[Pancreatic neuroendocrine tumors and von Hippel-Lindau disease].
Pancreatic neuroendocrine tumors are rare in von Hippel-Lindau disease, most often asymptomatic, nonfunctioning, non secreting, and benign. We report a case of low grade malignant pancreatic, secreting and asymptomatic neuroendocrine tumors, occurring in a 27 year old woman in the setting von Hippel-Lindau disease with recurrent pheochromocytoma, retinal and medullary hemangioblastomas, paraganglioma of the carotid body and ovarian cystadenoma. Neuroendocrine pancreatic tumors of von Hippel-Lindau disease are often constituted by clear cells, in the contrary of other neuroendocrine tumors of the pancreas. Occurrence of a pancreatic neuroendocrine tumor, especially in association with pheochromocytoma, may be misdiagnosed with a type 2 multiple endocrine neoplasia syndrom instead of von Hippel-Lindau disease.
Musso
C
C
Service d'Anatomie Pathologique, Centre Hospitalier Régional Universitaire Dupuytren, 87042 Limoges, France.
Paraf
F
F
Petit
B
B
Archambeaud-Mouveroux
F
F
Valleix
D
D
Labrousse
F
F
D000310:Adrenal Gland Neoplasms / Q000473:pathology; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D016609:Neoplasms, Second Primary / Q000473:pathology; D018358:Neuroendocrine Tumors / Q000150:complications / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology*; D010673:Pheochromocytoma / Q000473:pathology; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000473:pathology*