moreover, two BRCA2 pathogenic mutations were found in both tumors, confirmed as germline by the sequencing of normal tissue. The BRCA2 mutations were c.631G>A, resulting in the amino-acid change p.V211I, and c.7008-2A>T, causing a splice acceptor site loss. However, as the two neoplasms showed neither loss of heterozygosity nor somatic mutation in the second BRCA2 allele, they cannot be considered as BRCA-dependent tumors. Nevertheless, this study highlights the important opportunities opened by extensive tumor molecular profiling. In this particular case, it permitted the detection of BRCA2-germline mutations, essential for addressing the necessary BRCA-related genetic counseling, surveillance, and screening for the patient and her family.
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Affiliation(s)
- Andrea Mafficini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy; (A.M.); (R.T.L.); (C.C.); (A.N.); (A.S.)
| | - Rita T. Lawlor
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy; (A.M.); (R.T.L.); (C.C.); (A.N.); (A.S.)
| | - Claudio Ghimenton
- ARC-Net Research Centre, University and Hospital Trust of Verona, 37134 Verona, Italy; (C.G.); (G.P.); (M.L.P.)
| | - Davide Antonello
- Department of Surgery, The Pancreas Institute, University of Verona, 37134 Verona, Italy; (D.A.); (R.S.)
| | - Cinzia Cantù
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy; (A.M.); (R.T.L.); (C.C.); (A.N.); (A.S.)
| | - Gaetano Paolino
- ARC-Net Research Centre, University and Hospital Trust of Verona, 37134 Verona, Italy; (C.G.); (G.P.); (M.L.P.)
| | - Alessia Nottegar
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy; (A.M.); (R.T.L.); (C.C.); (A.N.); (A.S.)
| | - Maria L. Piredda
- ARC-Net Research Centre, University and Hospital Trust of Verona, 37134 Verona, Italy; (C.G.); (G.P.); (M.L.P.)
| | - Roberto Salvia
- Department of Surgery, The Pancreas Institute, University of Verona, 37134 Verona, Italy; (D.A.); (R.S.)
| | - Michele Milella
- Department of Medicine, Section of Medical Oncology, University of Verona, 37134 Verona, Italy;
| | - Angelo P. Dei Tos
- Department of Medicine (DIMED), Section of Pathological Anatomy, University of Padua, 35121 Padua, Italy; (A.P.D.T.); (M.F.)
| | - Matteo Fassan
- Department of Medicine (DIMED), Section of Pathological Anatomy, University of Padua, 35121 Padua, Italy; (A.P.D.T.); (M.F.)
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy; (A.M.); (R.T.L.); (C.C.); (A.N.); (A.S.)
- ARC-Net Research Centre, University and Hospital Trust of Verona, 37134 Verona, Italy; (C.G.); (G.P.); (M.L.P.)
| | - Claudio Luchini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy; (A.M.); (R.T.L.); (C.C.); (A.N.); (A.S.)
- Correspondence: ; Tel.: +39-045-8127548
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Lee G, Sung YN, Kim SJ, Lee JH, Song KB, Hwang DW, Kim J, Lee SS, Kim SC, Hong SM. Large tumor size, lymphovascular invasion, and synchronous metastasis are associated with the recurrence of solid pseudopapillary neoplasms of the pancreas. HPB (Oxford) 2021; 23:220-230. [PMID: 32654914 DOI: 10.1016/j.hpb.2020.05.015] [Citation(s) in RCA: 21] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2020] [Revised: 05/23/2020] [Accepted: 05/29/2020] [Indexed: 12/12/2022]
Abstract
BACKGROUND Solid pseudopapillary neoplasms (SPNs) of the pancreas have low malignant potential. However, malignant SPNs are not fully understood. METHODS To evaluate risk factors affecting malignant potential, the clinicopathologic features of 375 surgically resected SPNs were compared. RESULTS Fifty (13.3%) had malignant histologic features. Twenty-seven and 22 had perineural and lymphovascular invasions, respectively. Adjacent organ invasion was noted in 9 cases. Recurrence occurred in 8 cases. The median recurrence time after surgical resection was 67 months and was associated with a higher pT category (P = 0.001), lymphovascular invasion (P < 0.001), and synchronous metastasis (P < 0.001). SPN patients with malignant histologic features had worse recurrence-free survival (RFS; 10-year survival rate, 73.2%) than those without malignant histologic features (96.3%; P = 0.01). Patients with a higher pT category (P = 0.04), synchronous metastasis (P < 0.01), and lymphovascular invasion (P < 0.01) had worse RFS. Lymphovascular invasion (P = 0.042) and a higher T category (P = 0.002) were poor prognostic factors for recurrence. CONCLUSION Lymphovascular invasion and a higher T category were worse prognostic factors for recurrence in SPN patients with malignant histologic features. For SPN patients with malignant histologic features, a longer follow-up may be required.
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Affiliation(s)
- Goeun Lee
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - You-Na Sung
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Sung Joo Kim
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Jae Hoon Lee
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Ki-Byung Song
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Dae Wook Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Jihun Kim
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Sang Soo Lee
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Song Cheol Kim
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Seung-Mo Hong
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
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Rebai W, Ben Mahmoud A, Chammakhi A, Haddad A, Maghrebi H, Chaker Y, Ksantini R, Jouini M, Kacem MJ. Management of Solid and Pseudopapillary Tumors of the Pancreas: About 3 Case Reports. J Gastrointest Cancer 2021; 52:1119-1124. [PMID: 33484437 DOI: 10.1007/s12029-021-00589-y] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/17/2021] [Indexed: 12/11/2022]
Affiliation(s)
- Wael Rebai
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
| | - Ahmed Ben Mahmoud
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia. .,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia.
| | - Amine Chammakhi
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
| | - Anis Haddad
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
| | - Houcine Maghrebi
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
| | - Youssef Chaker
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
| | - Rachid Ksantini
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
| | - Mohamed Jouini
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
| | - Montasser Jameleddine Kacem
- Department of General Surgery A, La Rabta Hospital, Tunis, Tunisia.,Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia
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Din NU, Rahim S, Abdul-Ghafar J, Ahmed A, Ahmad Z. Clinicopathological and immunohistochemical study of 29 cases of solid-pseudopapillary neoplasms of the pancreas in patients under 20 years of age along with detailed review of literature. Diagn Pathol 2020; 15:139. [PMID: 33298094 PMCID: PMC7724627 DOI: 10.1186/s13000-020-01058-z] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2020] [Accepted: 11/29/2020] [Indexed: 01/12/2023] Open
Abstract
Background Pancreatic Solid Pseudopapillary Neoplasms (SPNs) are rare low-grade malignant tumors with a marked preponderance for young females. Objective was to describe the morphology, differential diagnosis, and prognosis of SPNs in patients under 20 years of age and present a detailed review of literature. Methods A total of 29 cases in patients under 20 years of age reported as SPN during the period January 2014 to December 2019, were included in the study. These included 19 resection specimens, 4 incision biopsies and 6 cases received as blocks for second opinion. Hematoxylin and eosin (H&E) slides as well as immunohistochemistry (IHC) slides of all cases were retrieved and reviewed by the authors. TFE3 and Progesterone Receptor were performed retrospectively. Results Twenty-eight of the 29 patients were females. Ages of patients ranged from 12 to 19 years. Nineteen cases were resections. Tail was the commonest location. Mean tumor size was 9.5 cm. In 89.5% cases, tumor was confined to the pancreas. In 2 cases, distant metastasis was present. In 2 cases, extension beyond pancreas was seen. Solid and pseudopapillary areas were seen in all cases while other features were variable. Beta catenin and Cyclin D1 were positive in most cases while TFE3 was positive in 57% cases. Progesterone Receptor (PR) was positive in all 13 cases in which it was performed. Follow up was available in 14 patients. Follow up period ranged from 3 to 70 months. Twelve were alive and well without recurrence or metastasis while 2 were alive with recurrence and metastasis to liver and omentum respectively. Conclusions Although many studies on SPNs have been published, surgeons, oncologists and even pathologists in this part of the world are often not aware of these rare tumors leading to inaccuracies and delays in diagnosis. In addition, this paper focusses on the interesting observation that the majority of SPNs diagnosed in our department during study period occurred in patients under 20 years of age (29 versus 21 in patients over 20). However, clinico-epidemiological, morphologic and prognostic features were similar in both age groups. Possibility of SPNs should always be considered in case of pancreatic neoplasms occurring in patients under 20 years of age as well. We believe that this is a very interesting and helpful study for the clinicians as well as the pathologists.
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Affiliation(s)
- Nasir Ud Din
- Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
| | - Shabina Rahim
- Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
| | - Jamshid Abdul-Ghafar
- Department of Pathology and Clinical Laboratory, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan.
| | - Arsalan Ahmed
- Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
| | - Zubair Ahmad
- Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan
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Kodama R, Koh Y, Midorikawa H, Yokota Y, Saegusa H, Ushimaru H. A case of recurrence of a solid pseudopapillary neoplasm of the pancreas effectively treated with proton beam radiotherapy. Clin J Gastroenterol 2020; 14:375-381. [PMID: 33052580 DOI: 10.1007/s12328-020-01262-w] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/30/2020] [Accepted: 10/05/2020] [Indexed: 02/08/2023]
Abstract
We report a case of a male in his 50 s who underwent pancreaticoduodenectomy for solid pseudopapillary neoplasm (SPN) of the pancreas at 30 years. He developed a liver abscess 15 years after the surgery, and CT scan revealed a swollen retroperitoneum lymph node and a tumor in the liver. Symptoms, including abdominal distension, appetite loss, and epigastric pain, appeared due to lymph node metastasis. Endoscopic ultrasonography-guided fine-needle aspiration against the lymph node revealed SPN recurrence. The tumor had invaded the common hepatic artery, and surgery was not indicated. Chemotherapy of Gemcitabine/nab-Paclitaxel biweekly was performed 8 times; however, no reduction in tumor size was observed, and the patient's symptoms worsened. Proton beam therapy (67.5 GyE in 25 fractions) was subsequently performed for lymph node metastasis, and led to a gradual reduction in lymph node metastasis, and an improvement in symptoms. No re-expansion of lymph node metastasis has been observed 3 years after proton beam therapy. Since SPN is low malignancy and most cases can be expected to be cured by surgery, there is currently no standard treatment of unresectable SPN. This case is the first report of proton beam therapy for SPN, and was considered to be effective.
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Affiliation(s)
- Ryo Kodama
- Department of Gastroenterology, JA Nagano Koseiren Minaminagano Medical Center, Shinonoi General Hospital, Nagano, Japan.
| | - Youshin Koh
- Department of Gastroenterology, JA Nagano Koseiren Minaminagano Medical Center, Shinonoi General Hospital, Nagano, Japan
| | - Hajime Midorikawa
- Department of Gastroenterology, JA Nagano Koseiren Minaminagano Medical Center, Shinonoi General Hospital, Nagano, Japan
| | - Yukiko Yokota
- Department of Gastroenterology, JA Nagano Koseiren Minaminagano Medical Center, Shinonoi General Hospital, Nagano, Japan
| | - Hisanobu Saegusa
- Department of Gastroenterology, JA Nagano Koseiren Minaminagano Medical Center, Shinonoi General Hospital, Nagano, Japan
| | - Hiroyasu Ushimaru
- Department of Gastroenterology, JA Nagano Koseiren Minaminagano Medical Center, Shinonoi General Hospital, Nagano, Japan
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33
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Luchini C, Grillo F, Fassan M, Vanoli A, Capelli P, Paolino G, Ingravallo G, Renzulli G, Doglioni C, D’Amuri A, Mattiolo P, Pecori S, Parente P, Florena AM, Zamboni G, Scarpa A. Malignant epithelial/exocrine tumors of the pancreas. Pathologica 2020; 112:210-226. [PMID: 33179623 PMCID: PMC7931574 DOI: 10.32074/1591-951x-167] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2020] [Accepted: 07/07/2020] [Indexed: 02/07/2023] Open
Abstract
Pancreatic malignant exocrine tumors represent the most important cause of cancer-related death for pancreatic neoplasms. The most common tumor type in this category is represented by pancreatic ductal adenocarcinoma (PDAC), an ill defined, stroma-rich, scirrhous neoplasm with glandular differentiation. Here we present the relevant characteristics of the most important PDAC variants, namely adenosquamous carcinoma, colloid carcinoma, undifferentiated carcinoma, undifferentiated carcinoma with osteoclast-like giant cells, signet ring carcinoma, medullary carcinoma and hepatoid carcinoma. The other categories of malignant exocrine tumors, characterized by fleshy, stroma-poor, circumscribed neoplasms, include acinar cell carcinoma (pure and mixed), pancreatoblastoma, and solid pseudopapillary neoplasms. The most important macroscopic, histologic, immunohistochemical and molecular hallmarks of all these tumors, highlighting their key diagnostic/pathological features are presented. Lastly, standardized indications regarding gross sampling and how to compile a formal pathology report for pancreatic malignant exocrine tumors will be provided.
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Affiliation(s)
- Claudio Luchini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Federica Grillo
- Anatomic Pathology, San Martino IRCCS Hospital, Genova, Italy
- Anatomic Pathology, Department of Surgical Sciences and Integrated Diagnostics (DISC), University of Genova, Italy
| | - Matteo Fassan
- Surgical Pathology Unit, Department of Medicine (DIMED), University of Padua, Italy
| | - Alessandro Vanoli
- Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, and IRCCS San Matteo Hospital, Italy
| | - Paola Capelli
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Gaetano Paolino
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Giuseppe Ingravallo
- Department of Emergency and Organ Transplantation, Section of Pathological Anatomy, University of Bari Aldo Moro, Bari, Italy
| | - Giuseppina Renzulli
- Department of Emergency and Organ Transplantation, Section of Pathological Anatomy, University of Bari Aldo Moro, Bari, Italy
| | - Claudio Doglioni
- Vita e Salute University, Milan, Italy
- Pathology Unit, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | | | - Paola Mattiolo
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Sara Pecori
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Paola Parente
- Pathology Unit, Fondazione IRCCS Ospedale Casa Sollievo della Sofferenza, San Giovanni Rotondo (FG), Italy
| | - Ada M. Florena
- Department of Sciences for Promotion of Health and Mother and Child Care, Anatomic Pathology, University of Palermo, Italy
| | - Giuseppe Zamboni
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
- IRCSS Sacro Cuore Don Calabria Hospital, Negrar, Italy
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
- ARC-NET Research Centre, University of Verona, Verona, Italy
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Solid Pseudopapillary Neoplasm of the Pancreas with High-Grade Malignant Transformation Involving p16-RB Pathway Alterations. Case Rep Surg 2020; 2020:5980382. [PMID: 32158585 PMCID: PMC7059088 DOI: 10.1155/2020/5980382] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2019] [Accepted: 12/30/2019] [Indexed: 12/16/2022] Open
Abstract
Solid pseudopapillary neoplasm (SPN) of the pancreas has generally been regarded as a low-grade malignant tumour that preferentially develops in young women and can have a good prognosis with surgery. Among the few patients who have died from metastatic SPN are mostly those whose tumours harbour an undifferentiated component characterized by diffuse sheets of cells with increased nuclear atypia and proliferative index. We herein report a case of an aggressive, fatal, solid pseudopapillary neoplasm (SPN) of the pancreas in a 63-year-old woman complaining of epigastric pain. Despite having undergone surgical resection for a 10 cm pancreatic mass and multiple liver metastases, the patient later died due to uncontrollable metastases 36 months after the initial surgery. Histological examination showed that the tumour displayed unusual high-grade malignant features, showing diffuse sheets of cells with increased nuclear atypia and proliferative activity, along with conventional low-grade malignant features. The tumour was subsequently recognized as an SPN with foci of high-grade malignant transformation according to the 2010 World Health Organization classification. Immunohistochemical studies revealed that p16-RB pathway alterations contributed to the high-grade malignant transformation. The present case report suggests the necessity for developing diagnostic and treatment methods targeting p16 and RB for high-grade variants of SPN.
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35
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Yepuri N, Naous R, Meier AH, Cooney RN, Kittur D, Are C, Jain A, Dhir M. A systematic review and meta-analysis of predictors of recurrence in patients with Solid Pseudopapillary Tumors of the Pancreas. HPB (Oxford) 2020; 22:12-19. [PMID: 31350105 DOI: 10.1016/j.hpb.2019.06.005] [Citation(s) in RCA: 21] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2018] [Revised: 06/02/2019] [Accepted: 06/09/2019] [Indexed: 12/12/2022]
Abstract
BACKGROUND The recurrence rates and predictors of recurrence in patients with Solid Pseudopapillary tumors (SPT) are unclear, which makes it challenging to determine the duration of follow-up. The aim of the current study was to perform a systematic review and meta-analysis to determine the recurrence rates and pathologic factors associated with recurrence in patients with SPT. METHODS A PubMed, Scopus, and Web of Science search was conducted to identify studies of SPT published during the last 15 years: (09/2002-09/2017). Studies reporting on patients with SPT and follow-up of >5 years were included. The search strategy was conducted per 2009 PRISMA guidelines. RESULTS A total of 103 studies reporting on 2599 non-metastatic SPT patients were identified. Sixty-nine patients (2.6%) developed recurrence during follow-up. Pooled estimates from studies with a sample size >20 (N = 33) noted an overall recurrence rate of 2% (95% CI 1-2%). Male gender (OR 1.960), positive lymph nodes (OR 11.9), R1 margins (OR 11.1), and LVI (OR 5.5), were associated with a significantly (all p < 0.05) increased risk of recurrence. CONCLUSION Current meta-analysis suggests that only 2% of patients with SPT experience recurrence after resection. These data will guide the treating physicians and patients regarding recurrence rates and help identify patients at increased risk of recurrence during follow-up.
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Affiliation(s)
- Natesh Yepuri
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Rana Naous
- Department of Pathology, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Andreas H Meier
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Robert N Cooney
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Dilip Kittur
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Chandrakanth Are
- Department of Surgery, Division of Surgical Oncology, University of Nebraska Medical Center, Omaha, NE, 68198, USA
| | - Ajay Jain
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA
| | - Mashaal Dhir
- Department of Surgery, SUNY Upstate Medical University, Syracuse, NY, 13210, USA.
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Solid Pseudopapillary Neoplasms of the Pancreas: Clinicopathologic and Radiologic Features According to Size. AJR Am J Roentgenol 2019; 213:1073-1080. [DOI: 10.2214/ajr.18.20715] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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Dokmak S, Aussilhou B, Paci M, Ftériche FS, Cros J, Maire F, Soubrane O, Sauvanet A. Extended Laparoscopic Central Pancreatectomy with Clamping of the Mesentericoportal Vein and Resection of the Splenic Vessels for a Large Solid Pseudopapillary Tumor. Ann Surg Oncol 2019; 26:3709-3710. [DOI: 10.1245/s10434-019-07689-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2019] [Indexed: 11/18/2022]
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Wang J, Chen X, Wang C, Cui W, Ren S, Wang Z, Li H, Wang Z. Differentiation of aggressive from non-aggressive pancreatic solid pseudopapillary neoplasms using computed tomography. Abdom Radiol (NY) 2019; 44:2448-2458. [PMID: 30850890 DOI: 10.1007/s00261-019-01969-6] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
PURPOSES Microscopic aggressive behaviors may be related with the prognosis of solid pseudopapillary neoplasms (SPNs). In this study, we investigate computed tomography (CT) features and differential diagnosis of aggressive and non-aggressive SPNs in pancreas. MATERIALS AND METHODS 122 patients with pathologically proven SPNs in pancreas were included. Patients' age, tumor site, texture, shape, margins, exophytic growth, capsule, calcification, hemorrhage, pancreatic duct dilatation or pancreatic parenchyma atrophy, peripancreatic infiltration or metastases, vascular encasement, and enhancement pattern were assessed. The diagnostic accuracy was analyzed by using the receiver operating characteristic curve (ROC). RESULTS There were 30 aggressive SPNs and 92 non-aggressive SPNs. Aggressive SPNs showed significantly higher frequencies of an ill-defined margin, patient age > 40.5 years, and tumor size < 42.1 mm, but lower frequencies of complete capsule, hemorrhage compared with non-aggressive SPNs (p < 0.05). Lack of complete capsule and age > 40.5 years were independent risk factors of aggressive SPNs (odd ratio 7.08 and 3.1, respectively). When we applied the two predictors in the logistic regression model, the area under the curve (AUC) was 0.77 with sensitivity of 86.7% and specificity of 55.4%. CONCLUSION Size less than 42.1 mm, lack of complete capsule, ill-defined, and absent bleeding are useful CT imaging features for predicating aggressive SPNs. Patient age > 40.5 years and lack of complete capsule showed acceptable diagnostic performance for discriminating aggressive from non-aggressive SPNs.
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Affiliation(s)
- Jianhua Wang
- Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, No. 155 Hanzhong Road, Nanjing, 210029, Jiangsu, China
| | - Xiao Chen
- Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, No. 155 Hanzhong Road, Nanjing, 210029, Jiangsu, China
| | - Cheng Wang
- Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, No. 155 Hanzhong Road, Nanjing, 210029, Jiangsu, China
| | - Wenjing Cui
- Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, No. 155 Hanzhong Road, Nanjing, 210029, Jiangsu, China
| | - Shuai Ren
- Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, No. 155 Hanzhong Road, Nanjing, 210029, Jiangsu, China
| | - Zhonglan Wang
- Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, No. 155 Hanzhong Road, Nanjing, 210029, Jiangsu, China
| | - Hui Li
- Department of Pathology, The Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, 210029, Jiangsu, China
| | - Zhongqiu Wang
- Department of Radiology, The Affiliated Hospital of Nanjing University of Chinese Medicine, No. 155 Hanzhong Road, Nanjing, 210029, Jiangsu, China.
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Outcome and prognosis after pancreatectomy in patients with solid pseudopapillary neoplasms. Pancreatology 2019; 19:699-709. [PMID: 31227367 DOI: 10.1016/j.pan.2019.06.008] [Citation(s) in RCA: 33] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2019] [Revised: 05/30/2019] [Accepted: 06/12/2019] [Indexed: 12/11/2022]
Abstract
BACKGROUND Solid pseudopapillary neoplasms (SPN) are rare and represent approximately 4% of all cystic pancreatic tumors. The prognosis is excellent, although 10-15% of SPN patients show metastasis at the time of surgery or tumor recurrence during follow-up after pancreatectomy. Aim of the study was to analyze surgical management, risk factors for malignancy as well as long-term outcome and prognosis of this distinct tumor entity. METHOD All patients with pancreatic resection for SPN between 10/2001 and 07/2018 in the authors' institution were identified from a prospective database. Clinicopathologic details, perioperative data and long-term follow-up results were retrospectively analyzed. RESULTS Fifty-two patients were identified, 44 (85%) of them were female and the median age was 29 years (IQR 9-71). Seven (13%) patients showed a malignant behaviour of SPN with N1 (n = 2) or M1 (n = 1) disease at resection; 5 patients developed tumor recurrence, after a median of 21 months. During follow-up time (median 54 months) all patients were alive, the 5- and 10-year rates for disease-free survival were 89.0% and 81.6%, respectively. Significant risk factors for recurrence were age <18 years (p = 0.0087) and parenchyma-preserving surgical approaches (p = 0.0006). The postoperative long-term outcome showed ECOG = 0-1 in all patients, with resection related exocrine insufficiency in 20 (41%) and diabetes mellitus in 2 (4%) patients. CONCLUSIONS Age < 18 years is a significant risk factor for malignancy in SPN, and parenchyma preserving resections harbor a significant risk for tumor recurrence. As recurrence may occur late, a systematic life-long follow-up should be performed.
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40
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Dioguardi Burgio M, Ronot M, Vilgrain V. Rare Solid Tumor of the Exocrine Pancreas: A Pictorial Review. Semin Ultrasound CT MR 2019; 40:483-499. [PMID: 31806147 DOI: 10.1053/j.sult.2019.04.007] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Numerous other solid primary neoplasms may arise from the pancreas besides primary ductal adenocarcinomas and neuroendocrine tumors. Although diagnosis can be difficult because of the very low incidence of these tumors, knowledge of several, typical, epidemiologic, biological, and imaging features can help obtain a correct diagnosis. This pictorial review describes the features of solid rare primary pancreatic neoplasms on computed tomography and magnetic resonance imaging focusing on characteristics that can help radiologists differentiate them from classical forms of ductal pancreatic adenocarcinoma and neuroendocrine tumors. Cystic pancreatic neoplasms are beyond the scope of the current review.
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Affiliation(s)
- Marco Dioguardi Burgio
- Department of Radiology, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France; INSERM U1149, Centre de Recherche Biomédicale Bichat-Beaujon, CRB3, Paris, France.
| | - Maxime Ronot
- Department of Radiology, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France; INSERM U1149, Centre de Recherche Biomédicale Bichat-Beaujon, CRB3, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France
| | - Valérie Vilgrain
- Department of Radiology, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France; INSERM U1149, Centre de Recherche Biomédicale Bichat-Beaujon, CRB3, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France
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41
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Costales C, Mehta A, Kulkarni S, Larson BK. Clear Cell Variant of Solid Pseudopapillary Neoplasm of the Pancreas: A Report of a Rare Variant and Review of the Literature. Int J Surg Pathol 2019; 27:535-540. [PMID: 30845855 DOI: 10.1177/1066896919833790] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
The clear cell variant of solid pseudopapillary neoplasm (ccSPN) of the pancreas was first described in 2006. In this article, we report a case of this rare variant and review the few published reports. Both the current and previous reports show that ccSPN has several morphologic differences from conventional SPN, including clear vacuoles, fewer pseudopapillary formations, more solid/diffuse architecture, less hemorrhage, and fewer cholesterol clefts. Some of these features peculiar to ccSPN, such as solid/diffuse architecture, have been proposed to suggest aggressive behavior, though reports of ccSPN are rare and often have limited clinical follow-up. ccSPN also appears to occur more frequently in males than conventional SPNs. These clinical and pathologic features lead to unique set of differential diagnostic considerations for ccSPN, including metastatic renal cell carcinoma, perivascular epithelial cell tumor, and clear cell variants of other carcinomas. These unique features, atypical differential, and uncertain prognostic ramifications all make ccSPN an important variant to be aware of and report.
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Affiliation(s)
| | - Arjun Mehta
- 1 University of Southern California, Los Angeles, CA, USA
| | - Sujit Kulkarni
- 1 University of Southern California, Los Angeles, CA, USA
| | - Brent K Larson
- 1 University of Southern California, Los Angeles, CA, USA
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42
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Hu S, Zhang H, Wang X, Sun Z, Ge Y, Yan G, Zhao C, Chen K. Asymptomatic versus symptomatic solid pseudopapillary tumors of the pancreas: clinical and MDCT manifestations. Cancer Imaging 2019; 19:13. [PMID: 30845987 PMCID: PMC6407219 DOI: 10.1186/s40644-019-0198-4] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2018] [Accepted: 02/21/2019] [Indexed: 12/19/2022] Open
Abstract
BACKGROUND To delineate the features of multi-detector computed tomography (MDCT) images and clinical characteristics of pancreatic solid pseudopapillary tumors (SPTs) of the pancreas in asymptomatic patients and compare these features and characteristics between asymptomatic and symptomatic patients. METHODS This work is a retrospective study approved by our institutional review board. MDCT images and clinical data of 109 patients with pathologically proven SPTs obtained from October 2008 to October 2016 were reviewed. Patients were categorized into two groups: asymptomatic patients and patients with symptomatic disease. Cases were reviewed to determine the reason for detection, intervention, shape, diameter, location, calcification, encapsulation, internal composition, CT attenuation, enhancement pattern, and tumor pathology. Clinical factors and imaging features were also compared between groups. Statistical analysis was performed using χ2 and t-tests. RESULTS Data from 49 asymptomatic and 60 symptomatic patients were collected. Asymptomatic SPTs were identified most frequently during routine health examination (18 patients, 36.7%), various screening purposes (12 patients, 24.5%), and traumatic injury (9 patients, 18.4%). Except for a smaller tumor size (5.8 cm in asymptomatic SPTs vs. 7.4 cm in symptomatic SPTs, P = 0.023), the clinical factors or imaging features of asymptomatic patients were very similar to those of symptomatic patients. CONCLUSIONS The current research is the first single-center study to characterize SPTs in asymptomatic patients. Asymptomatic SPTs are gradually being identified with greater frequency. Although generally smaller in size than that in symptomatic patients, an asymptomatic pancreatic mass with the typical imaging features of SPT may be found, the treatment for which is similar to that for symptomatic patients. Evaluating asymptomatic SPTs requires further systematic and multi-center trials.
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Affiliation(s)
- Shudong Hu
- Department of Radiology, Affiliated Hospital, Jiangnan University, No. 200, Huihe Road, Wuxi, 214062, Jiangsu, China. .,Department of Radiology, Affiliated Renmin Hospital, Jiangsu University, No. 8, Dianli Road, Zhenjiang, 212002, Jiangsu, China.
| | - Heng Zhang
- Department of Radiology, Affiliated Renmin Hospital, Jiangsu University, No. 8, Dianli Road, Zhenjiang, 212002, Jiangsu, China
| | - Xian Wang
- Department of Radiology, Affiliated Renmin Hospital, Jiangsu University, No. 8, Dianli Road, Zhenjiang, 212002, Jiangsu, China
| | - Zongqiong Sun
- Department of Radiology, Affiliated Hospital, Jiangnan University, No. 200, Huihe Road, Wuxi, 214062, Jiangsu, China
| | - Yuxi Ge
- Department of Radiology, Affiliated Hospital, Jiangnan University, No. 200, Huihe Road, Wuxi, 214062, Jiangsu, China
| | - Gen Yan
- Department of Radiology, Affiliated Hospital, Jiangnan University, No. 200, Huihe Road, Wuxi, 214062, Jiangsu, China
| | - Changyong Zhao
- Department of General Surgery, Affiliated Hospital, Jiangnan University, No. 200, Huihe Road, Wuxi, 214062, Jiangsu, China
| | - Kemin Chen
- Department of Radiology, Ruijin Hospital, Shanghai Jiao tong University, School of Medicine, No. 197, Ruijin Er Road, Shanghai, 200025, China
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Bowman AW, Bolan CW. MRI evaluation of pancreatic ductal adenocarcinoma: diagnosis, mimics, and staging. Abdom Radiol (NY) 2019; 44:936-949. [PMID: 29967986 DOI: 10.1007/s00261-018-1686-x] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
The radiologist's role in the evaluation of pancreatic ductal adenocarcinoma remains critical in the management of this deadly disease. Imaging plays a vital role in the diagnosis and staging of pancreatic cancer. Although CT is more commonly used for staging pancreatic cancer, MR is increasingly playing an important role in this regard. In our institution, all pancreatic malignancies undergo staging with MRI. In this pictoral essay, we illustrate the MR imaging features of pancreatic ductal adenocarcinoma and its mimics, and we also discuss pearls and pitfalls in MR staging of pancreatic carcinoma.
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Affiliation(s)
- Andrew W Bowman
- Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.
| | - Candice W Bolan
- Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA
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44
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Hansen CP, Kristensen TS, Storkholm JH, Federspiel BH. Solid pseudopapillary neoplasm of the pancreas: Clinical-pathological features and management, a single-center experience. Rare Tumors 2019; 11:2036361319878513. [PMID: 31598207 PMCID: PMC6764028 DOI: 10.1177/2036361319878513] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/31/2019] [Accepted: 08/22/2019] [Indexed: 12/14/2022] Open
Abstract
Solid pseudopapillary neoplasm of the pancreas is a rare tumor of low malignancy that occurs most often in females. The study describes the clinicopathologic characteristics of the tumor and common differential diagnoses. Data were collected from a prospectively maintained database. Of 1661 patients operated for pancreatic tumors between January 2001 and September 2018, 15 patients were recorded. Patients included 12 females and 3 males, median age 40 (range 10 -87) years. Computed tomography or magnetic resonance imaging was diagnostic in eight patients and a preoperative biopsy in eight out of 10 patients. Median tumor size was 5 cm (range 2 -16 cm), 12 tumors were in the head, six in the body, and three in the tail of the gland. All patients except one had radical resection including one with hepatic and lymph node metastases, no patient underwent oncologic treatment. All patients are alive from 17.5 to 209.4 months postoperatively and without recurrence. Radical operation is usually curative and should also be offered to patients with metastases or recurrence as oncologic treatment has limited effect.
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Affiliation(s)
- Carsten Palnaes Hansen
- Department of Surgery, Rigshospitalet,
Copenhagen University Hospital, Copenhagen, Denmark
- Carsten Palnaes Hansen, Department of
Surgery, Rigshospitalet, Copenhagen University Hospital, DK-2100 Copenhagen,
Denmark.
| | | | - Jan Henrik Storkholm
- Department of Surgery, Rigshospitalet,
Copenhagen University Hospital, Copenhagen, Denmark
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45
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Srinivasa GY, Kapoor R, Dracham C, Gupta A. Expanding role of radiation in pancreatic tumors – A rare case report and a new horizon. INTERNATIONAL JOURNAL OF NONCOMMUNICABLE DISEASES 2019. [DOI: 10.4103/jncd.jncd_4_19] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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46
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Zhang MY, Tian BL. Pancreatic panniculitis and solid pseudopapillary tumor of the pancreas: A case report. World J Clin Cases 2018; 6:1036-1041. [PMID: 30568960 PMCID: PMC6288509 DOI: 10.12998/wjcc.v6.i15.1036] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/14/2018] [Revised: 11/03/2018] [Accepted: 11/07/2018] [Indexed: 02/05/2023] Open
Abstract
Solid pseudopapillary tumor of the pancreas (SPTP), also known as solid and papillary epithelial neoplasm of the pancreas, is a rare pancreatic exocrine tumor that is difficult to diagnose before surgery. Pancreatic panniculitis is a rare type that occurs in less than 3% of all patients with pancreatic diseases. We here report a 19-year-old woman who presented with persistent left upper quadrant pain without obvious cause for 1 d. The patient also developed subcutaneous nodules involving lower abdomen bilaterally and lower limbs, and subcutaneous nodules were pathologically diagnosed as pancreatic panniculitis. Plain abdominal computed tomography revealed a soft-tissue mass in the body and tail of the pancreas, which was closely associated with the gastric wall. Contrast-enhanced ultrasound showed inhomogeneous echogenicity in the anterior pancreatic body, which had blurred parenchymal demarcation of the body and tail of the pancreas. Contrast-enhanced abdominal computed tomography revealed a mixed density mass with solid and cystic components in the body and tail of the pancreas, and the solid component was markedly enhanced. The lesion was pathologically diagnosed as SPTP after laparoscopic resection. Clinicians should be aware of the clinical manifestation, diagnosis, and treatment of pancreatic panniculitis and SPTP.
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Affiliation(s)
- Meng-Yu Zhang
- Department of Pancreatic Surgery, West China Hospital/West China School of Medicine, Sichuan University, Chengdu 610041, Sichuan Province, China
- Department of Hepatobiliary Surgery, the Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Bo-Le Tian
- Department of Pancreatic Surgery, West China Hospital/West China School of Medicine, Sichuan University, Chengdu 610041, Sichuan Province, China
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47
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Amato E, Mafficini A, Hirabayashi K, Lawlor RT, Fassan M, Vicentini C, Barbi S, Delfino P, Sikora K, Rusev B, Simbolo M, Esposito I, Antonello D, Pea A, Sereni E, Ballotta M, Maggino L, Marchegiani G, Ohike N, Wood LD, Salvia R, Klöppel G, Zamboni G, Scarpa A, Corbo V. Molecular alterations associated with metastases of solid pseudopapillary neoplasms of the pancreas. J Pathol 2018; 247:123-134. [PMID: 30306561 PMCID: PMC6588017 DOI: 10.1002/path.5180] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2018] [Revised: 09/13/2018] [Accepted: 10/04/2018] [Indexed: 12/13/2022]
Abstract
Solid pseudopapillary neoplasms (SPN) of the pancreas are rare, low‐grade malignant neoplasms that metastasise to the liver or peritoneum in 10–15% of cases. They almost invariably present somatic activating mutations of CTNNB1. No comprehensive molecular characterisation of metastatic disease has been conducted to date. We performed whole‐exome sequencing and copy‐number variation (CNV) analysis of 10 primary SPN and comparative sequencing of five matched primary/metastatic tumour specimens by high‐coverage targeted sequencing of 409 genes. In addition to CTNNB1‐activating mutations, we found inactivating mutations of epigenetic regulators (KDM6A, TET1, BAP1) associated with metastatic disease. Most of these alterations were shared between primary and metastatic lesions, suggesting that they occurred before dissemination. Differently from mutations, the majority of CNVs were not shared among lesions from the same patients and affected genes involved in metabolic and pro‐proliferative pathways. Immunostaining of 27 SPNs showed that loss or reduction of KDM6A and BAP1 expression was significantly enriched in metastatic SPNs. Consistent with an increased transcriptional response to hypoxia in pancreatic adenocarcinomas bearing KDM6A inactivation, we showed that mutation or reduced KDM6A expression in SPNs is associated with increased expression of the HIF1α‐regulated protein GLUT1 at both primary and metastatic sites. Our results suggest that BAP1 and KDM6A function is a barrier to the development of metastasis in a subset of SPNs, which might open novel avenues for the treatment of this disease. © 2018 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.
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Affiliation(s)
- Eliana Amato
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy.,Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Andrea Mafficini
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy.,Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Kenichi Hirabayashi
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy.,Department of Pathology, Tokai University School of Medicine, Isehara, Japan
| | - Rita T Lawlor
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy.,Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Matteo Fassan
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy
| | - Caterina Vicentini
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy.,Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Stefano Barbi
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Pietro Delfino
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Katarzyna Sikora
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy
| | - Borislav Rusev
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy
| | - Michele Simbolo
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy
| | - Irene Esposito
- Institute of Pathology, Heinrich-Heine-University and University Hospital of Düsseldorf, Düsseldorf, Germany
| | - Davide Antonello
- Department of Surgery, General Surgery B, University of Verona, Verona, Italy
| | - Antonio Pea
- Department of Surgery, General Surgery B, University of Verona, Verona, Italy
| | - Elisabetta Sereni
- Department of Surgery, General Surgery B, University of Verona, Verona, Italy
| | - Maria Ballotta
- Section of Anatomic Pathology, Azienda Ospedaliera Rovigo, Rovigo, Italy
| | - Laura Maggino
- Department of Surgery, General Surgery B, University of Verona, Verona, Italy
| | | | - Nobuyuki Ohike
- Department of Pathology and Laboratory Medicine, Showa University Fujigaoka Hospital, Yokohama, Japan
| | - Laura D Wood
- Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
| | - Roberto Salvia
- Department of Surgery, General Surgery B, University of Verona, Verona, Italy
| | - Günter Klöppel
- Department of Pathology, Technical University Munich, Munich, Germany
| | - Giuseppe Zamboni
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy.,Division of Pathology, Sacro Cuore-Don Calabria Hospital, Negrar, Italy
| | - Aldo Scarpa
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy.,Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Vincenzo Corbo
- ARC-Net Research Centre, University and Hospital Trust of Verona, Verona, Italy.,Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
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48
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Clinical and Pathological Features of Solid Pseudopapillary Neoplasms of the Pancreas: A Nationwide Multicenter Study in Japan. Pancreas 2018; 47:1019-1026. [PMID: 30059473 DOI: 10.1097/mpa.0000000000001114] [Citation(s) in RCA: 39] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
OBJECTIVE The aim of this study was to evaluate the clinicopathological features of solid pseudopapillary neoplasms (SPNs). METHODS In this retrospective study, 288 SPNs were analyzed. RESULTS Overall, 214 patients (74%) were female. Distant metastases occurred in 4 patients, and splenic vein tumor thrombus occurred in 1 patient. Although imaging findings showed large (>2.0 cm) SPNs with clear, regular border, and heterogeneous internal structure, small SPNs appeared as almost entirely solid. Surgical resection was performed in 278 cases. The 5-year survival rate was 98.8%. Six patients had tumor recurrence after the initial resection. The detection rate in typical pathological findings was low for small SPNs. Tumor extension to the pancreatic parenchyma was detected in greater than 70% of the cases. Tumor invasion to adjacent organs was detected in 13 cases. One was given a diagnosis of apparent high-grade malignant transformation. CONCLUSIONS The proportion of male cases was higher than that in previous studies, and there were statistically significant differences in the onset age and tumor diameter between male and female patients. Therefore, women seemed to have an early occurrence of SPNs, suggesting a difference in the developmental stage between men and women. Images and pathological findings of SPNs varied according to tumor size. Our findings indicated that SPN patients have excellent survival after margin-negative surgical resection.
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Abstract
OBJECTIVES Prognostic factors for solid pseudopapillary neoplasms (SPN) of the pancreas have been incompletely defined in the literature. We aimed to use the National Cancer Database to describe survival and characterize prognostic factors in patients with pancreatic pseudopapillary neoplasms. METHODS We identified 304 patients with pancreatic SPN diagnosed between 2004 and 2012 using the National Cancer Database. All patients were included in the survival analysis. Kaplan-Meier method, χ, and log-rank tests were used for statistical analysis. Cox proportional hazard regression model was used for multivariate analysis. RESULTS The median age at diagnosis was 36.5 years. Eighty-five percent of patients were female, 69% were white, and 25% were black. Metastases were observed in 5% of patients. Median tumor size was 5.1 cm. Ninety-two percent of patients underwent primary tumor resection. At 60 months, 98% of patients who underwent resection were alive, and 40% who did not undergo resection were alive. On multivariate analysis, female sex, resection of primary tumor, and absence of metastasis were correlated with improved survival. CONCLUSIONS Patients with SPN who undergo resection have an excellent survival at 5 years. Surgery should be considered in all patients with this diagnosis.
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50
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You L, Yang F, Fu DL. Prediction of malignancy and adverse outcome of solid pseudopapillary tumor of the pancreas. World J Gastrointest Oncol 2018; 10:184-193. [PMID: 30079144 PMCID: PMC6068856 DOI: 10.4251/wjgo.v10.i7.184] [Citation(s) in RCA: 29] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2018] [Revised: 04/23/2018] [Accepted: 05/23/2018] [Indexed: 02/05/2023] Open
Abstract
Since solid pseudopapillary tumor of the pancreas (SPTP) was officially classified by the World Health Organization in 1996, SPTP has recently received special attention in the literature. Studies have shown that SPTP is a heterogeneous tumor, with a small percentage of patients harboring aggressive behaviors. However, criteria for malignancy grade in SPTP have not been well established. The prognosis of SPTP is generally good, with cases having a chance for long-term survival even with recurrence and/or metastasis after surgical resection. The current American Joint Committee on Cancer/Union for International Cancer Control tumor, node, metastasis staging system is not specific to SPTP. The lack of a predictive staging classification that accurately describes the heterogeneity of this disease hinders meaningful research into optimal individualized therapy. Here we summarize and discuss the associated factors proposed for appraisal of the malignant potential and adverse outcome of SPTP.
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Affiliation(s)
- Li You
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China
| | - Feng Yang
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China
| | - De-Liang Fu
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China
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