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Lampridis S. Unraveling the landscape of pediatric pancreatic tumors: Insights from Japan. World J Gastrointest Oncol 2025; 17:101477. [PMID: 39817131 PMCID: PMC11664617 DOI: 10.4251/wjgo.v17.i1.101477] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/15/2024] [Revised: 10/03/2024] [Accepted: 10/18/2024] [Indexed: 12/12/2024] Open
Abstract
Pediatric pancreatic tumors, though rare, pose significant diagnostic and management challenges. The recent, 22-year nationwide survey on pediatric pancreatic tumors in Japan by Makita et al offers valuable insights into this uncommon entity, revealing striking geographical variations and questioning current treatment paradigms. This editorial commentary analyzes the study's key findings, including the predominance of solid pseudopapillary neoplasms and their younger age of onset, which contrast sharply with Western data. It explores the implications for clinical practice and research, emphasizing the need for population-specific approaches to diagnosis and treatment. The revealed limited institutional experience and surgical management patterns prompt a reevaluation of optimal care delivery for these complex cases, suggesting benefits of centralizing healthcare services. Furthermore, the commentary advocates for international collaborative studies to elucidate the genetic, environmental, and lifestyle factors influencing the development and progression of pediatric pancreatic tumors across diverse populations. It also outlines future directions, calling for advancements in precision medicine and innovative care delivery models to improve global patient outcomes. Unraveling Makita et al's findings within the broader landscape of pediatric oncology can stimulate further research and clinical advancements in managing pancreatic and other rare tumors in children.
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Affiliation(s)
- Savvas Lampridis
- Faculty of Medicine, Imperial College London, London SW7 2AZ, United Kingdom
- Department of Surgical Oncology, 424 General Military Training Hospital, Thessaloniki 56429, Greece
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Chen L, Yuan M, Wang M, Luo C, Gao M, Wan Y, Zhou Z. Comparison between pancreatoblastoma (PB) and solid pseudopapillary neoplasm (SPN) in pediatric patients with enhanced CT. Pancreatology 2024; 24:1152-1159. [PMID: 39299885 DOI: 10.1016/j.pan.2024.09.014] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2024] [Revised: 07/26/2024] [Accepted: 09/12/2024] [Indexed: 09/22/2024]
Abstract
RATIONALE AND OBJECTIVES To investigate whether computed tomography features can differentiate pancreatoblastoma (PB) from solid pseudopapillary tumor (SPN) in children. MATERIALS AND METHODS Clinical and imaging data of 18 cases of PB and 61 cases of SPN confirmed by surgery or biopsy were retrospectively analyzed. All enrolled patients underwent 3 phases (non-contrast, arterial, and portal venous phases) of CT scanning. Qualitative CT analysis (location, margin, solid/cystic component proportion, calcification, hemorrhage, peritumoral vascularity, bile duct dilatation, pancreatic duct dilatation, pancreatic atrophy, vascular invasion, peripancreatic invasion, and distant metastases) and quantitative analysis (maximum tumor diameter, interface between tumor and parenchyma [delta], arterial enhancement ratio [AER], and portal enhancement ratio [PER]) were performed. The general CT morphologic features, age and tumor markers were compared also compared between the groups. Univariate analysis and the F test were conducted to identify features of PB. Then logistic Regression classifier was trained using the top five features with the highest F-value. Moreover, we used 5-fold cross-validation techniques for the validation of our model. RESULTS PB exhibited a significantly higher frequency of location in the body/tail, larger tumor size, poorly defined margins, calcification, peritumoral vascularity, pancreatic atrophy, and less hemorrhage. In addition, PB had higher AER, PER and lower delta relative to SPN (p < 0.05). PB presented a younger age and higher levels of AFP. Results of the F test indicated that AFP, AER, Age, calcification and pancreatic atrophy were the top five features included in the model that could differentiate pediatric PB from SPN. The combined model of CT and clinical features performed well in differentiating PB from SPN, with an AUC of 0.981 in the training cohort and 0.953 in the validation cohort. CONCLUSIONS AFP, AER, age, calcification and pancreatic atrophy are robust CT and clinical features for differentiating pediatric PB from SPN. A combination of qualitative and quantitative CT features may provide good diagnostic accuracy in differentiating PB from SPN in children.
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Affiliation(s)
- Lin Chen
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Mengchen Yuan
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Meng Wang
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Chenglong Luo
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Mengyu Gao
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yamin Wan
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Zhigang Zhou
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
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McRae JJLH, Thobani H, Sacks MA, Raymond SL, Shah A, Radulescu A, Khan FA. Pancreatic masses in children: a single-center experience over two decades. Eur J Pediatr 2024; 183:4467-4476. [PMID: 39145888 DOI: 10.1007/s00431-024-05731-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2024] [Revised: 07/31/2024] [Accepted: 08/11/2024] [Indexed: 08/16/2024]
Abstract
Pancreatic masses are extremely rare in pediatric patients, with limited data available. This lack of data makes the diagnosis and management of these tumors in children extremely challenging. Therefore, we aimed to describe the presentations, clinical course, and outcomes of children with pancreatic tumors at our center. A retrospective analysis was performed of all pediatric patients diagnosed with pancreatic masses between 2003 and 2022 in an academic freestanding children's hospital. Data including demographics, clinical presentation, workup, management, and subsequent morbidity and mortality were collected and aggregated. Furthermore, we reviewed cases of pancreatic tumor resections in the National Surgical Quality Improvement Program - Pediatric (NSQIP-P) database to identify common adverse outcomes and measures for quality improvement. In total, 17 patients were identified at our institution. Diagnoses included solid pseudopapillary (n = 9), gastrinoma (n = 1), rhabdomyosarcoma (n = 2), pancreatoblastoma (n = 2), and insulinoma (n = 1). Two patients did not have a histopathologic diagnosis and were excluded from subsequent analysis. Overall, 12 patients underwent surgical intervention, with the most common procedures being pancreaticoduodenectomy and distal pancreatectomy, and all 12 were known to be alive at last contact. There were 3 deaths, all due to complications related to metastatic disease. Furthermore, 30-day postoperative outcomes in the NSQIP-P dataset for pancreatic surgeries in pediatric patients are excellent, with negligible morbidity and no mortalities after the index surgery. CONCLUSIONS Children with pancreatic tumors amenable to surgical resection appear to have adequate long-term survival. Short-term outcomes at diagnosis are excellent and mainly appear to be influenced by the presence of metastatic disease at initial presentation. WHAT IS KNOWN • Pancreatic masses are a rare entity in children with limited data on their presentation, management and surgical outcomes. • Solid Pseudopapillary tumors are one of the most common pancreatic tumors in children with a fair prognosis after surgical intervention. WHAT IS NEW • Surgical management of pediatric patients with pancreatic tumors is safe and effective in patients who do not have aggressive tumor types or metastatic disease. • Our case series provides a notable cohort of these pancreatic tumors with insight into the presentation, management and outcomes of five of these tumor types.
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Affiliation(s)
- Joyce J L H McRae
- Division of Pediatric Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, USA
| | - Humza Thobani
- Division of Pediatric General Surgery, Stanford University, Palo Alto, CA, USA
| | - Marla A Sacks
- Department of General Surgery, SUNY Downstate Medical Center, Brooklyn, NY, USA
| | - Steven L Raymond
- Division of Pediatric Surgery, University of Florida, Gainesville, FL, USA
| | - Adil Shah
- Division of Pediatric Surgery, Children's Nebraska, Omaha, NE, USA
| | - Andrei Radulescu
- Division of Pediatric Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, USA
| | - Faraz A Khan
- Division of Pediatric General Surgery, Stanford University, Palo Alto, CA, USA.
- Stanford Medicine Children's Health Division of Pediatric Surgery, 453 Quarry Road, Palo Alto, CA, 94304, USA.
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4
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Hasan HA, Johnstone LS, Benedetti DJ. A case of pancreatoblastoma in a child with Simpson-Golabi-Behmel syndrome: Highlighting the importance of alpha fetoprotein monitoring. Pediatr Blood Cancer 2024; 71:e31097. [PMID: 38773720 DOI: 10.1002/pbc.31097] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/30/2024] [Revised: 05/04/2024] [Accepted: 05/12/2024] [Indexed: 05/24/2024]
Affiliation(s)
- Hira A Hasan
- Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA
| | - Lindsey S Johnstone
- Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, Nashville, Tennessee, USA
| | - Daniel J Benedetti
- Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA
- Division of Pediatric Hematology Oncology, Vanderbilt University Medical Center, Nashville, Tennessee, USA
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Kahraman G, Arslan-Kahraman DI. Optimizing Preoperative Strategies for Occult Metastasis Detection in PDAC: Integrating Multimodal Imaging and Pathological Correlation for Improved Patient Outcomes. Acad Radiol 2024; 31:2188. [PMID: 38290890 DOI: 10.1016/j.acra.2024.01.020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2024] [Revised: 01/10/2024] [Accepted: 01/11/2024] [Indexed: 02/01/2024]
Affiliation(s)
- Gökhan Kahraman
- Department of Radiology, Ministry of Health Suluova State Hospital, Amasya, Turkey (G.K.).
| | - Dilara Irem Arslan-Kahraman
- Department of Pathology, Ministry of Health Merzifon Kara Mustafa Pasa State Hospital, Amasya, Turkey (D.I.A.K.)
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Venkatakrishna SSB, Ghosh A, Sharma P, Miranda-Schaeubinger M, Prakash AV, Addisu L, Sze RW, Andronikou S. Frequency and classification of addenda in paediatric neuroradiological reports as part of quality assurance. Clin Radiol 2024; 79:303-311. [PMID: 38310059 DOI: 10.1016/j.crad.2023.12.025] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2023] [Revised: 11/29/2023] [Accepted: 12/31/2023] [Indexed: 02/05/2024]
Abstract
AIM To determine the frequency and classification of addenda seen in paediatric brain magnetic resonance imaging (MRI) reports. MATERIALS AND METHODS A retrospective review of the addenda of brain MRI reports from a large tertiary children's hospital was undertaken between January 2013 to December 2021 and a subset of above radiology reports was used to classify addenda over 6-month periods, October to March, spanning 2018 to 2021. A radiology fellow and a medical doctor classified the addenda into previously published categories using their best judgement. RESULTS Out of 73,643 brain MRI reports over 9 years (108 months) included in the study, only 923 reports (1.25%) had addenda. There was a total of 13,615 brain MRI reports from 6-month periods, of which only 179 reports (1.31%) had an addendum. The number of errors according to categories were: observational 88/13,615 (0.65%); interpretational 16/13,615 (0.12%); non-observational and non-interpretative 82/13,615 (0.6%). Notifications to referring physician made in 29/13,615 (0.21%). CONCLUSIONS The overall proportion of addenda to the brain MRI reports of children in the present study was low, at 1.25%. Categorisation of different addenda revealed the most common errors to be observational in 0.65%, including under-reading in the region of interest in 0.25%. Appropriate measures can now be introduced to minimise the error-based addenda further and improve MRI diagnosis in children. Other paediatric practices may choose to follow suit in evaluating their addenda and errors to improve practice.
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Affiliation(s)
- S S B Venkatakrishna
- Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA
| | - A Ghosh
- Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
| | - P Sharma
- Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA
| | - M Miranda-Schaeubinger
- Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA
| | - A V Prakash
- Department of Internal Medicine, St Vincent Hospital, Worcester, MA, USA
| | - L Addisu
- Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA
| | - R W Sze
- Department of Radiology, University of California San Francisco, Benioff Children's Hospital Oakland, Oakland, CA, USA
| | - S Andronikou
- Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA; Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Blvd, Philadelphia, PA 19104, USA
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7
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Remo A, Negro S, Bao RQ, d’Angelo E, Alaggio R, Crivellari G, Mammi I, Intini R, Bergamo F, Fassan M, Agostini M, Vitellaro M, Pucciarelli S, Urso EDL. Association between Pancreatoblastoma and Familial Adenomatous Polyposis: Review of the Literature with an Additional Case. Genes (Basel) 2023; 15:44. [PMID: 38254934 PMCID: PMC10815143 DOI: 10.3390/genes15010044] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2023] [Revised: 12/24/2023] [Accepted: 12/26/2023] [Indexed: 01/24/2024] Open
Abstract
BACKGROUND Adult pancreatoblastoma (PBL) is a rare pancreatic malignancy, with recent evidence suggesting a possible link to familial adenomatous polyposis (FAP). This study aims to review the latest evidence and explore a possible association between adult PBL and FAP. METHODS Two independent literature reviews were conducted: (1) on PBL and FAP, and (2) on PBL in the adult population not diagnosed with FAP. RESULTS Out of 26 articles on PBL and FAP screened, 5 were selected for systematic review, including 1 additional case. We identified eight FAP-related PBL cases, with a median age of 40 (IQR: 34-50). Of these, seven (87%) occurred in adults. We found 65 cases of adult PBL not FAP-related; thus, 7 out of 65 cases (10.7%) of adult PBL reported in the literature are associated with a clinical diagnosis of FAP or were carriers of APC germline pathogenic variants (GPVs). CONCLUSION Data suggest a non-random association between adult PBL and FAP. Further research is essential to optimise surveillance protocols and develop more effective treatment strategies.
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Affiliation(s)
- Andrea Remo
- Pathology Unit, ULSS9 “Scaligera”, 37122 Verona, Italy;
| | - Silvia Negro
- General Surgery 3, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, 35121 Padua, Italy; (R.Q.B.); (M.A.); (S.P.); (E.D.L.U.)
| | - Riccardo Quoc Bao
- General Surgery 3, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, 35121 Padua, Italy; (R.Q.B.); (M.A.); (S.P.); (E.D.L.U.)
| | - Edoardo d’Angelo
- General Surgery 3, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, 35121 Padua, Italy; (R.Q.B.); (M.A.); (S.P.); (E.D.L.U.)
| | - Rita Alaggio
- Pathology Department, Ospedale Pediatrico Bambino Gesù, IRCCS, 00165 Roma, Italy
| | - Gino Crivellari
- Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IOV-IRCCS, 35121 Padua, Italy; (G.C.); (I.M.)
| | - Isabella Mammi
- Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IOV-IRCCS, 35121 Padua, Italy; (G.C.); (I.M.)
| | - Rossana Intini
- Oncology 1, Veneto Institute of Oncology, IOV-IRCCS, 35121 Padua, Italy; (R.I.); (F.B.)
| | - Francesca Bergamo
- Oncology 1, Veneto Institute of Oncology, IOV-IRCCS, 35121 Padua, Italy; (R.I.); (F.B.)
| | - Matteo Fassan
- Department of Medicine-DIMED, University of Padova, 35121 Padua, Italy;
| | - Marco Agostini
- General Surgery 3, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, 35121 Padua, Italy; (R.Q.B.); (M.A.); (S.P.); (E.D.L.U.)
| | - Marco Vitellaro
- Unit of Hereditary Digestive Tract Tumors, Fondazione IRCCS Istituto Nazionale dei Tumori, 20133 Milan, Italy;
| | - Salvatore Pucciarelli
- General Surgery 3, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, 35121 Padua, Italy; (R.Q.B.); (M.A.); (S.P.); (E.D.L.U.)
| | - Emanuele Damiano Luca Urso
- General Surgery 3, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, 35121 Padua, Italy; (R.Q.B.); (M.A.); (S.P.); (E.D.L.U.)
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8
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Ravanbakhsh N, Shillingford N, Piester TL. A Diagnostic Conundrum: A Case of Pediatric Autoimmune Pancreatitis. JPGN REPORTS 2023; 4:e326. [PMID: 37600616 PMCID: PMC10435041 DOI: 10.1097/pg9.0000000000000326] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 12/14/2022] [Accepted: 04/14/2023] [Indexed: 08/22/2023]
Abstract
Autoimmune pancreatitis (AIP) is rare cause of abdominal pain in children who often present with obstructive jaundice, mimicking malignancy. An investigation of clinical symptoms, serology, imaging, and histopathology is necessary for diagnosis. We report a 10-year-old female presenting with abdominal pain and jaundice, ultimately found to have AIP after confirmation with tissue pathology. Our patient's prompt response to corticosteroid initiation is characteristic of this disease state. AIP has 2 subtypes, the second of which is more frequently found in children. Our patient's pathology did not fit perfectly with either subtype, but had features found in each one. While diagnostic criteria for AIP have not established in pediatrics, our case highlights the combination of clinical symptoms, imaging, and histopathology that children classically present with. While rare, the diagnosis of AIP is associated with comorbidities and must be considered in any child presenting with a pancreatic mass or biliary stricture.
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Affiliation(s)
- Naseem Ravanbakhsh
- From the Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital Los Angeles, Los Angeles, CA, USA
| | - Nick Shillingford
- Division of Pathology, Children’s Hospital Los Angeles, Los Angeles, CA, USA
| | - Travis L. Piester
- From the Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital Los Angeles, Los Angeles, CA, USA
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9
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Eklund MJ, States LJ, Acord MR, Alazraki AL, Behr GG, El-Ali AM, Morin CE, Saigal G, Shet NS, Thacker PG, Trout AT. Imaging of pediatric pancreas tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper. Pediatr Blood Cancer 2023; 70 Suppl 4:e29975. [PMID: 36215203 PMCID: PMC10642208 DOI: 10.1002/pbc.29975] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2022] [Accepted: 08/18/2022] [Indexed: 11/10/2022]
Abstract
Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic type is the solid pseudopapillary neoplasm, followed by pancreatoblastoma. This paper describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow-up.
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Affiliation(s)
- Meryle J Eklund
- Department of Radiology and Radiological Science, Medical University of South Carolina, Charleston, South Carolina, USA
| | - Lisa J States
- Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA
| | - Michael R Acord
- Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA
| | - Adina L Alazraki
- Departments of Pediatrics and Radiology, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia, USA
| | - Gerald G Behr
- Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
| | - Alexander M El-Ali
- Department of Radiology, NYU Grossman School of Medicine, New York, New York, USA
| | - Cara E Morin
- Department of Radiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | - Gaurav Saigal
- Department of Radiology, University of Miami Miller School of Medicine, Miami, Florida, USA
| | - Narendra S Shet
- Department of Diagnostic Imaging and Radiology, Children's National Hospital, Washington, District of Columbia, USA
| | - Paul G Thacker
- Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA
| | - Andrew T Trout
- Department of Radiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
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10
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Bhattarai M, Shrestha A, Bhandari S, Buha N, Baral R, Shrivastav S, Lakhey PJ. Pancreatic solid pseudopapillary neoplasm with concomitant left unilateral renal agenesis and bicornuate uterus: a case report. Ann Med Surg (Lond) 2023; 85:1166-1171. [PMID: 37113970 PMCID: PMC10129163 DOI: 10.1097/ms9.0000000000000360] [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: 11/16/2022] [Accepted: 02/12/2023] [Indexed: 04/29/2023] Open
Abstract
A solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor of the pancreas. Concomitant SPN with urogenital anomalies is a very rare presentation. Case Presentation A 16-year-old female presented with a chief complaint of abdominal pain 30 days back. Solid pseudopapillary neoplasm (SPN) of the pancreas was diagnosed with the aid of ultrasonography and contrast-enhanced computed tomography of the abdomen and pelvis. Incidentally, concomitant left unilateral renal agenesis and bicornuate uterus were also detected in radiological findings. The patient underwent spleen-preserving distal pancreatectomy, and SPN was confirmed with the histopathological report. Discussion Symptomatic SPN patients present with an abdominal mass and pain or very rarely jaundice. Most of the SPNs are benign. Complete surgical excision results in more than 95% cure. SPN with concomitant urogenital anomalies is extremely rare, and their concurrent occurrence can be better attributed to Wnt signaling pathway owing to their similar pathogenic mechanism. Conclusion The solid pseudopapillary tumor has an excellent prognosis if timely resected. Proper evaluation of the patient with imaging is necessary to suspect and diagnose SPN who has urogenital anomalies and vice versa.
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Affiliation(s)
- Madhur Bhattarai
- Institute of Medicine, Tribhuvan University, Maharajgunj Medical Campus
| | - Ayush Shrestha
- Institute of Medicine, Tribhuvan University, Maharajgunj Medical Campus
| | - Suyog Bhandari
- Department of Surgical Gastroenterology
- Corresponding author. Address: Institute of Medicine, Tribhuvan University, Maharajgunj 44600, Nepal. Tel. +977-9841881823. E-mail address: (S. Bhandari)
| | | | - Rituraj Baral
- Department of Pathology, Institute of Medicine, Tribhuvan University, Maharajgunj, Nepal
| | - Shreya Shrivastav
- Department of Pathology, Institute of Medicine, Tribhuvan University, Maharajgunj, Nepal
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11
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The Role of MRI in the Diagnosis of Solid Pseudopapillary Neoplasm of the Pancreas and Its Mimickers: A Case-Based Review with Emphasis on Differential Diagnosis. Diagnostics (Basel) 2023; 13:diagnostics13061074. [PMID: 36980388 PMCID: PMC10046973 DOI: 10.3390/diagnostics13061074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2023] [Revised: 03/05/2023] [Accepted: 03/09/2023] [Indexed: 03/15/2023] Open
Abstract
Solid pseudopapillary neoplasm (SPN) is rare pancreatic tumor occurring most commonly in young females. The typical imaging appearance of SPN is of well-defined, encapsulated, and large heterogeneous tumors, consisting of solid and cystic components due to various degrees of intralesional hemorrhage and necrosis. However, atypical imaging presentation in the form of small solid tumors or uniformly cystic lesions might also be seen, which can be explained by specific pathological characteristics. Other imaging features such as a round shape, the absence of main pancreatic duct dilatation, and slow growth, in combination with vague symptoms, favor the diagnosis of SPNs. Nevertheless, the radiological findings of SPN might overlap with other solid and cystic pancreatic neoplasms, such as neuroendocrine tumors, serous and mucinous neoplasms, and even small pancreatic adenocarcinomas. In addition, a few benign non-tumorous conditions including walled-of-necrosis, and intrapancreatic accessory spleen may also pose diagnostic dilemmas simulating SPNs on imaging studies. The aim of this manuscript is to provide a comprehensive overview of the typical and atypical imaging features of SPNs and to describe useful tips for differential diagnosis with its potential mimickers.
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12
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Bevere M, Gkountakos A, Martelli FM, Scarpa A, Luchini C, Simbolo M. An Insight on Functioning Pancreatic Neuroendocrine Neoplasms. Biomedicines 2023; 11:303. [PMID: 36830839 PMCID: PMC9953748 DOI: 10.3390/biomedicines11020303] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2022] [Revised: 01/16/2023] [Accepted: 01/18/2023] [Indexed: 01/24/2023] Open
Abstract
Pancreatic neuroendocrine neoplasms (PanNENs) are rare neoplasms arising from islets of the Langerhans in the pancreas. They can be divided into two groups, based on peptide hormone secretion, functioning and nonfunctioning PanNENs. The first group is characterized by different secreted peptides causing specific syndromes and is further classified into subgroups: insulinoma, gastrinoma, glucagonoma, somatostatinoma, VIPoma and tumors producing serotonin and adrenocorticotrophic hormone. Conversely, the second group does not release peptides and is usually associated with a worse prognosis. Today, although the efforts to improve the therapeutic approaches, surgery remains the only curative treatment for patients with PanNENs. The development of high-throughput techniques has increased the molecular knowledge of PanNENs, thereby allowing us to understand better the molecular biology and potential therapeutic vulnerabilities of PanNENs. Although enormous advancements in therapeutic and molecular aspects of PanNENs have been achieved, there is poor knowledge about each subgroup of functioning PanNENs.Therefore, we believe that combining high-throughput platforms with new diagnostic tools will allow for the efficient characterization of the main differences among the subgroups of functioning PanNENs. In this narrative review, we summarize the current landscape regarding diagnosis, molecular profiling and treatment, and we discuss the future perspectives of functioning PanNENs.
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Affiliation(s)
- Michele Bevere
- Department of Diagnostics and Public Health, Section of Anatomical Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy
- ARC-Net Applied Research on Cancer Centre, University and Hospital Trust of Verona, 37134 Verona, Italy
| | - Anastasios Gkountakos
- Department of Diagnostics and Public Health, Section of Anatomical Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy
- ARC-Net Applied Research on Cancer Centre, University and Hospital Trust of Verona, 37134 Verona, Italy
| | - Filippo Maria Martelli
- Department of Diagnostics and Public Health, Section of Anatomical Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Anatomical Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy
- ARC-Net Applied Research on Cancer Centre, University and Hospital Trust of Verona, 37134 Verona, Italy
| | - Claudio Luchini
- Department of Diagnostics and Public Health, Section of Anatomical Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy
| | - Michele Simbolo
- Department of Diagnostics and Public Health, Section of Anatomical Pathology, University and Hospital Trust of Verona, 37134 Verona, Italy
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13
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Mc Garry J, Sri Paran T, Brennan S, Gallagher T. Paediatric pancreatoduodenectomy – A video vignette. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2022.102274] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
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14
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Patterson KN, Trout AT, Shenoy A, Abu-El-Haija M, Nathan JD. Solid pancreatic masses in children: A review of current evidence and clinical challenges. Front Pediatr 2022; 10:966943. [PMID: 36507125 PMCID: PMC9732489 DOI: 10.3389/fped.2022.966943] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2022] [Accepted: 11/03/2022] [Indexed: 11/26/2022] Open
Abstract
Pancreatic tumors in children are infrequently encountered in clinical practice. Their non-specific clinical presentation and overlapping imaging characteristics often make an accurate preoperative diagnosis difficult. Tumors are categorized as epithelial or non-epithelial, with epithelial tumors further classified as tumors of the exocrine or endocrine pancreas. Although both are tumors of the exocrine pancreas, solid pseudopapillary neoplasm is the most prevalent solid pancreatic tumor in children, while pancreatoblastoma is the most common malignant tumor. Insulinoma is the most common pediatric pancreatic tumor of the endocrine pancreas. Malignant tumors require a complete, often radical, surgical resection. However, pancreatic parenchyma-sparing surgical procedures are utilized for benign tumors and low-grade malignancy to preserve gland function. This review will discuss the epidemiology, pathophysiology, clinical and diagnostic characteristics, and management options associated with both common and rare solid pancreatic masses in children. We will also discuss current challenges encountered in their evaluation and treatment.
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Affiliation(s)
- Kelli N Patterson
- Center for Surgical Outcomes Research, Abigail Wexner Research Institute, Nationwide Children's Hospital, Columbus, OH, United States
| | - Andrew T Trout
- Department of Radiology, Cincinnati Children's Hospital Medical Center, Departments of Radiology and Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Archana Shenoy
- Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Department of Pathology, The Ohio State University College of Medicine, Columbus, OH, United States
| | - Maisam Abu-El-Haija
- Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Jaimie D Nathan
- Department of Abdominal Transplant and Hepatopancreatobiliary Surgery, Nationwide Children's Hospital, Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, United States
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15
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Kim HHR, Hull NC, Lee EY, Phillips GS. Pediatric Abdominal Masses: Imaging Guidelines and Recommendations. Radiol Clin North Am 2021; 60:113-129. [PMID: 34836559 DOI: 10.1016/j.rcl.2021.08.008] [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] [Indexed: 11/17/2022]
Abstract
Pediatric abdominal masses are commonly encountered in the pediatric population, with a broad differential diagnosis that encompasses benign and malignant entities. The primary role of abdominal imaging in the setting of a suspected pediatric abdominal mass is to establish its presence, as nonneoplastic entities can mimic an abdominal mass, and to identify characteristic imaging features that narrow the differential diagnosis. In the setting of a neoplasm, various imaging modalities play an important role to characterize the mass, stage extent of disease, and assist in presurgical planning. The purpose of this article is to discuss a practical imaging algorithm for suspected pediatric abdominal masses and to describe typical radiological findings of the commonly encountered abdominal masses in neonates and children with emphasis on imaging guidelines and recommendations.
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Affiliation(s)
- Helen H R Kim
- Department of Radiology, Seattle Children's Hospital and University of Washington, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA.
| | - Nathan C Hull
- Department of Radiology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA
| | - Edward Y Lee
- Department of Radiology, Boston Children's Hospital, Harvard Medical School, 330 Longwood Avenue, Boston, MA 02115, USA
| | - Grace S Phillips
- Department of Radiology, Seattle Children's Hospital and University of Washington, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA
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16
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Abbey P, Anand R. Pancreatoblastoma with Portal Venous Invasion: A Case Series. Indian J Radiol Imaging 2021; 31:611-617. [PMID: 34790306 PMCID: PMC8590571 DOI: 10.1055/s-0041-1735914] [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] [Indexed: 11/22/2022] Open
Abstract
We describe the sonography and computed tomography (CT) findings in three children with pancreatoblastoma and highlight the tendency of these tumors to invade the splenoportal axis. All three of our cases showed tumor thrombus within the portal vein which is a helpful diagnostic feature on imaging studies.
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Affiliation(s)
- Pooja Abbey
- Department of Radio-Diagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India
| | - Rama Anand
- Department of Radio-Diagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India
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17
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Tagawa M, Morita A, Imagawa K, Mizokami Y. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound in children. Dig Endosc 2021; 33:1045-1058. [PMID: 33423305 DOI: 10.1111/den.13928] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/23/2020] [Revised: 01/04/2021] [Accepted: 01/06/2021] [Indexed: 12/13/2022]
Abstract
Gastrointestinal endoscopy is fundamental to diagnostic and therapeutic procedures in pediatric gastroenterology. In the decades since endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound (EUS) for hepatobiliary and pancreatic disease were introduced into clinical practice, there has been increasing interest in these procedures, and practice guidelines and position papers that clearly define the role of ERCP and EUS in children have been published. Based on the distinction of endoscopy between children and adults, this review focuses on the current state of ERCP and EUS procedures in children, including the types of endoscopes used in children, general anesthesia and radiation exposure, biliary and pancreatic indications, considerations of education and training for ERCP and EUS procedures in children, and expectations for development of endoscopes for children.
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Affiliation(s)
- Manabu Tagawa
- Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan
| | - Atsushi Morita
- Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan
| | - Kazuo Imagawa
- Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan
| | - Yuji Mizokami
- Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.,Endoscopic Center, University of Tsukuba Hospital, Ibaraki, Japan
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18
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Qiu L, Trout AT, Ayyala RS, Szabo S, Nathan JD, Geller JI, Dillman JR. Pancreatic Masses in Children and Young Adults: Multimodality Review with Pathologic Correlation. Radiographics 2021; 41:1766-1784. [PMID: 34597223 DOI: 10.1148/rg.2021210008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
Masses and masslike lesions of the pancreas are uncommon in the pediatric population. However, owing to overlapping clinical and imaging features, it can be challenging to differentiate the various causes of pediatric pancreatic masses at initial patient presentation. Clinical data such as patient age, signs and symptoms at presentation, laboratory test results, and potential underlying cancer predisposition syndrome can be helpful when formulating a differential diagnosis. US may be the first imaging study to depict a pancreatic mass in a child, as this examination is frequently performed in children with nonspecific abdominal signs and symptoms because of its wide availability and relatively low cost and the lack of a need for sedation or anesthesia. CT or MRI is typically required for more thorough characterization of the mass and surgical planning. Complete characterization of pancreatic masses includes assessment of vascular involvement, local invasion, and extrapancreatic spread of tumor. The authors provide an up-to-date comprehensive review of the clinical manifestations, histopathologic features, and imaging findings of primary and secondary tumors of the pancreas in children and young adults. Advances in imaging, current prognostic information, and treatment paradigms also are highlighted. Finally, nontumorous masslike lesions of the pediatric pancreas, including vascular malformations, cystic disorders (eg, von Hippel-Lindau syndrome, cystic fibrosis), intrapancreatic accessory spleen, and autoimmune pancreatitis, are discussed. Online supplemental material is available for this article. ©RSNA, 2021.
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Affiliation(s)
- Lisa Qiu
- From the Department of Radiology (L.Q., A.T.T., R.S.A., J.R.D.), Department of Pathology (S.S.), Division of Pediatric General and Thoracic Surgery (J.D.N.), and Division of Oncology (J.I.G.), Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Ave, Cincinnati, OH 45229; and Departments of Radiology (A.T.T., R.S.A., J.R.D.), Pediatrics (A.T.T., J.I.G.), Pathology (S.S.), and Surgery (J.D.N.), University of Cincinnati College of Medicine, University of Cincinnati, Cincinnati, Ohio
| | - Andrew T Trout
- From the Department of Radiology (L.Q., A.T.T., R.S.A., J.R.D.), Department of Pathology (S.S.), Division of Pediatric General and Thoracic Surgery (J.D.N.), and Division of Oncology (J.I.G.), Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Ave, Cincinnati, OH 45229; and Departments of Radiology (A.T.T., R.S.A., J.R.D.), Pediatrics (A.T.T., J.I.G.), Pathology (S.S.), and Surgery (J.D.N.), University of Cincinnati College of Medicine, University of Cincinnati, Cincinnati, Ohio
| | - Rama S Ayyala
- From the Department of Radiology (L.Q., A.T.T., R.S.A., J.R.D.), Department of Pathology (S.S.), Division of Pediatric General and Thoracic Surgery (J.D.N.), and Division of Oncology (J.I.G.), Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Ave, Cincinnati, OH 45229; and Departments of Radiology (A.T.T., R.S.A., J.R.D.), Pediatrics (A.T.T., J.I.G.), Pathology (S.S.), and Surgery (J.D.N.), University of Cincinnati College of Medicine, University of Cincinnati, Cincinnati, Ohio
| | - Sara Szabo
- From the Department of Radiology (L.Q., A.T.T., R.S.A., J.R.D.), Department of Pathology (S.S.), Division of Pediatric General and Thoracic Surgery (J.D.N.), and Division of Oncology (J.I.G.), Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Ave, Cincinnati, OH 45229; and Departments of Radiology (A.T.T., R.S.A., J.R.D.), Pediatrics (A.T.T., J.I.G.), Pathology (S.S.), and Surgery (J.D.N.), University of Cincinnati College of Medicine, University of Cincinnati, Cincinnati, Ohio
| | - Jaimie D Nathan
- From the Department of Radiology (L.Q., A.T.T., R.S.A., J.R.D.), Department of Pathology (S.S.), Division of Pediatric General and Thoracic Surgery (J.D.N.), and Division of Oncology (J.I.G.), Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Ave, Cincinnati, OH 45229; and Departments of Radiology (A.T.T., R.S.A., J.R.D.), Pediatrics (A.T.T., J.I.G.), Pathology (S.S.), and Surgery (J.D.N.), University of Cincinnati College of Medicine, University of Cincinnati, Cincinnati, Ohio
| | - James I Geller
- From the Department of Radiology (L.Q., A.T.T., R.S.A., J.R.D.), Department of Pathology (S.S.), Division of Pediatric General and Thoracic Surgery (J.D.N.), and Division of Oncology (J.I.G.), Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Ave, Cincinnati, OH 45229; and Departments of Radiology (A.T.T., R.S.A., J.R.D.), Pediatrics (A.T.T., J.I.G.), Pathology (S.S.), and Surgery (J.D.N.), University of Cincinnati College of Medicine, University of Cincinnati, Cincinnati, Ohio
| | - Jonathan R Dillman
- From the Department of Radiology (L.Q., A.T.T., R.S.A., J.R.D.), Department of Pathology (S.S.), Division of Pediatric General and Thoracic Surgery (J.D.N.), and Division of Oncology (J.I.G.), Cincinnati Children's Hospital Medical Center, MLC 5031, 3333 Burnet Ave, Cincinnati, OH 45229; and Departments of Radiology (A.T.T., R.S.A., J.R.D.), Pediatrics (A.T.T., J.I.G.), Pathology (S.S.), and Surgery (J.D.N.), University of Cincinnati College of Medicine, University of Cincinnati, Cincinnati, Ohio
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19
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Morales GES, Payan HL, Valladares RAM, Rosado ID, Chan C. Adult pancreatoblastoma: A rare malignant tumor of the pancreas. Ann Hepatobiliary Pancreat Surg 2021; 25:436-439. [PMID: 34402449 PMCID: PMC8382863 DOI: 10.14701/ahbps.2021.25.3.436] [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: 12/24/2020] [Revised: 02/15/2021] [Accepted: 02/15/2021] [Indexed: 11/21/2022] Open
Abstract
Pancreatoblastoma (PB) is a rare malignant tumor in adults. It has an overall incidence of 0.004 per 100,000 inhabitants. Its diagnosis with fine-needle aspiration (FNA) is difficult due to multiple differentiation lines present on PB that overlap with other tumors. A 76-year-old male patient presented with jaundice, weight loss of 10 kg in 6 months, and appetite loss. Abdominal computed tomography scan showed a tumor in the pancreas head. Transendoscopic ultrasound with FNA biopsy revealed a malignant epithelial neoplasia compatible with PB with immunohistochemistry CK19 (+), P63(+), synaptophysin (–), and Ki67 50%. The patient underwent surgical resection and adjuvant chemotherapy. Currently, the patient is in follow-up with the latest imaging showing no evidence of disease at 32 months after his initial diagnosis. PB is an uncommon malignant neoplasia with an aggressive behavior. Its diagnostic and therapeutic protocols are unclear. Its preoperative diagnosis may be difficult since its imaging features and serological markers are non-specific. However, FNA may be useful in some situations. Surgical resection is currently the recommended treatment that is associated with the best long-term survival.
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Affiliation(s)
| | - Hillary Lizarraga Payan
- Department of General Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | | | - Ismael Domínguez Rosado
- Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Carlos Chan
- Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
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20
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Diaconescu S, Gîlcă-Blanariu GE, Poamaneagra S, Marginean O, Paduraru G, Stefanescu G. Could the burden of pancreatic cancer originate in childhood? World J Gastroenterol 2021; 27:5322-5340. [PMID: 34539135 PMCID: PMC8409163 DOI: 10.3748/wjg.v27.i32.5322] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2021] [Revised: 06/08/2021] [Accepted: 07/30/2021] [Indexed: 02/06/2023] Open
Abstract
The presence of pancreatic cancer during childhood is extremely rare, and physicians may be tempted to overlook this diagnosis based on age criteria. However, there are primary malignant pancreatic tumors encountered in pediatric patients, such as pancreatoblastoma, and tumors considered benign in general but may present a malignant potential, such as the solid pseudo-papillary tumor, insulinoma, gastrinoma, and vasoactive intestinal peptide secreting tumor. Their early diagnosis and management are of paramount importance since the survival rates tend to differ for various types of these conditions. Many pediatric cancers may present pancreatic metastases, such as renal cell carcinoma, which may evolve with pancreatic metastatic disease even after two or more decades. Several childhood diseases may create a predisposition for the development of pancreatic cancer during adulthood; hence, there is a need for extensive screening strategies and complex programs to facilitate the transition from pediatric to adult healthcare. Nevertheless, genetic studies highlight the fact the specific gene mutations and family aggregations may be correlated with a special predisposition towards pancreatic cancer. This review aims to report the main pancreatic cancers diagnosed during childhood, the most important childhood diseases predisposing to the development of pancreatic malignancies, and the gene mutations associates with pancreatic malignant tumors.
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Affiliation(s)
- Smaranda Diaconescu
- Department of Pediatrics, “Grigore T. Popa” University of Medicine and Pharmacy, Iasi 700115, Romania
- Department of Pediatric Gastroenterology, St Mary Emergency Children's Hospital, Iasi 700309, Romania
| | - Georgiana Emmanuela Gîlcă-Blanariu
- Department of Gastroenterology and Hepatology, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi 700115, Romania
- Department of Gastroenterology and Hepatology, “St. Spiridon” Emergency Hospital, Iasi 700111, Romania
| | - Silvia Poamaneagra
- Department of Pediatric Gastroenterology, St Mary Emergency Children's Hospital, Iasi 700309, Romania
- Doctoral School, George Emil Palade University of Medicine, Pharmacy, Science and Technology, Targu Mures 540142, Romania
| | - Otilia Marginean
- Department of Pediatrics, Research Center of Disturbance of Growth and Development on Children-Belive, University of Medicine and Pharmacy “Victor Babes” Timisoara, Timisoara 300041, Romania
- First Clinic of Pediatrics, "Louis Turcanu" Emergency Childen's Hospital, Timisoara 300011, Romania
| | - Gabriela Paduraru
- Department of Pediatrics, “Grigore T. Popa” University of Medicine and Pharmacy, Iasi 700115, Romania
- Department of Pediatric Gastroenterology, St Mary Emergency Children's Hospital, Iasi 700309, Romania
| | - Gabriela Stefanescu
- Department of Gastroenterology and Hepatology, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi 700115, Romania
- Department of Gastroenterology and Hepatology, “St. Spiridon” Emergency Hospital, Iasi 700111, Romania
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21
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Omiyale AO. Solid pseudopapillary neoplasm of the pancreas. World J Hepatol 2021; 13:896-903. [PMID: 34552696 PMCID: PMC8422912 DOI: 10.4254/wjh.v13.i8.896] [Citation(s) in RCA: 22] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/02/2021] [Revised: 05/16/2021] [Accepted: 08/03/2021] [Indexed: 02/06/2023] Open
Abstract
Solid pseudopapillary neoplasms are rare. This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas, including the epidemiology, cytology, molecular pathology, differential diagnosis, treatment, and prognosis. Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns. Solid pseudopapillary neoplasms occur predominantly in young women. Although solid pseudopapillary neoplasms can occur throughout the pancreas, they arise slightly more frequently in the tail of the pancreas. The aetiology is unknown. Extremely rare cases have been reported in the setting of familial adenomatous polyposis. There are no symptoms unique to solid pseudopapillary neoplasms, however, the most common symptom is abdominal pain or discomfort. The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour. Typically, well-demarcated masses with variably solid and cystic appearances. Microscopically, these tumours are composed of epithelial cells forming solid and pseudopapillary structures, frequently undergoing haemorrhagic cystic degeneration. Typically, these tumours express nuclear and/or cytoplasmic β-catenin. Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1, the gene encoding β-catenin. The overall prognosis is excellent, and most patients are cured by complete surgical resection.
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Affiliation(s)
- Ayo O Omiyale
- Department of Cellular Pathology, Imperial College Healthcare NHS Trust, Charing Cross Hospital, London W6 8RF, United Kingdom
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22
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Bien E, Roganovic J, Krawczyk MA, Godzinski J, Orbach D, Cecchetto G, Barthlen W, Defachelles AS, Ferrari A, Weldon CB, Brecht IB, Schneider DT, Bisogno G, Kolenova A, Ben-Ami T, Martinova K, Virgone C, Stachowicz-Stencel T, Kachanov D, Reguerre Y. Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations. Pediatr Blood Cancer 2021; 68 Suppl 4:e29112. [PMID: 34174157 DOI: 10.1002/pbc.29112] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/22/2021] [Revised: 04/20/2021] [Accepted: 04/25/2021] [Indexed: 12/17/2022]
Abstract
Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neoadjuvant chemotherapy. This manuscript presents the international consensus recommendations for the diagnosis and treatment of children with PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the EU-funded PARTNER (Paediatric Rare Tumors Network - European Registry) project.
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Affiliation(s)
- Ewa Bien
- Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
| | - Jelena Roganovic
- Department of Pediatrics, Clinical Hospital Center Rijeka, University of Rijeka, Rijeka, Croatia
| | - Malgorzata A Krawczyk
- Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
| | - Jan Godzinski
- Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland.,Department of Pediatric Traumatology and Emergency Medicine, Wroclaw Medical University, Wroclaw, Poland
| | - Daniel Orbach
- SIREDO Oncology Center (Care, Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France
| | - Giovanni Cecchetto
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Winfred Barthlen
- Pediatric Surgery, Universitaetsmedizin Greifswald, Greifswald, Germany
| | | | - Andrea Ferrari
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Christopher B Weldon
- Departments of Surgery, Oncology and Anesthesiology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard University, Boston, Massachusetts, USA
| | - Ines B Brecht
- Pediatric Hematology and Oncology, Children's Hospital, Eberhard-Karls-Universitaet Tübingen, Tübingen, Germany
| | | | - Gianni Bisogno
- Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padua, Italy
| | | | - Tal Ben-Ami
- Pediatric Hematology Unit, Kaplan Medical Center, Rehovot, Israel
| | - Kata Martinova
- Department of Hematology and Oncology, University Clinic for Children`s Diseases, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of North Macedonia
| | - Calogero Virgone
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | | | - Denis Kachanov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia
| | - Yves Reguerre
- Department of Pediatric Hematology and Oncology, Félix Guyon University Hospital, St Denis, Réunion Island, France
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23
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Bonilla Gonzalez C, Rusinque J, Uribe C, Carias A, Contreras ML. Pancreatic VIPoma as a Differential Diagnosis in Chronic Pediatric Diarrhea: A Case Report and Review of the Literature. J Med Cases 2021; 12:195-201. [PMID: 34434457 PMCID: PMC8383534 DOI: 10.14740/jmc3535] [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: 11/08/2020] [Accepted: 11/26/2020] [Indexed: 11/11/2022] Open
Abstract
Chronic diarrhea is a common chief complaint in the pediatric population with a wide range of diagnostic differentials; as such, whilst suspecting common causes, less prevalent conditions tend to be overlooked, such as neuroendocrine tumor pathologies. VIPomas are characterized by hypersecretion of the vasoactive intestinal peptide (VIP), causing watery diarrhea, hypokalemia, and achlorhydria. Nonetheless, its low incidence rate in children makes it an easily unnoticed pathology. Herein, we report a case of a 14-year-old female patient and a review of relevant literature. The patient complained of 7-month history of watery diarrhea, multiple emetic episodes, and relevant past medical history of multiple hospitalizations. Chronic diarrheal disease work-up studies, including a high VIP scintigram, showed a lesion suggestive of a VIPoma-type neuroendocrine pancreatic tumor. A distal pancreatectomy was performed with a complete resolution of the symptoms. When faced with a pediatric patient presenting with chronic secretory diarrhea and whose work-up studies rule out the most common pathologies, the possible presence of a neuroendocrine tumor as VIPoma should be considered.
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Affiliation(s)
| | | | | | - Ailim Carias
- Pediatric Gastroenterology, University Hospital Fundacion Santa Fe de Bogota, Bogota, Columbia
| | - Maria Luisa Contreras
- Pediatric Critical Care, University Hospital Fundacion Santa Fe de Bogota, Bogota, Columbia
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24
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Brillantino C, Rossi E, Pirisi P, Gaglione G, Errico ME, Minelli R, Menna BF, Zeccolini R, Zeccolini M. Pseudopapillary solid tumour of the pancreas in paediatric age: description of a case report and review of the literature. J Ultrasound 2021; 25:251-257. [PMID: 33895967 DOI: 10.1007/s40477-021-00587-7] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2021] [Accepted: 04/13/2021] [Indexed: 12/01/2022] Open
Abstract
Pseudopapillary solid tumour of the pancreas is a rare neoplasm that mainly affects young women in the second and third decade of life and less frequently children; originates from the exocrine component of the pancreas; and is characterized by slow growth, low potential for malignancy, and excellent prognosis following complete surgical resection. The tumour often presents as an asymptomatic abdominal mass that is accidentally detected during radiological investigations performed for other reasons. In this article, we report the clinical case of a 10-year-old girl who came to our observation for pain in the left hypochondrium, which had arisen for a week following a trauma; the imaging methods revealed a voluminous expansive pancreatic formation in the abdomen; on histological examination, the mass was a solid pseudopapillary tumour. Furthermore, we present a review of the literature aimed at highlighting the salient features of this neoplasm in paediatric age.
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Affiliation(s)
- Carmela Brillantino
- Unit of Radiology and Ultrasound, Pausilipon Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Eugenio Rossi
- Unit of Radiology and Ultrasound, Pausilipon Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Pietro Pirisi
- Surgical Oncology Unit, Pausilipon Hospital, A.O.R.N. Santobono Pausilipon, Naples, Italy
| | - Giovanni Gaglione
- UOC Paediatric Surgery A.O.R.N. Santobono-Pausilipon, Santobono Hospital, Naples, Italy
| | - Maria E Errico
- Unit of Pathological Anatomy, Pausilipon Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Rocco Minelli
- Department of Life and Health "V. Tiberio", University of Molise, Francesco De Sanctis st 1, 86100, Campobasso, Italy.
| | - Biagio F Menna
- Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini 5, 80131, Naples, Italy
| | | | - Massimo Zeccolini
- Unit of Radiology, Santobono Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
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Garnier H, Loo C, Czauderna P, Vasudevan SA. Pediatric Gastrointestinal Stromal Tumors and Neuroendocrine Tumors: Advances in Surgical Management. Surg Oncol Clin N Am 2021; 30:219-233. [PMID: 33706897 DOI: 10.1016/j.soc.2020.11.001] [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: 02/07/2023]
Abstract
Gastrointestinal stromal tumors and neuroendocrine tumors in adult and pediatric populations differ immensely. Despite these established differences, the extreme rarity of gastrointestinal stromal tumors and neuroendocrine tumors in the pediatric population has resulted in the lack of consensus management guidelines, making optimal surgical approaches unclear. Comprehensive management principles to guide surgical approaches in adult literature are extensive. However, these are still lacking for pediatric patients. International cooperation to develop standardized pediatric-specific guidelines is urgently warranted in the future. This article highlights the vast differences between adult and pediatric parameters and provides recommendations on optimal and novel surgical approaches in children.
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Affiliation(s)
- Hanna Garnier
- Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Marii Skłodowskiej-Curie 3a, Gdańsk 80-210, Poland
| | - Caitlyn Loo
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Surgical Oncology Program, Texas Children's Liver Tumor Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, 7200 Cambridge Street, 7th Floor, Houston, TX 77030, USA; School of Medicine, Royal College of Surgeons in Ireland, 123 St Stephens Green, Saint Peter's, Dublin D02 YN77, Ireland
| | - Piotr Czauderna
- Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Marii Skłodowskiej-Curie 3a, Gdańsk 80-210, Poland
| | - Sanjeev A Vasudevan
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Surgical Oncology Program, Texas Children's Liver Tumor Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, 7200 Cambridge Street, 7th Floor, Houston, TX 77030, USA.
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26
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Cramer S, Marcus MA, Ramkissoon S, Szabo S, Pressey JG. Pediatric BRAF (V600E)-Mutated Pancreatic Acinar Cell Carcinoma With Complete and Durable Response to Dabrafenib and Trametinib. JCO Precis Oncol 2020; 4:801-805. [PMID: 35050754 DOI: 10.1200/po.19.00343] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022] Open
Affiliation(s)
| | | | - Shakti Ramkissoon
- Foundation Medicine, Morrisville, NC.,Wake Forest Comprehensive Cancer Center and Department of Pathology, Winston-Salem, NC.,Wake Forest University School of Medicine, Winston-Salem, NC
| | - Sara Szabo
- Cincinnati Children's Hospital, Cincinnati, OH
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Yang Z, Gong Y, Ji M, Yang B, Qiao Z. Differential diagnosis of pancreatoblastoma (PB) and solid pseudopapillary neoplasms (SPNs) in children by CT and MR imaging. Eur Radiol 2020; 31:2209-2217. [PMID: 32997172 DOI: 10.1007/s00330-020-07309-3] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/29/2020] [Revised: 08/01/2020] [Accepted: 09/17/2020] [Indexed: 11/24/2022]
Abstract
OBJECTIVES To determine whether features on computed tomographic and/or magnetic resonance imaging can differentiate pancreatoblastoma (PB) from solid pseudopapillary neoplasms (SPNs) of the pancreas in children. METHODS Clinical and imaging data for 20 cases of SPNs and 14 cases of PB confirmed by surgery or biopsy were retrospectively analysed. The size, border, calcification, haemorrhage, solid/cystic component proportion, intratumoural vessels, tumour capsulation, pancreatic duct dilatation, peripancreatic vessel invasion, distant metastasis status and apparent diffusion coefficient (ADC) values of the two groups were examined, and key diagnostic features were identified. Statistical analysis was performed using the chi-square test and Student's t test. Sensitivity and specificity values were calculated when a single criterion was used. RESULTS Age ≤ 5 years, elevated serum α-fetoprotein (AFP), larger size, ill-defined border, calcification, absence of haemorrhage, intratumoural vessel, peripancreatic vessel invasion and distant metastasis differentiated PB from SPN (p < 0.05). ADC values of SPN were higher than those of PB (p = 0.001). There were no significant differences regarding tumour capsule (p = 0.435), pancreatic duct dilatation (p = 1.000) or cystic degeneration area over 50% of the tumour volume (p = 1.000) between the two groups. CONCLUSIONS The following features are helpful for differentiating PB from SPN: age ≤ 5 years, elevated serum AFP, larger size, ill-defined border, calcification, haemorrhage absence, intratumoural vessel, peripancreatic vessel invasion, distant metastasis and lower ADC value. KEY POINTS • CT and MRI are helpful to differentiate pancreatoblastoma (PB) from solid pseudopapillary neoplasms (SPNs) of the pancreas in children. • The following features are helpful to differentiate PB from SPN: age ≤ 5 years, elevated serum AFP, larger size, ill-defined border, calcification, absence of haemorrhage, intratumoural vessel, peripancreatic vessel invasion, distant metastasis and lower ADC value.
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Affiliation(s)
- Zhaoxia Yang
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Rd., Shanghai, 200032, China
| | - Ying Gong
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Rd., Shanghai, 200032, China.
| | - Min Ji
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Rd., Shanghai, 200032, China
| | - Bin Yang
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Rd., Shanghai, 200032, China
| | - Zhongwei Qiao
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Rd., Shanghai, 200032, China
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Vasudevan SA, Ha TAN, Zhu H, Heaton TE, LaQuaglia MP, Murphy JT, Barry WE, Goodhue C, Kim ES, Aldrink JH, Polites SF, Leraas HJ, Rice HE, Tracy ET, Lautz TB, Superina RA, Davidoff AM, Langham MR, Murphy AJ, Bütter A, Davidson J, Glick RD, Grijalva J, Gow KW, Ehrlich PF, Newman EA, Lal DR, Malek MM, Le-Nguyen A, Piché N, Rothstein DH, Short SS, Meyers R, Dasgupta R. Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study. Pediatr Blood Cancer 2020; 67:e28425. [PMID: 32658372 PMCID: PMC7674210 DOI: 10.1002/pbc.28425] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/29/2019] [Revised: 04/23/2020] [Accepted: 05/04/2020] [Indexed: 02/06/2023]
Abstract
BACKGROUND To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD). METHODS Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected. RESULTS Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival. CONCLUSION This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.
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Affiliation(s)
- Sanjeev A. Vasudevan
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children’s Surgical Oncology Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, TX, US
| | - Tu-Anh N. Ha
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children’s Surgical Oncology Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, TX, US
| | - Huirong Zhu
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children’s Surgical Oncology Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, TX, US
| | - Todd E. Heaton
- Division of Pediatric Surgery, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York City, NY, US
| | - Michael P. LaQuaglia
- Division of Pediatric Surgery, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York City, NY, US
| | - Joseph T. Murphy
- Division of Pediatric Surgery, UT Southwestern Medical School, Children’s Medical Center, Dallas, TX, US
| | - Wesley E. Barry
- Division of Pediatric Surgery, Department of Surgery, Children’s Hospital Los Angeles, USC Keck School of Medicine, Los Angeles, CA, US
| | - Catherine Goodhue
- Division of Pediatric Surgery, Department of Surgery, Children’s Hospital Los Angeles, USC Keck School of Medicine, Los Angeles, CA, US
| | - Eugene S. Kim
- Division of Pediatric Surgery, Department of Surgery, Children’s Hospital Los Angeles, USC Keck School of Medicine, Los Angeles, CA, US
| | - Jennifer H. Aldrink
- Division of Pediatric Surgery, Department of Surgery, Nationwide Children’s Hospital, The Ohio State University College of Medicine, Columbus, OH, US
| | - Stephanie F. Polites
- Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, OH, US
| | - Harold J Leraas
- Division of Pediatric Surgery, Duke University Medical Center, Durham, NC, US
| | - Henry E Rice
- Division of Pediatric Surgery, Duke University Medical Center, Durham, NC, US
| | - Elisabeth T Tracy
- Division of Pediatric Surgery, Duke University Medical Center, Durham, NC, US
| | - Timothy B Lautz
- Division of Pediatric Surgery, Division of Transplant Surgery, Ann & Robert H Lurie Children’s Hospital of Chicago, Northwestern University, Chicago, IL, US
| | - Riccardo A Superina
- Division of Pediatric Surgery, Division of Transplant Surgery, Ann & Robert H Lurie Children’s Hospital of Chicago, Northwestern University, Chicago, IL, US
| | - Andrew M. Davidoff
- Department of Surgery, St. Jude Children’s Research Hospital, University of Tennessee Health Science Center, Memphis, TN, US
| | - Max R. Langham
- Department of Surgery, St. Jude Children’s Research Hospital, University of Tennessee Health Science Center, Memphis, TN, US
| | - Andrew J. Murphy
- Department of Surgery, St. Jude Children’s Research Hospital, University of Tennessee Health Science Center, Memphis, TN, US
| | - Andreana Bütter
- Division of Pediatric Surgery, Children’s Hospital, London Health Sciences Centre, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada
| | - Jacob Davidson
- Division of Pediatric Surgery, Children’s Hospital, London Health Sciences Centre, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada
| | - Richard D. Glick
- Division of Pediatric Surgery, Cohen Children’s Medical Center, Zucker School of Medicine at Hofstra/Northwell, Queens, NY, US
| | - James Grijalva
- Division of Pediatric Surgery, Seattle Children’s Hospital, Seattle, WA, US
| | - Kenneth W. Gow
- Division of Pediatric Surgery, Seattle Children’s Hospital, Seattle, WA, US
| | - Peter F. Ehrlich
- Department of Surgery, C.S. Mott Children’s Hospital, The University of Michigan, Ann Arbor, MI, US
| | - Erika A. Newman
- Department of Surgery, C.S. Mott Children’s Hospital, The University of Michigan, Ann Arbor, MI, US
| | - Dave R. Lal
- Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, US
| | - Marcus M. Malek
- Division of Pediatric General and Thoracic Surgery, Children’s Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, PA, US
| | - Annie Le-Nguyen
- Division of Pediatric Surgery, CHU Sainte-Justine, University of Montreal, Montreal, Qc, Canada
| | - Nelson Piché
- Division of Pediatric Surgery, CHU Sainte-Justine, University of Montreal, Montreal, Qc, Canada
| | - David H. Rothstein
- Department of Pediatric Surgery, John R. Oishei Children’s Hospital, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, US
| | - Scott S. Short
- Division of Pediatric Surgery, Primary Children’s Hospital, University of Utah School of Medicine, Salt Lake City, UT, US
| | - Rebecka Meyers
- Division of Pediatric Surgery, Primary Children’s Hospital, University of Utah School of Medicine, Salt Lake City, UT, US
| | - Roshni Dasgupta
- Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, OH, US
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Berrada G, Belaaroussi S, Chbani K, Salam S, Laoudiyi D, Ouzidane L, Kebir AE, Guebessi NB, Benayad S, Mernissi F, Karkouri M, Anis S, Zemmouri MA. Solid pseudopapillary tumor of the pancreas: a rare entity in children. Pan Afr Med J 2020; 35:137. [PMID: 32655751 PMCID: PMC7335250 DOI: 10.11604/pamj.2020.35.137.22404] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2020] [Accepted: 04/09/2020] [Indexed: 02/06/2023] Open
Abstract
Solid pseudopapillary tumors (SPTs) constitute 0.2 to 2.7% of non-endocrine primary tumors of the pancreas and comprise the majority (70%) of pediatric pancreatic neoplasms. These tumors are of unclear pathogenesis, low malignancy and favorable prognosis. Surgical resection offers an excellent chance for longterm survival, even in the presence of distant metastasis. The objective of this study is to review our experience in the management of SPT in a 12 years old girl at the pediatric hospital of the University hospital of Casablanca, in Morocco and provide an update on current management in pediatric population.
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Affiliation(s)
- Ghita Berrada
- Department of Pediatric Radiology, Abderrahim Harouchi Pediatric Hospital, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Soukaina Belaaroussi
- Department of Pediatric Radiology, Abderrahim Harouchi Pediatric Hospital, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Kamilia Chbani
- Department of Pediatric Radiology, Abderrahim Harouchi Pediatric Hospital, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Siham Salam
- Department of Pediatric Radiology, Abderrahim Harouchi Pediatric Hospital, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Dalal Laoudiyi
- Department of Pediatric Radiology, Abderrahim Harouchi Pediatric Hospital, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Lahcen Ouzidane
- Department of Pediatric Radiology, Abderrahim Harouchi Pediatric Hospital, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Asmaa El Kebir
- Department of Pathology, Ibn Rochd University Hospital, Casablanca, Morocco
| | | | - Samira Benayad
- Department of Pathology, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Farida Mernissi
- Department of Pathology, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Mehdi Karkouri
- Department of Pathology, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Salma Anis
- Department of Surgery, Ibn Rochd University Hospital, Casablanca, Morocco
| | - Mounia Al Zemmouri
- Department of Surgery, Ibn Rochd University Hospital, Casablanca, Morocco
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30
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Adult Pancreatoblastoma With Liver Metastasis Diagnosed by 18F-FDG PET/CT and 2 Years' Postoperative Follow-up. Clin Nucl Med 2020; 45:e24-e28. [PMID: 31274558 DOI: 10.1097/rlu.0000000000002684] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Pancreatoblastoma is an extremely rare malignant tumor of the pancreas. Most patients are infants and children; however, a few cases have been reported in adults. Herein, we present the case of a 49-year-old man with pancreatoblastoma and liver metastasis who underwent preoperative multimodal imaging, in which F-FDG PET/CT showed a markedly increased F-FDG uptake in the metastatic lesion and a slightly increased uptake in the primary lesion. Subsequently, the patient underwent complete resection of the primary and metastatic lesions. No recurrences or metastases were found in 2 years of follow-up.
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31
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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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Abstract
MR and F-FDG PET/MR images of a 15-year-old boy with recurrent mild abdominal pain and hyperamylasemia demonstrating a pancreatic head lesion hypointense on the T1-weighted and slightly hyperintense on the T2-weighted images, with very small pseudocystic areas, restricted diffusion on the apparent diffusion coefficient map, and focal FDG uptake. No other pathologic findings such as enlarged lymph nodes, vascular invasion, metastases, dilatation of the main pancreatic duct, or pathologic uptake of the parotid glands were detected. The MR features, even if atypical, together with the focal uptake, allowed the diagnosis of pediatric solid pseudopapillary pancreatic tumor, which was then confirmed by histology.
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33
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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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34
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Pediatric ultrasonography of the pancreas: normal and abnormal findings. J Ultrasound 2018; 22:261-272. [PMID: 30552664 DOI: 10.1007/s40477-018-0348-8] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2018] [Accepted: 12/04/2018] [Indexed: 12/11/2022] Open
Abstract
The pancreas is easily investigated in children thanks to the relative lack of fat tissue and the large left hepatic lobe with an optimal acoustic window. The use of high frequency, even linear transducers, usually results in detailed images of all pancreatic areas. A wide spectrum of pancreatic pathologic conditions can be identified and monitored at ultrasound although they are relatively uncommon during childhood compared to the adult. In this paper we briefly review the anatomy, technique, and sonographic aspects of normal and pathological pediatric pancreas.
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35
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Solid Pseudo-Papillary Tumor Mimicking as Complicated Pseudocyst: Multimodality Imaging and Pathological Correlation. Clin Nucl Med 2018; 43:e368-e371. [PMID: 30080687 DOI: 10.1097/rlu.0000000000002225] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Pancreatic neoplasm is very rare in the pediatric population. Malignant tumors represent less than 0.2% of pediatric cancer-related mortality. Pancreas lesions can be from exocrine or endocrine origin or present themselves as cystic masses. Clinical, biological, and radiological findings usually are sufficient to establish diagnosis, but in some cases, they may be misleading. We present the case of a young patient presenting a pancreatic mass where anatomical and metabolic characteristics of the lesion were discordant to the final diagnosis.
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36
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Bohl CE, Federico SM, Robinson GW, Bahrami A, Shulkin BL. FDG-PET CT in the evaluation of primary and secondary pancreatic malignancies. Pediatr Blood Cancer 2018; 65:e27115. [PMID: 29750397 DOI: 10.1002/pbc.27115] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/20/2017] [Revised: 02/28/2018] [Accepted: 03/16/2018] [Indexed: 11/06/2022]
Abstract
PURPOSE Primary pancreatic carcinoma and pancreatic metastases are rare in the pediatric population. Pancreatoblastoma is the most common pancreatic malignant tumor in young children and solid-pseudopapillary tumor in teenagers. Pancreatic adenocarcinoma is extremely rare under the age of 40 and is usually associated with underlying genetic abnormalities. Secondary malignancies of the pancreas occur more frequently than primary pancreatic malignancies in children and are most commonly seen with non-Hodgkin lymphomas (NHL) and mesenchymal sarcomas. The purpose of this study was to characterize the metabolism of primary and secondary tumors of the pancreas in pediatric patients. MATERIALS AND METHODS A retrospective analysis of all primary and secondary pancreatic malignancies imaged with 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) computed tomography (CT) was conducted. RESULTS Three patients with primary pancreatic cancers were identified, one each with pancreatoblastoma, solid-pseudopapillary tumor, and adenocarcinoma. Each tumor showed elevated uptake of FDG. Metastatic disease in the pancreas was identified in 12 patients-five NHL (including three Burkitt lymphomas), six sarcomas (three osteosarcomas, two rhabdomyosarcomas, and one Ewing sarcoma family tumor), and one malignant rhabdoid tumor. Elevated but variable uptake of FDG was found in each of the tumors of patients with metastatic disease within the pancreas. CONCLUSION Both primary malignancies and metastatic disease within the pancreas, though very rare in children, adolescents, and young adults, are metabolically active and can be functionally characterized using FDG-PET CT.
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Affiliation(s)
- Casey E Bohl
- Department of Radiology, Methodist University Hospital, Memphis, Tennessee
| | - Sara M Federico
- Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee
| | - Giles W Robinson
- Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee
| | - Armita Bahrami
- Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee
| | - Barry L Shulkin
- Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, Tennessee.,Department of Radiology, University of Tennessee Health Science Center, Memphis, Tennessee
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38
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Escartín R, Brun N, García Monforte MN, Ferreres JC, Corripio R. Insulinoma: A Rare Cause of Hypoglycemia in Childhood. AMERICAN JOURNAL OF CASE REPORTS 2018; 19:1121-1125. [PMID: 30237390 PMCID: PMC6161565 DOI: 10.12659/ajcr.910426] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Patient: Male, 11 Final Diagnosis: Insulinoma Symptoms: Dizziness • psychological issues Medication: — Clinical Procedure: — Specialty: Endocrinology and Metabolic
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Affiliation(s)
- Rocío Escartín
- Department of Pediatric Endocrinology, Parc Taulí Hospital Universitari, Research and Innovation Institute Parc Taulí I3PT, Autonomous University of Barcelona, Sabadell, Spain
| | - Nuria Brun
- Department of Surgery, Parc Taulí Hospital Universitari, Research and Innovation Institute Parc Taulí I3PT, Autonomous University of Barcelona, Sabadell, Spain
| | - M Nieves García Monforte
- Department of Surgery, Parc Taulí Hospital Universitari, Research and Innovation Institute Parc Taulí I3PT, Autonomous University of Barcelona, Sabadell, Spain
| | - Joan Carles Ferreres
- Department of Pathology, Parc Taulí Hospital Universitari, Research and Innovation Institute Parc Taulí I3PT, Autonomous University of Barcelona, Sabadell, Spain
| | - Raquel Corripio
- Department of Pediatric Endocrinology, Parc Taulí Hospital Universitari, Research and Innovation Institute Parc Taulí I3PT, Autonomous University of Barcelona, Sabadell, Spain
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39
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Cao G, Mendez J, Navacchia D. Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases. Ecancermedicalscience 2018; 12:861. [PMID: 30174723 PMCID: PMC6113986 DOI: 10.3332/ecancer.2018.861] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2018] [Indexed: 11/06/2022] Open
Abstract
Pancreatoblastoma is a rare paediatric malignant neoplasm. The treatment of choice is complete surgical resection. However, it is often unresectable due to its large size, local infiltration or distant metastasis. Since the condition is rare, there is currently no standard treatment regimen. We outline the case of a 4-year-old child who presented with abdominal pain and distention, together with an enlarged liver and elevated serum α-fetoprotein levels. Imaging studies showed the presence of an abnormal pancreatic tumour and multiple nodular lesions in the liver, the biopsies from which led to a diagnosis of pancreatoblastoma. In this case, the patient received cycles of neoadjuvant chemotherapy, combining cisplatin and doxorubicin. The patient subsequently underwent scheduled surgery in which the primary pancreatic lesion was resected, obtaining a circumscribed and nodular specimen measuring 7 × 6 cm and weighing 150 g. Given the extent of the metastasis, the child is currently awaiting a liver transplant.
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Affiliation(s)
- Gabriel Cao
- Anatomical Pathology Division, Pedro De Elizalde Children's Hospital, Avenida Montes de Oca 40 (C1270AAN), Buenos Aires, Argentina.,National Council of Scientific and Technical Research (CONICET), Godoy Cruz 2290 (C1425FQB), Buenos Aires, Argentina
| | - Julián Mendez
- Anatomical Pathology Division, Pedro De Elizalde Children's Hospital, Avenida Montes de Oca 40 (C1270AAN), Buenos Aires, Argentina
| | - Daniel Navacchia
- Anatomical Pathology Division, Pedro De Elizalde Children's Hospital, Avenida Montes de Oca 40 (C1270AAN), Buenos Aires, Argentina
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40
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Qi B, Crawford AJ, Wojtynek NE, Holmes MB, Souchek JJ, Almeida-Porada G, Ly QP, Cohen SM, Hollingsworth MA, Mohs AM. Indocyanine green loaded hyaluronan-derived nanoparticles for fluorescence-enhanced surgical imaging of pancreatic cancer. NANOMEDICINE : NANOTECHNOLOGY, BIOLOGY, AND MEDICINE 2018; 14:769-780. [PMID: 29325740 PMCID: PMC5899013 DOI: 10.1016/j.nano.2017.12.015] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/14/2017] [Revised: 11/20/2017] [Accepted: 12/19/2017] [Indexed: 01/14/2023]
Abstract
Pancreatic ductal adenocarcinoma is highly lethal and surgical resection is the only potential curative treatment for the disease. In this study, hyaluronic acid derived nanoparticles with physico-chemically entrapped indocyanine green, termed NanoICG, were utilized for intraoperative near infrared fluorescence detection of pancreatic cancer. NanoICG was not cytotoxic to healthy pancreatic epithelial cells and did not induce chemotaxis or phagocytosis, it accumulated significantly within the pancreas in an orthotopic pancreatic ductal adenocarcinoma model, and demonstrated contrast-enhancement for pancreatic lesions relative to non-diseased portions of the pancreas. Fluorescence microscopy showed higher fluorescence intensity in pancreatic lesions and splenic metastases due to NanoICG compared to ICG alone. The in vivo safety profile of NanoICG, including, biochemical, hematological, and pathological analysis of NanoICG-treated healthy mice, indicates negligible toxicity. These results suggest that NanoICG is a promising contrast agent for intraoperative detection of pancreatic tumors.
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Affiliation(s)
- Bowen Qi
- Department of Pharmaceutical Sciences, University of Nebraska Medical Center, Omaha, NE
| | - Ayrianne J Crawford
- Eppley Institute for Research in Cancer and Allied Diseases, University of Nebraska Medical Center, Omaha, NE
| | - Nicholas E Wojtynek
- Eppley Institute for Research in Cancer and Allied Diseases, University of Nebraska Medical Center, Omaha, NE
| | - Megan B Holmes
- Department of Pharmaceutical Sciences, University of Nebraska Medical Center, Omaha, NE
| | - Joshua J Souchek
- Department of Pharmaceutical Sciences, University of Nebraska Medical Center, Omaha, NE
| | - Graca Almeida-Porada
- Wake Forest Institute for Regenerative Medicine, Wake Forest University Health Sciences, Winston-Salem, NC
| | - Quan P Ly
- Department of Surgery, University of Nebraska Medical Center, Omaha, NE; Fred and Pamela Buffett Cancer Center, University of Nebraska Medical Center, Omaha, NE
| | - Samuel M Cohen
- Fred and Pamela Buffett Cancer Center, University of Nebraska Medical Center, Omaha, NE; Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE; Havlik-Wall Professor of Oncology, University of Nebraska Medical Center, Omaha, NE
| | - Michael A Hollingsworth
- Eppley Institute for Research in Cancer and Allied Diseases, University of Nebraska Medical Center, Omaha, NE; Fred and Pamela Buffett Cancer Center, University of Nebraska Medical Center, Omaha, NE
| | - Aaron M Mohs
- Department of Pharmaceutical Sciences, University of Nebraska Medical Center, Omaha, NE; Fred and Pamela Buffett Cancer Center, University of Nebraska Medical Center, Omaha, NE; Department of Biochemistry and Molecular Biology, University of Nebraska Medical Center, Omaha, NE.
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Abstract
In this review, we will focus on rare pancreatic tumors. Most of these tumors do not have distinct characteristic appearances so the key to diagnosis requires a combination of imaging appearance, laboratory data, patient demographics, and associated medical syndromes in order to narrow the differential diagnosis. Nonetheless, imaging plays a vital role in narrowing the differential and guiding management. While there are many variant pathologic entities that cannot be encompassed by a single review, we aim to illustrate the imaging appearance of less common pancreatic tumors highlighting key distinctive diagnostic characteristics and discuss the implications for management. While there is overlap in the imaging appearances of many of these entities, for educational purposes, lesions will be categorized into solid (hypoenhancing and hyperenhancing), cystic lesions, mesenchymal neoplasms, and neoplasms seen in younger patients (< 40 years).
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Affiliation(s)
- Jonathan Steinman
- Columbia University Medical Center, 622 W. 168th Street, PB 1-301, New York, NY, 10032, USA
| | - Atif Zaheer
- Johns Hopkins University School of Medicine, 601 N. Caroline Street, Baltimore, MD, USA
| | - Michael D Kluger
- Columbia University Medical Center, 161 Fort Washington Avenue, New York, NY, 10032, USA
| | - Helen Remotti
- Columbia University Medical Center, 630 West 168th Street, VC 14-215, New York, NY, 10032, USA
| | - Elizabeth M Hecht
- Columbia University Medical Center, 622 W. 168th Street, PB 1-301, New York, NY, 10032, USA.
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Rai S, Prabhu S, Rai S, Nirupama M, Adiga DS, Kumar A, Chakraborti S. Image Findings of Solid Pseudopapillary Neoplasms of the Pancreas on Multiphasic Multidetector CT Scan-A Single Institute Experience from Southern India. J Clin Diagn Res 2017; 11:TC01-TC05. [PMID: 29207802 DOI: 10.7860/jcdr/2017/24190.10530] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2016] [Accepted: 04/29/2017] [Indexed: 12/20/2022]
Abstract
Introduction Solid Pseudopapillary Neoplasms (SPN) are uncommon pancreatic tumours and are slow growing with uncertain malignant potential, showing female preponderance. Postoperative prognosis is good and metastasis is rare. Aim To summarise the imaging and pathological features of seven cases of SPN in three years period, from January 2013 to January 2016. Materials and Methods In this retrospective study the imaging features of seven cases on triphasic multidetector Computed Tomogram (CT), a 16-slice scanner, were reviewed along with CT-guided Fine Needle Aspiration Cytology (FNAC) and histopathological examination. Statistics were expressed in terms of percentiles. Results All cases were female patients with an age range of 13-35 years (mean: 23.3 years). On CT assessment, the size of the tumours varied from 2.5-14 cm (mean: 5.3 cm). All these tumours were well capsulated and round to oval in shape. In four out of seven cases, the tumour was located in the tail of pancreas. All the solid enhancing portions showed moderate enhancement of at least 20-30 HU compared to unenhanced scan, on the other hand the cystic parts remained unenhanced with <5 HU variation in comparison to the plain scan. Histopathological examination exhibited characteristic poorly cohesive cuboidal cells arranged in papillaroid pattern having fine nuclear chromatin with nuclear grooves. Conclusion Solid pseudopapillary neoplasm is a high diagnostic possibility in case of a young female having pancreatic mass and needs to be evaluated with triphasic contrast enhanced CT scan, followed by FNAC and or histopathological examination.
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Affiliation(s)
- Santosh Rai
- Associate Professor, Department of Radiodiagnosis and Imaging, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
| | - Sonali Prabhu
- Assistant Professor, Department of Radiodiagnosis and Imaging, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
| | - Sharada Rai
- Associate Professor, Department of Pathology, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
| | - Murali Nirupama
- Associate Professor, Department of Pathology, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
| | - Deepa Sa Adiga
- Associate Professor, Department of Pathology, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
| | - Ashvini Kumar
- Professor, Department of Radiodiagnosis and Imaging, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
| | - Shrijeet Chakraborti
- Associate Professor, Department of Pathology, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
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Shapira G, Fisher Y, Ilivitzki A. Bifocal primary pancreatic Burkitt's lymphoma in a 4-year-old child. JOURNAL OF CLINICAL ULTRASOUND : JCU 2017; 45:171-174. [PMID: 27245638 DOI: 10.1002/jcu.22372] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/06/2016] [Revised: 04/29/2016] [Accepted: 05/02/2016] [Indexed: 06/05/2023]
Abstract
Pancreatic tumors are uncommon in children and rarely result in biliary obstruction. Primary pancreatic Burkitt's lymphoma is an exceptionally rare entity with only a few cases described in the literature. We present a case of a bifocal primary pancreatic Burkitt's lymphoma in a 4-year-old child presenting with jaundice. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:171-174, 2017.
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Affiliation(s)
- Gali Shapira
- Pediatric Radiology Unit, Radiology Department, Rambam Medical Healthcare Center, Haifa, Israel
| | - Yael Fisher
- Pathology Department, Rambam Medical Healthcare Center, Haifa, Israel
| | - Anat Ilivitzki
- Pediatric Radiology Unit, Radiology Department, Rambam Medical Healthcare Center, Haifa, Israel
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Yao L, Xie ZB, Jin C, Jiang YJ, Li J, Yang F, Lin QJ, Fu DL. Radical resection and enucleation in Chinese adolescents with pancreatic tumors: A 15-year case series. Medicine (Baltimore) 2017; 96:e6438. [PMID: 28328854 PMCID: PMC5371491 DOI: 10.1097/md.0000000000006438] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/24/2016] [Revised: 02/22/2017] [Accepted: 02/25/2017] [Indexed: 12/24/2022] Open
Abstract
Pancreatic tumors rarely occur in adolescents, and the appropriateness of radical resection for these patients remains controversial.Medical records were retrospectively reviewed for patients younger than 19 years who underwent radical resection or limited resection (enucleation) between 2000 and 2015. Patient demographics, clinical characteristics, operative details, growth, and survival were analyzed.During the study period, 11 adolescents (mean age, 16.18 years; standard deviation, 1.99; interquartile range, 15.0-18.0) underwent radical resection (n = 7) or enucleation (n = 4) to treat solid pseudopapillary tumors (n = 5), pancreatic neuroendocrine tumors (n = 5), or pancreatic ductal adenocarcinoma (n = 1). None of the 7 patients who underwent radical resection experienced recurrence or serious complications, while 3 of 4 patients who underwent enucleation experienced recurrence (P = 0.02). Recurrence-free survival was slightly longer in patients who underwent radical resection, and this procedure did not appear to affect adolescent growth and development.Radical resection might be safe and effective for adolescents with pancreatic tumors.
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Specialized Imaging and Procedures in Pediatric Pancreatology: A North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Clinical Report. J Pediatr Gastroenterol Nutr 2017; 64:472-484. [PMID: 28230608 DOI: 10.1097/mpg.0000000000001371] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVES An increasing number of children are being diagnosed with pancreatitis and other pancreatic abnormalities. Dissemination of the information regarding existing imaging techniques and endoscopic modalities to diagnose and manage pancreatic disorders in children is sorely needed. METHODS We conducted a review of the medical literature on the use of the following imaging and procedural modalities in pediatric pancreatology: transabdominal ultrasonography (TUS), computed tomography (CT), magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP), endoscopic ultrasonography (EUS), and endoscopic retrograde cholangiopancreatography (ERCP). Recommendations for current use and future research were identified. RESULTS TUS offers noninvasive images of the pancreas but has limitations to details of parenchyma and ductal structures. CT offers improved detail of pancreatic parenchyma, solid masses, and traumatic injuries, but requires relatively high levels of ionizing radiation and does not adequately assess ductal anatomy. MRI/MRCP offers detailed intrinsic tissue assessment and pancreatic ductal characterization, but requires longer image acquisition time and is relatively poor at imaging calcifications. EUS provides excellent evaluation of pancreatic parenchyma and ductal anatomy, but can be subjective and operator dependent and requires sedation or anesthesia. EUS offers the capacity to obtain tissue samples and drain fluid collections and ERCP offers the ability to improve drainage by performing sphincterotomy or placing pancreatic stents across duct injuries and strictures. CONCLUSIONS Various imaging modalities may be used in pediatric pancreatology, but TUS and MRI/MRCP are favored. Interventional therapeutic maneuvers primarily involve use of ERCP and EUS. Future research is necessary to optimize equipment, expertise, and appropriate indications.
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Intraabdominal Lymphatic Malformations: Pearls and Pitfalls of Diagnosis and Differential Diagnoses in Pediatric Patients. AJR Am J Roentgenol 2017; 208:637-649. [DOI: 10.2214/ajr.16.17008] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
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Liu HK, Lin YC, Yeh ML, Chen YS, Su YT, Tsai CC. Inflammatory myofibroblastic tumors of the pancreas in children: A case report and literature review. Medicine (Baltimore) 2017; 96:e5870. [PMID: 28079824 PMCID: PMC5266186 DOI: 10.1097/md.0000000000005870] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/31/2016] [Revised: 12/16/2016] [Accepted: 12/17/2016] [Indexed: 12/31/2022] Open
Abstract
INTRODUCTION Inflammatory myofibroblastic tumors are an uncommon neoplasm, which are very rarely located in the pancreas. Clinically and radiologically, this rare pancreatic tumor presents as an abdominal mass lesion that mimics other pancreatic tumors, and should therefore be considered in the differential diagnosis of pancreatic tumors. CLINICAL FINDINGS AND DIAGNOSIS The 15-year-old boy complained of abdominal pain over the left upper quadrant with intermittent fever for 7 days. Abdominal sonography revealed one cystic lesion with a hyperechoic component in the left upper quadrant of the abdomen. Surgical excision was performed and postoperative findings indicated a pancreatic tail tumor. The pathology indicated inflammatory myofibroblastic tumors. To our knowledge, this patient is a unique case as the tumor was located in the pancreatic tail only, sparing the body. INTERVENTIONS AND OUTCOMES The patient underwent tumor resection and segmental resection of the transverse colon with simple closure. The patient had no evidence of disease recurrence at 3 years follow-up. CONCLUSION Inflammatory myofibroblastic tumors of the pancreas in children are extremely rare. Surgical excision is the standard treatment, and corticosteroids use in children need more large-scale studies.
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Affiliation(s)
| | | | | | - Yaw-Sen Chen
- Department of Surgery, E-Da Hospital, I-Shou University, Yanchao District, Kaohsiung, Taiwan
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Barral M, Faraoun S, Fishman E, Dohan A, Pozzessere C, Berthelin MA, Bazeries P, Barat M, Hoeffel C, Soyer P. Imaging features of rare pancreatic tumors. Diagn Interv Imaging 2016; 97:1259-1273. [DOI: 10.1016/j.diii.2016.07.013] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2016] [Revised: 07/11/2016] [Accepted: 07/18/2016] [Indexed: 02/07/2023]
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Odagiri S, Tokuhara D, Nishigaki S, Cho Y, Shintaku H. Double pancreatic tumors in an adolescent: Imaging features. Pediatr Int 2016; 58:1239-12342. [PMID: 27882732 DOI: 10.1111/ped.13142] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2015] [Revised: 06/23/2016] [Accepted: 08/15/2016] [Indexed: 11/29/2022]
Abstract
Insulinoma is generally identified as a single tumor and seldom occurs in children or adolescents. A 14-year-old girl with difficulty in waking was found to have hyperinsulinemic hypoglycemia. On abdominal ultrasonography two hypoechoic masses (8 and 12 mm in diameter) were seen in the pancreatic body: the larger mass was hypervascular, whereas the smaller one was hypovascular. Contrast-enhanced computed tomography showed enhancement of the larger mass, but did not delineate the smaller mass. On fat-suppressed T1-weighted magnetic resonance imaging, the larger mass was hypointense, but the smaller mass was hyperintense. Pathologically, the larger tumor was normal density, insulin positive, and rich in vascularity, whereas the smaller tumor was high density, insulin negative, and poor in vascularity. The present case suggests that difficulty waking should be considered as a potential etiology in insulinoma, and multiple suspected pancreatic insulinomas should be evaluated using a combination of imaging modalities to characterize each tumor.
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Affiliation(s)
- Shino Odagiri
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Daisuke Tokuhara
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Satsuki Nishigaki
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Yuki Cho
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Haruo Shintaku
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
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Marsman EM, de Rooij T, van Eijck CH, Boerma D, Bonsing BA, van Dam RM, van Dieren S, Erdmann JI, Gerhards MF, de Hingh IH, Kazemier G, Klaase J, Molenaar IQ, Patijn GA, Scheepers JJ, Tanis PJ, Busch OR, Besselink MG. Pancreatoduodenectomy with colon resection for cancer: A nationwide retrospective analysis. Surgery 2016; 160:145-152. [DOI: 10.1016/j.surg.2016.02.022] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2015] [Revised: 02/18/2016] [Accepted: 02/21/2016] [Indexed: 01/02/2023]
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