1
|
Vizcaino MA, Folpe AL, Huffman H, Panchal RR, Nielsen GP, Kipp BR, Turakulov R, Aldape K, Giannini C. Pseudoendocrine sarcoma: clinicopathologic, molecular, and epigenetic features of one case. Virchows Arch 2023; 483:899-904. [PMID: 37953374 DOI: 10.1007/s00428-023-03695-3] [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: 10/09/2023] [Revised: 10/24/2023] [Accepted: 10/31/2023] [Indexed: 11/14/2023]
Abstract
Pseudoendocrine sarcoma (PES) is a recently described neoplasm typically arising in paravertebral soft tissues. Histologically, PES resembles well-differentiated neuroendocrine tumors but lacks expression of epithelial/neuroendocrine markers, and most show aberrant nuclear β-catenin positivity. We describe the clinicopathological and molecular features and DNA methylation profile of one PES. A resected paraspinal soft tissue mass in a 52-year-old man showed a neuroendocrine-like neoplasm, negative for keratin, and synaptophysin and showing diffuse nuclear β-catenin expression. Targeted NGS confirmed a CTNNB1 (p.S37C) mutation. Whole genome methylation analysis showed no match to any methylation class in the central nervous system tumor (versions 11b6 and 12b6) or sarcoma classifier (calibrated scores of ≤0.3), but clustered together with a recently reported PES in which methylation analysis was also performed. He remained disease-free for 18 months after surgery, followed by chemoradiation. As more cases are examined, our findings suggest that PES may have a unique methylation profiling signature.
Collapse
Affiliation(s)
- M Adelita Vizcaino
- Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | - Andrew L Folpe
- Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | | | | | - G Petur Nielsen
- Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA
| | - Benjamin R Kipp
- Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | - Rust Turakulov
- Laboratory of Pathology, National Cancer Institute/Center for Cancer Research, Bethesda, MD, USA
| | - Kenneth Aldape
- Laboratory of Pathology, National Cancer Institute/Center for Cancer Research, Bethesda, MD, USA
| | - Caterina Giannini
- Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA.
| |
Collapse
|
2
|
Stefanova N, Kalinov T, Kolev N, Kalchev E. Frantz Tumor: A Case Report of Solid Pseudopapillary Tumor of Pancreas. Cureus 2023; 15:e41698. [PMID: 37575792 PMCID: PMC10413994 DOI: 10.7759/cureus.41698] [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] [Accepted: 07/10/2023] [Indexed: 08/15/2023] Open
Abstract
The solid pseudopapillary tumor (SPT) is a rare pancreatic lesion that usually affects young and middle-aged patients and has a female predominance and low malignant potential. The exact histogenesis of this tumor is still unclear. We present the case of a 60-year-old female patient with occasional abdominal pain. Positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) revealed a tumor mass in the pancreatic tail. Distal pancreatectomy and splenectomy were performed. The result from the pathology report was solid pseudopapillary neoplasm (SPN). The patient underwent four cycles of adjuvant chemotherapy with gemcitabine, which she tolerated well without complaints. A control computed tomography (CT) scan and PET/CT of the abdomen (five months after the operation) showed a cystic lesion suspicious for local recurrence in the pancreatic tail during the follow-up period. The patient underwent a second surgery operation. Subsequent histological examination showed chronic indurative pancreatitis, areas with steatonecrosis, lipogranulomas, and fibrosis without evidence of relapse. SPT is a rare pancreatic tumor that most commonly affects young women. Although the tumor has locally aggressive characteristics, the prognosis is excellent after surgical excision. Our case emphasizes that this tumor can occur not only in young women but also in older patients. Chronic granulomatous inflammation and indurative pancreatitis can sometimes mimic a relapse on CT and PET/CT image tests.
Collapse
Affiliation(s)
- Nadezhda Stefanova
- Department of General and Clinical Pathology, Forensic Medicine and Deontology, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
| | - Turgay Kalinov
- Department of General Surgery, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
| | - Nikola Kolev
- Department of General Surgery, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
| | - Emilian Kalchev
- Department of Diagnostic Imaging and Radiotherapy, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
| |
Collapse
|
3
|
Sharaf Aldeen R, Al Laham O, Ibrahim Basha Z, Zeen Aldeen H, Maged Agha A, Hamed H. Diagnosis and surgical management of a rare neoplastic entity: Solid Pseudopapillary Neoplasm of the Pancreas. Int J Surg Case Rep 2023; 105:108058. [PMID: 36989625 PMCID: PMC10074570 DOI: 10.1016/j.ijscr.2023.108058] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2023] [Revised: 03/21/2023] [Accepted: 03/22/2023] [Indexed: 03/29/2023] Open
Abstract
INTRODUCTION AND IMPORTANCE Solid Pseudopapillary Tumors of the Pancreas is an extremely rare pancreatic neoplastic entity that makes up barely 3 % of all types of exocrine pancreatic neoplasia. Symptoms vary and none of them are specific or pathognomonic for the disease. Therefore, delayed treatment or misdiagnoses could be the result. In turn, patients' morbidity and mortality significantly rise. Diagnosing or suspecting this type of critical type of neoplasia in the preoperative phase is a key component to performing appropriate and curative surgical interventions that result in increased patient survivability. CASE PRESENTATION We hereby present the rare case of a previously healthy 20-year-old female whose chief complaints were chronic epigastric and left hypochondriac region pain and discomfort along with loss of appetite. During our preoperative radiological investigation, we found a cystic mass with well-defined borders located between the head of the pancreas and the second part of the duodenum. It measured (63 × 45 mm). No metastasis or lymph node involvement was elicited. CLINICAL DISCUSSION The tumor was utterly resected via a successful Whipple procedure. A definitive diagnosis of a Solid Pseudopapillary Tumor was reached following meticulous histopathological and immunohistochemical analysis of the resected specimens. CONCLUSION Based on our review of the published literature, no previously published cases from our country of pancreatic Solid Pseudopapillary Tumors exist. Documentation of this rare neoplasia is warranted to raise awareness and to establish the necessary clinical protocols to optimally diagnose, timely treat, and adequately follow up on patients who present with this malignancy.
Collapse
Affiliation(s)
- Rahaf Sharaf Aldeen
- Department of Surgery, Al-Mouwasat University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic; Department of Surgery, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic; Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.
| | - Omar Al Laham
- Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.
| | - Zein Ibrahim Basha
- Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic; Department of Pathology, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic.
| | - Hasan Zeen Aldeen
- Department of Surgery, Al-Mouwasat University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic; Department of Surgery, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic; Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.
| | - Abdulmotaleb Maged Agha
- Department of Surgery, Al-Mouwasat University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic; Department of Surgery, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic; Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.
| | - Hamoud Hamed
- Department of Surgery, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic; Department of Surgery, Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.
| |
Collapse
|
4
|
pTINCR microprotein promotes epithelial differentiation and suppresses tumor growth through CDC42 SUMOylation and activation. Nat Commun 2022; 13:6840. [PMID: 36369429 PMCID: PMC9652315 DOI: 10.1038/s41467-022-34529-6] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2022] [Accepted: 10/27/2022] [Indexed: 11/13/2022] Open
Abstract
The human transcriptome contains thousands of small open reading frames (sORFs) that encode microproteins whose functions remain largely unexplored. Here, we show that TINCR lncRNA encodes pTINCR, an evolutionary conserved ubiquitin-like protein (UBL) expressed in many epithelia and upregulated upon differentiation and under cellular stress. By gain- and loss-of-function studies, we demonstrate that pTINCR is a key inducer of epithelial differentiation in vitro and in vivo. Interestingly, low expression of TINCR associates with worse prognosis in several epithelial cancers, and pTINCR overexpression reduces malignancy in patient-derived xenografts. At the molecular level, pTINCR binds to SUMO through its SUMO interacting motif (SIM) and to CDC42, a Rho-GTPase critical for actin cytoskeleton remodeling and epithelial differentiation. Moreover, pTINCR increases CDC42 SUMOylation and promotes its activation, triggering a pro-differentiation cascade. Our findings suggest that the microproteome is a source of new regulators of cell identity relevant for cancer.
Collapse
|
5
|
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.
Collapse
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.
| |
Collapse
|
6
|
Hirabayashi K, Saika T, Nakamura N. Background features in the cytology of pancreatic neoplasms. DEN OPEN 2022; 2:e105. [PMID: 35873514 PMCID: PMC9302047 DOI: 10.1002/deo2.105] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/29/2021] [Revised: 02/09/2022] [Accepted: 02/13/2022] [Indexed: 11/05/2022]
Abstract
Cytology is a useful method for diagnosing pancreatic neoplasms. Although endoscopic ultrasound‐guided fine‐needle aspiration has recently become the mainstream method for the diagnosis of pancreatic neoplasms, pancreatic juice and pancreatic duct brushing cytology continue to be useful diagnostic methods for the investigation of pancreatic neoplasms. Diagnoses using pancreatic cytology are primarily based on the features related to tumor cells; however, evaluation of the background features provides important information that could further aid the diagnosis. Pancreatic neoplasms show various histological types, each of which is associated with its own characteristic background features. The necrotic background, desmoplastic stroma, and presence of cancer‐associated fibroblasts are background features of pancreatic ductal adenocarcinoma, a mucinous background is associated with intraductal papillary mucinous neoplasms and mucinous cystic neoplasms, and hyaline globules are observed in solid pseudopapillary neoplasms. However, some background features are associated with more than one histological type of pancreatic neoplasm, highlighting the importance to base a diagnosis on the results of a comprehensive analysis of not only the background features but also the tumor cells. Here, we provide a review of the key background cytological features of pancreatic neoplasms, which can serve as a guide to improve diagnosis and research.
Collapse
Affiliation(s)
| | - Tsubasa Saika
- Diagnostic Pathology Center Tokai University Hospital Kanagawa Japan
| | - Naoya Nakamura
- Department of Pathology Tokai University School of Medicine Kanagawa Japan
| |
Collapse
|
7
|
Pseudoendocrine Sarcoma: Clinicopathologic Analysis of 23 Cases of a Distinctive Soft Tissue Neoplasm With Metastatic Potential, Recurrent CTNNB1 Mutations, and a Predilection for Truncal Locations. Am J Surg Pathol 2021; 46:33-43. [PMID: 34081037 DOI: 10.1097/pas.0000000000001751] [Citation(s) in RCA: 19] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
The number of recognized epithelioid soft tissue neoplasms continues to increase and includes epithelioid schwannoma, sclerosing epithelioid fibrosarcoma, and emerging entities such as sarcomas with GLI1 alterations. Here, we describe 23 cases of a previously unrecognized entity, provisionally termed "pseudoendocrine sarcoma." Pseudoendocrine sarcoma is a rare, distinctive tumor of uncertain lineage with a predilection for paravertebral soft tissue in older adults. Fifteen patients (65%) were male and 8 were female. Age at presentation ranged from 29 to 78 years (median: 62 y). Nineteen tumors (83%) occurred in truncal locations, including 15 tumors (65%) in paravertebral soft tissue; other locations included the posterior head (2 tumors), thigh (1), and orbit (1). Tumor size ranged from 2 to 19 cm (median: 6.35 cm). Pseudoendocrine sarcoma is composed of sheets, trabeculae, and nests of epithelioid or ovoid cells with indistinct borders, palely eosinophilic cytoplasm, and highly monomorphic, round nuclei with speckled chromatin. Pseudoglandular architecture was at least focally present in 16 tumors (70%), large extracellular hyaline globules were identified in 12 tumors (52%), and psammomatous calcifications were present in 8 (35%). Metaplastic ossification was identified in 2 tumors, and myxoid stroma was present in 1. Lymphovascular invasion was present in 5 of 18 tumors (28%). Immunohistochemistry demonstrated that most tumors showed nuclear positivity for β-catenin (20/21 tumors; 95%), and some showed at least focal positivity for S-100 (9/22; 41%), desmin (3/8; 38%), or CD34 (2/8; 25%). All tumors were negative for neuroendocrine and epithelial markers, including synaptophysin (21 tumors), chromogranin (19), INSM1 (4), pan-K (16), CAM5.2 (13), AE1/AE3 (6), epithelial membrane antigen (20), and E-cadherin (13). DNA sequencing detected CTNNB1 point mutations in all 6 sequenced tumors: D32H, S33C, S33F, S37A, S37C, and S37F. RNA sequencing was negative for gene fusions in all 6 sequenced tumors. Clinical follow-up was available for 17 patients (74%; range: 4 mo to 20 y; median: 3.5 y), including 14 patients with >1 year of follow-up. Six of 14 patients with long-term follow-up experienced local recurrence (43%, at intervals of 3 to 6 y). One tumor showed a local lymph node metastasis within the primary excision specimen, and 3 patients developed distant lung metastases (21%). No patient died of the disease as yet. Despite its bland morphology and resemblance to the well-differentiated neuroendocrine tumor, pseudoendocrine sarcoma is best considered an intermediate-grade sarcoma, given its pathologic characteristics and clinical behavior.
Collapse
|
8
|
Hayashi T, Kohsaka S, Takamochi K, Kishikawa S, Ikarashi D, Sano K, Hara K, Onagi H, Suehara Y, Takahashi F, Saito T, Nakatsura T, Kitano S, Suzuki K, Yao T. Histological characteristics of lung adenocarcinoma with uncommon actionable alterations: special emphasis on MET exon 14 skipping alterations. Histopathology 2021; 78:987-999. [PMID: 33249657 DOI: 10.1111/his.14311] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2020] [Revised: 02/26/2021] [Accepted: 11/24/2020] [Indexed: 12/18/2022]
Abstract
AIMS In the evolving era of precision medicine, increasing emphasis is placed on detecting molecular alterations driving the development of specific cancers and targeting them with matched therapies that can yield the best outcomes for patients. Lung adenocarcinomas with uncommon actionable alterations, including MET exon 14 skipping (METex14), ERBB2 and BRAF mutations, are rare and poorly characterised cancers. METHODS AND RESULTS To more clearly understand the histopathological features of lung adenocarcinoma with uncommon actionable alterations, we compared the histological features of 678 cases with mitogenic driver alterations from 996 surgically resected lung adenocarcinomas. Genomic data from our cohort revealed METex14, ERBB2 and BRAF mutations in 13, 16 and 15 cases, respectively. Patients who had lung adenocarcinoma with METex14 were often elderly females. Histological features such as clear cell features (23%), hyaline globules (31%) and nuclear pleomorphism (39%) were the most frequently identified in METex14-positive cases; among those, three cases (23%) had tumour cells with bizarre giant or multilobulated nuclei. Additionally, the micropapillary pattern was the most frequently identified in ERBB2-mutated lung adenocarcinoma (31%). Lung adenocarcinoma with BRAF mutations tended to be less invasive, and the BRAF V600E mutation was identified in only one case with lepidic adenocarcinoma. Immunohistochemically, all METex14, ERBB2 and BRAF-positive tumours, except for invasive mucinous adenocarcinoma, were positive for thyroid transcription factor 1 (TTF-1). CONCLUSIONS Our data from Japanese patients showed that lung adenocarcinoma with METex14 had unique clinicopathological characteristics: tumour cells with marked nuclear pleomorphism, hyaline globules and expression of TTF-1 in elderly women who never or lightly smoked.
Collapse
Affiliation(s)
- Takuo Hayashi
- Department of Human Pathology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Shinji Kohsaka
- Division of Cellular Signaling, National Cancer Center Research Institute, Tokyo, Japan
| | - Kazuya Takamochi
- Department of General Thoracic Surgery, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Satsuki Kishikawa
- Department of Human Pathology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Daiki Ikarashi
- Division of Cancer Immunotherapy, Exploratory Oncology Research and Clinical Trial Center, National Cancer Center, Kashiwa, Japan
| | - Kei Sano
- Division of Cancer Immunotherapy, Exploratory Oncology Research and Clinical Trial Center, National Cancer Center, Kashiwa, Japan
| | - Kieko Hara
- Department of Human Pathology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Hiroko Onagi
- Department of Human Pathology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Yoshiyuki Suehara
- Department of Orthopedic Surgery, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Fumiyuki Takahashi
- Department of Respiratory Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Tsuyoshi Saito
- Department of Human Pathology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Tetsuya Nakatsura
- Division of Cancer Immunotherapy, Exploratory Oncology Research and Clinical Trial Center, National Cancer Center, Kashiwa, Japan
| | - Shigehisa Kitano
- Division of Cancer Immunotherapy, Exploratory Oncology Research and Clinical Trial Center, National Cancer Center, Kashiwa, Japan.,Division of Cancer Immunotherapy Development, Advanced Medical Development Center, The Cancer Institute Hospital of JFCR, Tokyo, Japan
| | - Kenji Suzuki
- Department of General Thoracic Surgery, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Takashi Yao
- Department of Human Pathology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| |
Collapse
|
9
|
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.
Collapse
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
| |
Collapse
|
10
|
Lemoine A, Asmandar S, Boutroux H, Tounian P, Ducou Le Pointe H, Coulomb A, Irtan S. Extrapancreatic primary solid pseudopapillary tumor in the gastric antrum: Case report. Pediatr Blood Cancer 2020; 67:e28415. [PMID: 32779872 DOI: 10.1002/pbc.28415] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/15/2020] [Revised: 04/28/2020] [Accepted: 04/29/2020] [Indexed: 01/01/2023]
Affiliation(s)
- Anaïs Lemoine
- Department of Pediatric Nutrition and Gastroenterology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Safaa Asmandar
- Department of Pathological Anatomy and Cytology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Hélène Boutroux
- Department of Pediatric Hematology and Oncology, APHP, Trousseau Hospital, Paris, France
| | - Patrick Tounian
- Department of Pediatric Nutrition and Gastroenterology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Hubert Ducou Le Pointe
- Department of Pediatric Imaging, APHP, Sorbonne University, Trousseau Hospital, Paris, France
| | - Aurore Coulomb
- Department of Pathological Anatomy and Cytology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Sabine Irtan
- Department of Visceral and Neonatal Pediatric Surgery, APHP, Sorbonne University, Trousseau Hospital, Paris, France
| |
Collapse
|
11
|
Wilkinson PE, Merkourea S, Gopalakrishnan R, Argyris PP. Primary Intraosseous Xanthomas of the Jaws: A Series of Six Cases Including an Example with Formation of Apoptosis-Related Hyaline Globules, So-Called "Thanatosomes". Head Neck Pathol 2020; 14:859-868. [PMID: 31916206 PMCID: PMC7669974 DOI: 10.1007/s12105-020-01126-2] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/04/2019] [Accepted: 01/03/2020] [Indexed: 12/13/2022]
Abstract
Primary intraosseous xanthomas of the jaws (PIXJ) are rare and predominantly affect the posterior mandible (86%) of normolipemic patients, with a mean age of 30 years and no gender predilection. Clinically, PIXJ exhibit indolent biologic behavior; curettage is considered treatment of choice. Only 36 PIXJ have been reported. Apoptosis-related hyaline globules (HGs), also known as "thanatosomes", have not been previously reported in PIXJ. Cases diagnosed as xanthoma of bone were retrieved. Six cases fulfilling currently accepted criteria were identified and their clinicopathologic and immunohistochemical properties are presented herein. Mean age for PIXJ was 21.8 years (range = 12-33) and F:M ratio = 2:1. All cases presented as well-demarcated, unilocular or multilocular radiolucencies. Microscopically, PIXJ featured sheets of lipid-laden macrophages with eosinophilic or foamy cytoplasm. A secondary fibroblastic population lacking storiform pattern was evident in two cases. Adipocytes (3/6), peripheral neurovascular bundles (1/6), bone fragments (3/6) and dystrophic calcifications (3/6) were observed enclosed by the xanthoma cells. Notably, one case exhibited numerous, spherical, eosinophilic HGs containing apoptotic nuclei. PIXJ were consistently CD68(+) and negative for CD1α and S100. CD45 decorated lymphocytes and the membrane of foamy histiocytes. Xanthoma cells stained for lysozyme and plasma proteins including alpha-1 antitrypsin (AAT), IgG and IgA in one probed case. HGs were lysozyme(+), AAT(+), IgG(+), IgA(+), PAS(+) and diastase-resistant, and fuchsinophilic with Masson's trichrome. PIXJ represent infrequent, solitary, mandibular lesions with a predilection for the second and third decade of life. Thanatosomes associated with cell injury and death can be present in PIXJ.
Collapse
Affiliation(s)
- Peter E. Wilkinson
- grid.17635.360000000419368657Division of Oral and Maxillofacial Pathology, Department of Diagnostic and Biological Sciences, School of Dentistry, University of Minnesota, 515 Delaware Street SE 16-206B, Minneapolis, MN 55455 USA
| | - Stavroula Merkourea
- grid.5216.00000 0001 2155 0800School of Dentistry, National and Kapodistrian University of Athens, 11527 Athens, Greece
| | - Rajaram Gopalakrishnan
- grid.17635.360000000419368657Division of Oral and Maxillofacial Pathology, Department of Diagnostic and Biological Sciences, School of Dentistry, University of Minnesota, 515 Delaware Street SE 16-206B, Minneapolis, MN 55455 USA
| | - Prokopios P. Argyris
- grid.17635.360000000419368657Division of Oral and Maxillofacial Pathology, Department of Diagnostic and Biological Sciences, School of Dentistry, University of Minnesota, 515 Delaware Street SE 16-206B, Minneapolis, MN 55455 USA ,grid.17635.360000000419368657Department of Biochemistry, Molecular Biology, and Biophysics, College of Biological Sciences, University of Minnesota, Minneapolis, MN 55455 USA ,grid.17635.360000000419368657Masonic Cancer Center, University of Minnesota, Minneapolis, MN 55455 USA ,grid.17635.360000000419368657Institute for Molecular Virology, University of Minnesota, Minneapolis, MN 55455 USA
| |
Collapse
|
12
|
Fibrous Extracellular Spheroids in an Endoscopic Ultrasound-Guided Pancreatic Fine Needle Aspiration Correlating to a Gyriform Pancreatic Endocrine Tumor with a Unique Cobblestone Pavement Growth Pattern. Case Rep Pathol 2019; 2019:1701072. [PMID: 31772804 PMCID: PMC6854169 DOI: 10.1155/2019/1701072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2019] [Accepted: 08/22/2019] [Indexed: 11/17/2022] Open
Abstract
Pancreatic neuroendocrine neoplasms (PanNENs) are uncommon tumors. Fine needle aspiration (FNA) samples from PanNENs are typically of high cellularity and lack necrosis. In cytology slides from these tumors, dyscohesive cells are usually reported with variably round to oval to plasmacytoid forms exhibiting coarsely granular chromatin and showing immunoreactivity for synaptophysin. We present an unusual, and to our knowledge not previously described, example of an FNA of a PanNEN with large extracellular fibrous spheroids containing intrinsic fibroblasts and rimmed by small to intermediate sized neoplastic epithelial cells with high nuclear cytoplasmic ratios. The cytomorphology of the PanNEN in this case was in some ways reminiscent of that expected in adenoid cystic carcinomas of the salivary glands that most often contain large extracellular globules of basement membrane material and a somewhat biphasic population of lesional cells. The cytomorphology in this case was found to correlate well with the resection specimen histomorphology of an exaggerated gyriform pattern of growth resulting in a unique cobblestone-pavement like microscopic appearance. Knowledge of this potential cytomorphology will aid the cytology community through recognition and reporting of this previously undescribed pattern in an uncommon disease.
Collapse
|
13
|
Misra S, Saran RK, Srivastava S, Barman S, Dahale A. Utility of cytomorphology in distinguishing solid pseudopapillary neoplasm of pancreas from pancreatic neuroendocrine tumor with emphasis on nuclear folds and nuclear grooves. Diagn Cytopathol 2019; 47:531-540. [DOI: 10.1002/dc.24145] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2018] [Revised: 12/05/2018] [Accepted: 01/02/2019] [Indexed: 01/17/2023]
Affiliation(s)
- Sunayana Misra
- Department of PathologyGB Pant Institute of Post Graduate Medical Education and Research 1 Jawahar Lal Nehru Marg, New Delhi India
| | - RK Saran
- Department of PathologyGB Pant Institute of Post Graduate Medical Education and Research 1 Jawahar Lal Nehru Marg, New Delhi India
| | - Siddharth Srivastava
- Department of GastroenterologyGB Pant Institute of Post Graduate Medical Education and Research 1 Jawahar Lal Nehru Marg, New Delhi India
| | - Sandip Barman
- Department of PathologyGB Pant Institute of Post Graduate Medical Education and Research 1 Jawahar Lal Nehru Marg, New Delhi India
| | - Amol Dahale
- Department of GastroenterologyGB Pant Institute of Post Graduate Medical Education and Research 1 Jawahar Lal Nehru Marg, New Delhi India
| |
Collapse
|
14
|
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.
Collapse
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
| | | |
Collapse
|
15
|
|
16
|
Luthra AK, Arnold CA, Manilchuk AV, Krishna SG. The spectrum of confocal endomicroscopy findings in a cystic neuroendocrine tumor of the pancreas. Endoscopy 2018; 50:E323-E324. [PMID: 30107632 DOI: 10.1055/a-0667-8978] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Affiliation(s)
- Anjuli K Luthra
- Division of Gastroenterology, Hepatology and Nutrition, Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | | | - Andrei V Manilchuk
- Division of General and Gastrointestinal Surgery, Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Somashekar G Krishna
- Division of Gastroenterology, Hepatology and Nutrition, Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| |
Collapse
|
17
|
Lanke G, Ali FS, Lee JH. Clinical update on the management of pseudopapillary tumor of pancreas. World J Gastrointest Endosc 2018; 10:145-155. [PMID: 30283597 PMCID: PMC6162250 DOI: 10.4253/wjge.v10.i9.145] [Citation(s) in RCA: 38] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/14/2018] [Revised: 04/28/2018] [Accepted: 06/08/2018] [Indexed: 02/06/2023] Open
Abstract
Solid pseudopapillary neoplasm (SPN) is a rare tumor with malignant potential which is generally located in the tail of pancreas. The prevalence of SPN has increased with widespread use of cross sectional imaging. SPN is often misdiagnosed due to nonspecific clinical presentation and accurate diagnosis is essential for optimal management. Endoscopic ultrasound-FNA with immunohistochemistry can help in preoperative diagnosis. Surgery is the treatment of choice and a successful R0 resection is curative. Overall, SPN has a good prognosis. This review article focuses on pathogenesis, diagnosis and management of SPN.
Collapse
Affiliation(s)
- Gandhi Lanke
- Department of Gastroenterology, Hepatology and Nutrition, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Faisal S Ali
- Department of Gastroenterology, Hepatology and Nutrition, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Jeffrey H Lee
- Department of Gastroenterology, Hepatology and Nutrition, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| |
Collapse
|
18
|
Michalova K, Michal M, Kazakov DV, Sedivcova M, Hes O, Hadravsky L, Agaimy A, Tretiakova M, Bacchi C, Hartmann A, Kuroda N, Bulimbasic S, Coric M, Antic T, Michal M. Primary signet ring stromal tumor of the testis: a study of 13 cases indicating their phenotypic and genotypic analogy to pancreatic solid pseudopapillary neoplasm. Hum Pathol 2017; 67:85-93. [DOI: 10.1016/j.humpath.2017.07.010] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2017] [Revised: 06/11/2017] [Accepted: 07/04/2017] [Indexed: 11/25/2022]
|
19
|
Tanigawa M, Nakayama M, Taira T, Hattori S, Mihara Y, Kondo R, Kusano H, Nakamura K, Abe Y, Ishida Y, Okabe Y, Hisaka T, Okuda K, Fujino K, Ito T, Kawahara A, Naito Y, Yamaguchi R, Akiba J, Akagi Y, Yano H. Insulinoma-associated protein 1 (INSM1) is a useful marker for pancreatic neuroendocrine tumor. Med Mol Morphol 2017; 51:32-40. [PMID: 28849340 DOI: 10.1007/s00795-017-0167-6] [Citation(s) in RCA: 63] [Impact Index Per Article: 7.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2017] [Accepted: 08/10/2017] [Indexed: 01/15/2023]
Abstract
Insulinoma-associated protein 1 (INSM1) is an important biomarker of Achaete-scute homolog-like 1-driven pathways. For diagnosis of pancreatic neuroendocrine tumors (PanNET), chromogranin A (CGA), synaptophysin (SYP), and neural cell adhesion molecule (NCAM) were also considered as potential biomarkers. However, it is often difficult to diagnose it immunohistochemically. Hence, we examined the expression pattern of INSM1 in pancreatic solid tumors. We detected INSM1, CGA, SYP, and NCAM immunohistochemically, in 27 cases of NET [pure type: 25 cases, mixed adenoneuroendocrine carcinoma (MANEC): 2 cases]. We included 5 cases of solid-pseudopapillary neoplasm (SPN), 7 cases of acinar cell carcinoma (ACC), and 15 cases of pancreatic ductal adenocarcinoma (PDAC) as the control group. Nuclear expression of INSM1 was found in all PanNET pure type cases. However, expression of INSM1 was negative in PDAC, ACC, and SPN in all cases, whereas faint expression was seen in the cytoplasm from SPN. MANEC comprises of two components: neuroendocrine carcinoma and adenocarcinoma components. The NET component was positive for INSM1 expression, whereas the PDAC component does not express INSM1, which aids in distinguishing these components. Our results suggest that INSM1 is a useful immunohistochemical marker for diagnosing pancreatic neuroendocrine tumor.
Collapse
Affiliation(s)
- Masahiko Tanigawa
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan.,Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Masamichi Nakayama
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan
| | - Tomoki Taira
- Department of Diagnostic Pathology, Kurume University Hospital, Kurume, Japan
| | - Satoshi Hattori
- Biostatistics Center, Kurume University School of Medicine, Kurume, Japan
| | - Yutaro Mihara
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan.,Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Reiichiro Kondo
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan
| | - Hironori Kusano
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan
| | - Ken Nakamura
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan
| | - Yushi Abe
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan
| | - Yusuke Ishida
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
| | - Yoshinobu Okabe
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
| | - Toru Hisaka
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Koji Okuda
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Kosuke Fujino
- Department of Pathology and Experimental Medicine, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Takaaki Ito
- Department of Pathology and Experimental Medicine, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan
| | - Akihiko Kawahara
- Department of Diagnostic Pathology, Kurume University Hospital, Kurume, Japan
| | - Yoshiki Naito
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan. .,Department of Diagnostic Pathology, Kurume University Hospital, Kurume, Japan.
| | - Rin Yamaguchi
- Department of Pathology and Laboratory Medicine, Kurume University Medical Center, Kurume, Japan
| | - Jun Akiba
- Department of Diagnostic Pathology, Kurume University Hospital, Kurume, Japan
| | - Yoshito Akagi
- Department of Surgery, Kurume University School of Medicine, Kurume, Japan
| | - Hirohisa Yano
- Department of Pathology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan
| |
Collapse
|
20
|
Ersen A, Agalar AA, Ozer E, Agalar C, Unek T, Egeli T, Ozbilgin M, Astarcioglu I, Olguner M, Obuz F, Sagol O. Solid-Pseudopapillary neoplasm of the pancreas: A clinicopathological review of 20 cases including rare examples. Pathol Res Pract 2016; 212:1052-1058. [DOI: 10.1016/j.prp.2016.09.006] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/23/2016] [Revised: 08/17/2016] [Accepted: 09/12/2016] [Indexed: 02/07/2023]
|
21
|
Ohara Y, Oda T, Hashimoto S, Akashi Y, Miyamoto R, Enomoto T, Satomi K, Morishita Y, Ohkohchi N. Pancreatic neuroendocrine tumor and solid-pseudopapillary neoplasm: Key immunohistochemical profiles for differential diagnosis. World J Gastroenterol 2016; 22:8596-8604. [PMID: 27784972 PMCID: PMC5064041 DOI: 10.3748/wjg.v22.i38.8596] [Citation(s) in RCA: 49] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2016] [Revised: 07/04/2016] [Accepted: 08/01/2016] [Indexed: 02/06/2023] Open
Abstract
AIM To reveal better diagnostic markers for differentiating neuroendocrine tumor (NET) from solid-pseudopapillary neoplasm (SPN), focusing primarily on immunohistochemical analysis.
METHODS We reviewed 30 pancreatic surgical specimens of NET (24 cases) and SPN (6 cases). We carried out comprehensive immunohistochemical profiling using 9 markers: Synaptophysin, chromogranin A, pan-cytokeratin, E-cadherin, progesterone receptor, vimentin, α-1-antitrypsin, CD10, and β-catenin.
RESULTS E-cadherin staining in NETs, and nuclear labeling of β-catenin in SPNs were the most sensitive and specific markers. Dot-like staining of chromogranin A might indicate the possibility of SPNs rather than NETs. The other six markers were not useful because their expression overlapped widely between NETs and SPNs. Moreover, two cases that had been initially diagnosed as NETs on the basis of their morphological features, demonstrated SPN-like immunohistochemical profiles. Careful diagnosis is crucial as we actually found two confusing cases showing disagreement between the tumor morphology and immunohistochemical profiles.
CONCLUSION E-cadherin, chromogranin A, and β-catenin were the most useful markers which should be employed for differentiating between NET and SPN.
Collapse
|
22
|
Abstract
Nonductal pancreatic neoplasms, including solid pseudopapillary neoplasms, acinar cell carcinomas, and pancreatoblastomas, are uncommon. These entities share overlapping gross, microscopic, and immunohistochemical features, such as well-demarcated solid neoplasms, monotonous cellular tumor cells with little intervening stroma, and abnormal beta-catenin expression. Each tumor also has unique clinicopathologic characteristics with diverse clinical behavior. To differentiate nonductal pancreatic neoplasms, identification of histologic findings, such as pseudopapillae, acinar cell features, and squamoid corpuscles, is important. Immunostainings for acinar cell or neuroendocrine markers are helpful for differential diagnosis. This article describes the clinicopathologic and immunohistochemical features of nonductal pancreatic cancers.
Collapse
|
23
|
Weiss V, Dueber J, Wright JP, Cates J, Revetta F, Parikh AA, Merchant NB, Shi C. Immunohistochemical analysis of the Wnt/β-catenin signaling pathway in pancreatic neuroendocrine neoplasms. World J Gastrointest Oncol 2016; 8:615-622. [PMID: 27574554 PMCID: PMC4980652 DOI: 10.4251/wjgo.v8.i8.615] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2016] [Revised: 05/24/2016] [Accepted: 06/03/2016] [Indexed: 02/05/2023] Open
Abstract
AIM: To investigate the role of the Wnt/β-catenin pathway in pancreatic neuroendocrine neoplasms (PanNENs).
METHODS: Tissue microarrays containing 88 PanNENs were immunohistochemically labeled with antibodies to β-catenin, E-cadherin, adenomatous polyposis coli (APC), chromogranin and synaptophysin. One case had only metastatic tumors resected, whereas others (n = 87) received pancreatectomy with or without partial hepatectomy. Pathology slides, demographic, clinicopathologic, and follow up data were reviewed. Patients’ demographics, clinicopathologic features, and immunohistochemical results from 87 primary tumors were compared between patients with low stage (stage I/II) and high stage (stage III/IV) tumors. In addition, correlation of immunohistochemical results from primary tumors with disease-specific survival (DSS) was evaluated.
RESULTS: Strong membranous β-catenin staining in the primary tumor was observed in all 13 stage III/IV PanNENs as compared to 47% (35/74) of stage I/II tumors (P < 0.01). However, the strong membranous β-catenin staining was unassociated with tumor grade or DSS. Decreased membranous β-catenin staining was associated with decreased membranous E-cadherin labeling. Nuclear β-catenin staining was seen in 15% (2/13) of stage III/IV PanNENs as compared to 0% (0/74) of stage I/II tumors (P = 0.02). The case with metastasectomy also only showed nuclear β-catenin staining. Two of the three cases with nuclear β-catenin staining were familial adenomatous polyposis (FAP) patients. Lack of APC expression was seen in 70% (57/81) of the cases, including the 3 cases with nuclear β-catenin staining. Expression of E-cadherin and APC in primary tumor was not correlated with tumor grade, tumor stage, or disease specific survival.
CONCLUSION: The Wnt/β-catenin pathway was altered in some PanNENs, but did not Impact DSS. PanNENs in FAP patients demonstrated nuclear β-catenin accumulation and loss of APC.
Collapse
|
24
|
Pancreatic analogue solid pseudopapillary neoplasm arising in the paratesticular location. The first case report. Hum Pathol 2016; 56:52-6. [PMID: 27342913 DOI: 10.1016/j.humpath.2016.06.007] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2016] [Revised: 04/27/2016] [Accepted: 06/03/2016] [Indexed: 11/22/2022]
Abstract
We describe the first pancreatic analogue of solid pseudopapillary neoplasm arising in paratesticular location. It was a tumor arising in 32-year-old man adhering closely to the testis. The tumor had several morphologic components. The greatest was represented by signet ring cells which gradually changed into solid, non-signet ring cell areas, often being mixed together. It also formed distinct trabeculae and pseudopapillae frequently adhering to cystic areas of the tumor. Immunohistochemically, the tumor had an identical profile to its pancreatic counterpart. The tumor cells reacted diffusely with S100 protein, β-catenin, cyclin D1, Fli-1, vimentin, CD10, galectin-3, and neuron-specific enolase and focally with synaptophysin. CD56 and E-cadherin reacted only in those parts of the tumor, which formed pseudopapillae. Cytokeratin antibody AE1-AE3 was strongly positive in the areas of trabecular formation of the tumor. The mutational analysis of exon 3 of the CTNNB1 gene confirmed mutation in this exon.
Collapse
|
25
|
Abstract
Cancer is caused by the accumulation of inherited and/or acquired alterations in specific genes. The recent decline in the cost of DNA sequencing has allowed tumor sequencing to be conducted on a large scale, which, in turn, has led to an unprecedented understanding of the genetic events that drive neoplasia. This understanding, when integrated with meticulous histologic analyses and with clinical findings, has direct clinical implications. The recent sequencing of all of the major types of cystic and noncystic neoplasms of the pancreas has revealed opportunities for molecular diagnoses and for personalized treatment. This review summarizes the results from these recent studies focusing on the clinical relevance of genomic data.
Collapse
|
26
|
Rishi A, Goggins M, Wood LD, Hruban RH. Pathological and molecular evaluation of pancreatic neoplasms. Semin Oncol 2014; 42:28-39. [PMID: 25726050 DOI: 10.1053/j.seminoncol.2014.12.004] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Pancreatic neoplasms are morphologically and genetically heterogeneous and include a wide variety of tumors ranging from benign to malignant with an extremely poor clinical outcome. Our understanding of these pancreatic neoplasms has improved significantly with recent advances in cancer sequencing. Awareness of molecular pathogenesis brings new opportunities for early detection, improved prognostication, and personalized gene-specific therapies. Here we review the pathological classification of pancreatic neoplasms from the molecular and genetic perspectives.
Collapse
Affiliation(s)
- Arvind Rishi
- Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD
| | - Michael Goggins
- Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Oncology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD
| | - Laura D Wood
- Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Oncology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD
| | - Ralph H Hruban
- Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Oncology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD.
| |
Collapse
|
27
|
Abstract
Solid-pseudopapillary neoplasm of the pancreas (SPN) is an uncommon low-grade malignant neoplasm occurring mostly in young women. In addition to its distinctive pathological appearance of pseudopapillae with poorly cohesive neoplastic cells, rare variants exist raising the differential diagnosis especially with neuroendocrine neoplasms. The overall prognosis for patients with SPNs is excellent after surgical resection. Nevertheless, 10% of cases may have malignant behavior characterized by tumor recurrence and/or metastasis. Despite numerous studies, the histogenesis of this neoplasm remains unclear. Distinctive molecular alterations such as the presence of CTNNB1 mutations are observed in nearly all cases, while mutations classically observed in ductal adenocarcinoma, such as KRAS, TP53, and SMAD4, are not observed in SPNs, reinforcing its distinct nature compared to all other pancreatic neoplasms. Recent transcriptional studies have shown that activation of the Wnt/beta-catenin pathway in these tumors is associated with the upregulation of genes belonging to Notch, Hedgehog, and androgen receptor signaling pathways.
Collapse
Affiliation(s)
- Benoît Terris
- Service de Pathologie, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Hôpitaux universitaires Paris-Centre, Site Cochin, Paris, France.
| | | |
Collapse
|
28
|
Abstract
In the past decade, the clinico-pathologic characteristics of neuroendocrine tumors (NETs) in the pancreas have been further elucidated. Previously termed "islet cell tumors/carcinomas" or "endocrine neoplasms", they are now called pancreatic NETs (PanNETs). They occur in relatively younger patients and may arise anywhere in the pancreas. Some are associated with von Hippel-Lindau, MEN1, and other syndromes. It is now widely recognized that, with the exception of tumorlets (minute incipient neoplasms) that occur in some syndromes like MEN1, all PanNETs are malignant, albeit low-grade, and although they have a protracted clinical course and overall 10-year survival of 60-70 %, even low-stage and low-grade examples may recur and/or metastasize on long-term follow-up. Per recent consensus guidelines adopted by both European and North American NET Societies (ENETS and NANETs) and WHO-2010, PanNETs are now graded and staged separately, unlike previous classification schemes that used a combination of grade, stage, and adjunct prognosticators in an attempt to define "benign behavior" or "malignant" categories. For staging, the ENETs proposal may be more applicable than CAP/AJCC, which is based on the staging of exocrine tumors. Current grading of PanNETs is based on mitotic activity and ki-67 index. Other promising prognosticators such as necrosis, CK19, c-kit, and others are still under investigation. It has also been recognized that PanNETs have a rather wide morphologic repertoire including oncocytic, pleomorphic, ductulo-insular, sclerosing, and lipid-rich variants. Most PanNETs are diagnosed by fine needle aspiration biopsy, in which single, monotonous plasmacytoid cells with fair amounts of cytoplasm and distinctive neuroendocrine chromatin are diagnostic. Molecular alterations of PanNETs are also very different than that of ductal or acinar tumors. Loss of expression of DAXX and ATRX proteins has been recently identified in 45 %. Along with these improvements, several controversies remain, including grading, value of current cutoff ranges, and the best methods for counting ki-67 index (manual count by computer-captured image may be the most practical for the time being). More important is the controversial use of the term "carcinoma", which was previously employed in WHO-2004 only for invasive and metastatic cases but has now been made synonymous with grade 3 group of tumors. It is becoming clear that grade 3 group comprises two distinct categories: (1) differentiated but proliferatively more active tumors which typically have ki-67 indices in the 20-50 % range and (2) true poorly differentiated NE carcinomas as defined in the lung, with ki-67 typically >50 %. Further studies are needed to address these controversial aspects of PanNETs.
Collapse
Affiliation(s)
- Michelle D Reid
- Department of Pathology, Emory University School of Medicine, Atlanta, GA, USA
| | | | | | | |
Collapse
|
29
|
Shen Y, Wang Z, Zhu J, Chen Y, Gu W, Liu Q. α-Methylacyl-CoA racemase (P504S) is a useful marker for the differential diagnosis of solid pseudopapillary neoplasm of the pancreas. Ann Diagn Pathol 2014; 18:146-50. [PMID: 24675392 DOI: 10.1016/j.anndiagpath.2014.02.006] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/03/2013] [Revised: 01/25/2014] [Accepted: 02/18/2014] [Indexed: 12/11/2022]
Abstract
The differential diagnosis of solid pseudopapillary neoplasm (SPN) from some other nonductal pancreatic tumors may be difficult because of similarities in morphological features. Therefore, immunohistochemical staining is frequently necessary. α-Methylacyl-CoA racemase (AMACR) is a diagnostically useful marker for prostatic cancer and papillary renal cell carcinoma. The aim of this study was to investigate AMACR as a new immunohistochemical marker to differentiate SPNs from other nonductal pancreatic tumors. We investigated immunohistochemical staining for AMACR in 26 SPNs, 21 pancreatic neuroendocrine tumors, and 7 acinar cell carcinomas. All cases of SPN showed granular cytoplasmic expression of AMACR, whereas all cases of pancreatic neuroendocrine tumors and acinar cell carcinomas were negative for this immunohistochemical marker. Hence, our findings demonstrate for the first time that AMACR is a useful immunohistochemical marker for the differential diagnosis of SPNs.
Collapse
Affiliation(s)
- Yanying Shen
- Department of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
| | - Zhaoliang Wang
- Department of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
| | - Jianshan Zhu
- Department of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
| | - Yiming Chen
- Department of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
| | - Wanqing Gu
- Department of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
| | - Qiang Liu
- Department of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China.
| |
Collapse
|
30
|
Kim SA, Kim MS, Kim MS, Kim SC, Choi J, Yu E, Hong SM. Pleomorphic solid pseudopapillary neoplasm of the pancreas: degenerative change rather than high-grade malignant potential. Hum Pathol 2014; 45:166-74. [PMID: 24321526 DOI: 10.1016/j.humpath.2013.08.016] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2013] [Revised: 08/17/2013] [Accepted: 08/21/2013] [Indexed: 10/25/2022]
Abstract
Solid pseudopapillary neoplasms (SPNs) are rare tumors of the pancreas characterized by poorly cohesive uniform cells with solid and pseudopapillary growth patterns. Nuclear pleomorphism is not a well-recognized feature of SPNs and may complicate differentiation from other pancreatic neoplasms. We compared histologic, immunohistochemical, and clinical features of 18 pleomorphic SPNs with 121 conventional SPNs. The prevalence of pleomorphic SPN was 12.9% (18/139). Pleomorphic SPNs arose in older patients (median, 45 years versus 32 years; P < .001), but no differences were found in sex, tumor location, recurrence, and metastasis when compared with conventional SPNs. Except for pleomorphic nuclei, other cytologic and histologic features of pleomorphic SPNs, such as growth pattern, tumor size, infiltrative pattern, tumor extension, mitosis, and Ki-67 labeling index, were not different from those of conventional SPNs. Pleomorphic SPNs showed a significantly higher p53 protein expression (64.7% [11/17 cases]) than that of conventional SPNs (1.8% [2/112 cases], P < .001). However, immunoreactivity for β-catenin and E-cadherin was not different between pleomorphic and conventional SPNs. A TP53 gene mutation was observed in 2 of 3 p53-immunoreactive pleomorphic SPNs. In summary, nuclear pleomorphism occurs in a subset of SPNs. They are more often p53 immunoreactive than SPNs without pleomorphism, and some harbor TP53 gene mutations. However, pleomorphic SPNs do not appear to be more aggressive than conventional SPNs. Low mitotic rate and Ki-67 labeling index may suggest nuclear pleomorphisms as degenerative changes. Recognition of typical poorly cohesive tumor cells and immunohistochemical features could establish the correct diagnosis of SPNs.
Collapse
Affiliation(s)
- Sun A Kim
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 138-736, Korea
| | | | | | | | | | | | | |
Collapse
|
31
|
Bhatnagar R, Olson MT, Fishman EK, Hruban RH, Lennon AM, Ali SZ. Solid-pseudopapillary neoplasm of the pancreas: cytomorphologic findings and literature review. Acta Cytol 2014; 58:347-55. [PMID: 24969629 DOI: 10.1159/000363546] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2014] [Accepted: 05/08/2014] [Indexed: 01/17/2023]
Abstract
BACKGROUND Solid-pseudopapillary neoplasm (SPN) is a rare pancreatic malignancy with an excellent prognosis. It is most commonly diagnosed in young women. This article comprehensively reviews the clinical, pathological and radiological features of this neoplasm, as well as its clinical management. METHODS A literature review of SPN was performed of all articles published in the English language in PubMed prior to November 1, 2013. Cytomorphological features, histopathology, immunohistochemistry, patient general demographics, molecular studies, radiologic imaging and clinical management were reviewed. RESULTS SPN displays distinct cytomorphological features on fine-needle aspiration - thin, delicate, branching vessels in a 'Chinese character' pattern lined by one to several layers of loosely cohesive neoplastic cells. Nuclear features include indented or grooved nuclei with an evenly distributed chromatin pattern and small inconspicuous nucleoli. SPN is characteristically immunoreactive for CD10, β-catenin (in an abnormal nuclear pattern), CD99 in a perinuclear dot-like pattern, α1-antitrypsin, and progesterone receptor. Almost all SPNs harbor an activating point mutation in exon 3 of the β-catenin gene (CTNNB1). Clinicopathological features generally do not correlate with prognosis, and most patients experience excellent long-term survival. CONCLUSIONS SPN can mimic other neoplasms of the pancreas, which can lead to diagnostic challenges in a limited cytologic specimen. Distinct cytomorphological features can help distinguish SPNs from other pancreatic neoplasms. Complete surgical resection as well as resection of metastatic disease is preferred given a low rate of tumor recurrence and long periods of disease-free intervals.
Collapse
Affiliation(s)
- Ramneesh Bhatnagar
- Department of Pathology, The Sol Goldman Pancreatic Cancer Research Center, The Johns Hopkins University School of Medicine, Baltimore, Md., USA
| | | | | | | | | | | |
Collapse
|
32
|
Abstract
Cancer is fundamentally a genetic disease caused by the accumulation of somatic mutations in oncogenes and tumor suppressor genes. In the last decade, rapid advances in sequencing and bioinformatic technology led to an explosion in sequencing studies of cancer genomes, greatly expanding our knowledge of the genetic changes underlying a variety of tumor types. Several of these studies of cancer genomes have focused on pancreatic neoplasms, and cancers from the pancreas are some of the best characterized tumors at the genetic level. Pancreatic neoplasms encompass a wide array of clinical diseases, from benign cysts to deadly cancers, and the genetic alterations underlying neoplasms of the pancreas are similarly diverse. This new knowledge of pancreatic cancer genomes has deepened our understanding of tumorigenesis in the pancreas and has opened several promising new avenues for novel diagnostics and therapeutics.
Collapse
|
33
|
Gozalo AS, Zerfas PM, Starost MF, Lambert LE, Elkins WR. Pancreatic endocrine tumour with disseminated pulmonary thromboembolism in an owl monkey (Aotus nancymae). J Comp Pathol 2013; 149:132-6. [PMID: 23453490 PMCID: PMC3674181 DOI: 10.1016/j.jcpa.2012.11.235] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2011] [Revised: 09/27/2012] [Accepted: 11/17/2012] [Indexed: 11/25/2022]
Abstract
Pulmonary thromboembolism associated with pancreatic endocrine neoplasia is extremely uncommon in man and animals. Post-mortem examination of an adult owl monkey (Aotus nancymae) revealed extensive pulmonary arterial thromboembolism and a well-demarcated mass attached to the pancreas. Microscopically, the mass consisted of areas of interstitial fibrosis with loss of acini and islets and replacement by nests and sheets of polygonal cells with amphophilic cytoplasm, an eccentric round nucleus with stippled chromatin and, in some cells, with a single prominent eccentric nucleolus. Clusters of these cells were noted within vessels and adjacent lymph nodes. The cells did not express S100 or insulin, but were labelled strongly with SP-1/chromogranin. Rare individual cells expressed glucagon and somatostatin. A few cells in pulmonary thrombi/emboli and the adjacent lymph node also expressed SP-1/chromogranin. Based on cell morphology, location and immunohistochemistry the tumour was classified as pancreatic endocrine (islet cell) carcinoma with metastasis to regional lymph nodes and lung.
Collapse
Affiliation(s)
- A S Gozalo
- Comparative Medicine Branch, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
| | | | | | | | | |
Collapse
|
34
|
Abstract
Pancreatic neuroendocrine tumors (PanNETs) are typically solid neoplasms but in rare instances may present as cystic lesions. This unusual presentation can make clinical diagnosis challenging. In addition, the clinical and histopathologic characteristics of cystic PanNETs are poorly defined. We identified 53 cystic PanNETs in our single-institution experience of 491 surgically resected PanNETs. Similar to solid PanNETs, cystic PanNETs developed with an equal sex distribution and over a wide age range (23 to 91 y; mean, 52 y). The unusual cystic appearance made radiologic differentiation from other cystic pancreatic neoplasms difficult with a misdiagnosis in 23 of 53 (43%) cases. An association between cystic PanNETs and multiple endocrine neoplasia type 1 or multifocal disease [5 of 53 (9%) and 7 of 53 (13%), respectively] was not observed as compared with solid PanNETs (P=0.34 and P=0.31, respectively). Grossly, cystic PanNETs were predominantly located in the tail of the pancreas (n=28, 53%) and were similar in size (mean, 3.3 cm) to solid PanNETs (mean, 4.1 cm; P=0.12). All cysts were unilocular (n=53, 100%) and filled with clear to straw-colored fluid. Larger cysts were sometimes noted to be hemorrhagic. Histologically, the cysts were lined by a thin fibrous band that separated the cyst from the neoplastic cells. In comparison with their solid counterparts, cystic PanNETs were less likely to demonstrate tumor necrosis (6%; P=0.04), perineural invasion (8%; P<0.001), vascular invasion (4%; P<0.001), regional lymph node metastasis (13%; P<0.001), and synchronous distant metastasis (4%; P=0.015). The neoplastic cells of the cystic PanNETs were well differentiated (n=53, 100%) with a low mitotic rate and low Ki-67 proliferation index (range, 0.2% to 11%; mean, 1.8%). On the basis of both the American Joint Cancer Committee and European Neuroendocrine Tumor Society staging systems, the majority of cystic PanNETs presented at a lower pathologic stage as compared with solid PanNETs. In summary, cystic PanNETs are a distinctive subgroup of PanNETs with unique clinical, radiographic, and pathologic features.
Collapse
|
35
|
Cai H, Zhou M, Hu Y, He H, Chen J, Tian W, Deng Y. Solid-pseudopapillary neoplasms of the pancreas: clinical and pathological features of 33 cases. Surg Today 2012; 43:148-54. [PMID: 22825652 DOI: 10.1007/s00595-012-0260-3] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2011] [Accepted: 12/15/2011] [Indexed: 02/06/2023]
Abstract
PURPOSE Solid-pseudopapillary neoplasms (SPNs) are rare pancreatic tumors, with a low potential for malignancy. The clinical and pathological features of 33 SPNs were reviewed. METHODS This study conducted a retrospective analysis of 33 patients who underwent surgery for a pathologically confirmed SPN from 2000 to 2011. RESULTS Thirty of the 33 patients (91 %) were female, and the median age at diagnosis was 29.2 years (range 12-59). The most common symptom was abdominal discomfort with dull pain (58 %). Others included asymptomatic lesions that were only detected incidentally during imaging (21 %), a palpable abdominal mass (15 %) and indigestion (6 %). All 33 patients underwent surgery with a curative intent and 3 (9 %) underwent laparoscopic surgery. The mean diameter of the tumors was 4.9 cm (range 2-15 cm), and they occurred in the head (9, 27 %), neck (5, 15 %), body or tail (19, 58 %) of the pancreas. One patient had lymph node metastases, one patient had portal venous invasion and 8 patients had perineural invasion. The patient follow-up ranged from 4 to 118 months, and 32 patients were alive and well without recurrence. One patient relapsed 10 months after distal pancreatectomy with splenectomy and underwent a second surgery via laparotomy. Unfortunately, the patient died of multiple organ failure 12 days after the second surgery. CONCLUSION SPNs are rare neoplasms with malignant potential but excellent prognosis. Adequate surgical resection, including laparoscopic surgery, may therefore be performed safely and is associated with a long-term survival, even in invasive cases.
Collapse
Affiliation(s)
- Hongke Cai
- Department of Surgical Oncology, Second Affiliated Hospital, Zhejiang University College of Medicine, No. 88 Jiefang Road, Zhejiang, People's Republic of China
| | | | | | | | | | | | | |
Collapse
|
36
|
Samad A, Pambuccian SE. Intracellular and extracellular hyaline globules in endobronchial ultrasound-guided transbronchial needle aspiration of mediastinal lymph node: A clue for the diagnosis of metastatic hepatocellular carcinoma. Diagn Cytopathol 2012; 41:799-802. [DOI: 10.1002/dc.22849] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2011] [Revised: 11/01/2011] [Accepted: 01/23/2012] [Indexed: 12/18/2022]
Affiliation(s)
- Arbaz Samad
- Department of Laboratory Medicine and Pathology; University of Minnesota Medical School; Minneapolis; MN
| | - Stefan E. Pambuccian
- Department of Laboratory Medicine and Pathology; University of Minnesota Medical School; Minneapolis; MN
| |
Collapse
|
37
|
Schneider NI, Bauernhofer T, Schöllnast H, Ott A, Langner C. Pancreatic adenocarcinoma with multiple eosinophilic extracellular deposits consistent with noncalcified psammoma bodies. Virchows Arch 2011; 459:623-5. [PMID: 22037658 DOI: 10.1007/s00428-011-1159-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2011] [Accepted: 10/12/2011] [Indexed: 11/29/2022]
|