[Pancreatic neuroendocrine tumors and von Hippel-Lindau disease]. / Tumeurs neuro-endocrines du pancréas et maladie de von Hippel-Lindau.
Ann Pathol
; 20(2): 130-3, 2000 Mar.
Article
en Fr
| MEDLINE
| ID: mdl-10740008
ABSTRACT
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.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Pancreáticas
/
Tumores Neuroendocrinos
/
Enfermedad de von Hippel-Lindau
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Female
/
Humans
Idioma:
Fr
Revista:
Ann Pathol
Año:
2000
Tipo del documento:
Article
País de afiliación:
Francia