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Presented by Ralph Hruban, M.D. and prepared by Jeremy Vincent M.D.
Case 3: This patient with neurofibromatosis underwent a Whipple resection for a mass centered in the duodenum.
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1. Question
Week 508: Case 3
This patient with neurofibromatosis underwent a Whipple resection for a mass centered in the duodenum.images/1Alex/123450case601.jpg
images/1Alex/123450case602.jpg
images/1Alex/123450case603.jpgCorrect
Answer: Neurofibromatosis-associated vasculopathy
Histology: Many of the arteries in this pancreas show a peculiar neointimal thickening.
Discussion: Neurofibromatosis can be associated with a number of vascular changes. The neurofibromatosis-associated vasculopathy seen in this case is characterized by a peculiar neointimal thickening. The changes were seen immediately adjacent to the somatostatinoma (case 2) and in the surrounding normal pancreas. It is thought that the vasculopathy is related to NF-1 with abnormal neurofibromin possibly playing a role
Reference(s):
– Vasculopathic changes, a somatostatin-producing neuroendocrine carcinoma and a jejunal gastrointestinal stromal tumor in a patient with type-1 neurofibromatosis. Chetty R, Vajpeyi R. Endocr Pathol. 2009 Fall;20(3):177-81. PMID: 19488862Incorrect
Answer: Neurofibromatosis-associated vasculopathy
Histology: Many of the arteries in this pancreas show a peculiar neointimal thickening.
Discussion: Neurofibromatosis can be associated with a number of vascular changes. The neurofibromatosis-associated vasculopathy seen in this case is characterized by a peculiar neointimal thickening. The changes were seen immediately adjacent to the somatostatinoma (case 2) and in the surrounding normal pancreas. It is thought that the vasculopathy is related to NF-1 with abnormal neurofibromin possibly playing a role
Reference(s):
– Vasculopathic changes, a somatostatin-producing neuroendocrine carcinoma and a jejunal gastrointestinal stromal tumor in a patient with type-1 neurofibromatosis. Chetty R, Vajpeyi R. Endocr Pathol. 2009 Fall;20(3):177-81. PMID: 19488862
