Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreas

While neuroendocrine tumors of the pancreas are increasing in incidence, neural derived tumors are only rarely found in this organ. Paragangliomas, neuronal and nerve sheath tumors of the pancreas are limited to small series or case reports. The newly named composite gangliocytoma/neuroma and neuroe...

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Main Authors: Binny Khandakar, Moreen Ng, Laura Baum, Marie E. Robert
Format: Article
Language:English
Published: Elsevier 2024-11-01
Series:Human Pathology Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2772736X24000355
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author Binny Khandakar
Moreen Ng
Laura Baum
Marie E. Robert
author_facet Binny Khandakar
Moreen Ng
Laura Baum
Marie E. Robert
author_sort Binny Khandakar
collection DOAJ
description While neuroendocrine tumors of the pancreas are increasing in incidence, neural derived tumors are only rarely found in this organ. Paragangliomas, neuronal and nerve sheath tumors of the pancreas are limited to small series or case reports. The newly named composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET), formerly gangliocytic paraganglioma, occurs almost exclusively in the second part of the duodenum and periampullary region but has been reported in the pancreas. At the time of this writing four pancreatic CoGNETs have been reported in the pancreatic head, ranging in size from 2.2 to 5.3 cm; three in females, with a mean age of 63 years (range, 50–74 years). Herein, we report the clinico-pathologic findings of a 58-year-old male presenting with a low-grade neuroendocrine tumor showing focal gangliocytic differentiation within the head of pancreas that does not fit precisely into existing tumor classifications, having some but not all elements of CoGNET. The morphology and immunohistochemical profile of the tumor are presented in the context of the differential diagnosis and prior literature.
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spelling doaj-art-4bc3582a2e3a4da0b857e7aacc1b989b2025-08-20T02:39:28ZengElsevierHuman Pathology Reports2772-736X2024-11-013830076310.1016/j.hpr.2024.300763Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreasBinny Khandakar0Moreen Ng1Laura Baum2Marie E. Robert3Pathology and Laboratory Medicine, Donald Barbara Zucker School of Medicine at Hofstra/Northwell Health, Northwell Health Laboratories, Greenvale, NY, United StatesYale School of Medicine, New Haven, CT, United StatesDivision of Medical Oncology, Department of Medicine, Yale School of Medicine, New Haven, CT, United StatesDepartment of Pathology, Yale School of Medicine, New Haven, CT, United States; Corresponding author.While neuroendocrine tumors of the pancreas are increasing in incidence, neural derived tumors are only rarely found in this organ. Paragangliomas, neuronal and nerve sheath tumors of the pancreas are limited to small series or case reports. The newly named composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET), formerly gangliocytic paraganglioma, occurs almost exclusively in the second part of the duodenum and periampullary region but has been reported in the pancreas. At the time of this writing four pancreatic CoGNETs have been reported in the pancreatic head, ranging in size from 2.2 to 5.3 cm; three in females, with a mean age of 63 years (range, 50–74 years). Herein, we report the clinico-pathologic findings of a 58-year-old male presenting with a low-grade neuroendocrine tumor showing focal gangliocytic differentiation within the head of pancreas that does not fit precisely into existing tumor classifications, having some but not all elements of CoGNET. The morphology and immunohistochemical profile of the tumor are presented in the context of the differential diagnosis and prior literature.http://www.sciencedirect.com/science/article/pii/S2772736X24000355PancreasNeuroendocrine tumorComposite gangliocytoma/neuroma and neuroendocrine tumorGangliocytic paraganglioma
spellingShingle Binny Khandakar
Moreen Ng
Laura Baum
Marie E. Robert
Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreas
Human Pathology Reports
Pancreas
Neuroendocrine tumor
Composite gangliocytoma/neuroma and neuroendocrine tumor
Gangliocytic paraganglioma
title Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreas
title_full Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreas
title_fullStr Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreas
title_full_unstemmed Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreas
title_short Between ‘NET’ and ‘CoGNET’: A biphasic Neuroendocrine-Gangliocytic tumor of the pancreas
title_sort between net and cognet a biphasic neuroendocrine gangliocytic tumor of the pancreas
topic Pancreas
Neuroendocrine tumor
Composite gangliocytoma/neuroma and neuroendocrine tumor
Gangliocytic paraganglioma
url http://www.sciencedirect.com/science/article/pii/S2772736X24000355
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