[Variety of pancreatic lesions observed in von Hippel-Lindau disease. Apropos of 8 cases].
Von Hippel-Lindau's disease is a rare genetic disease, with an autosomal dominant mode of inheritance, characterised by the development of several tumours, such as haemangioblastoma of the central nervous system and retina, renal cysts or carcinoma and pheochromocytoma. Several pancreatic lesions, mainly represented by multiple cysts, are also encountered. We report here 8 cases of pancreatic involvement in patients affected with von Hippel-Lindau's disease. It consisted of multiple cysts (3 cases), serous cystadenoma (2 cases), endocrine tumour (1 case), haemangioblastoma (1 case) and ductal adenocarcinoma (1 case). Diagnosis of a rare lesion of the pancreas (multiple cysts, serous cystadenoma or vascularized tumour) in a young patient may lead to search the other lesions of von Hippel-Lindau's disease and to undergo a familial inquiry in order to propose a multidisciplinary approach for patients affected by the disease.
Hammel
P
P
Service d'Hépatologie et de Gastroentérologie, Hôpital Henri-Mondor, Créteil.
Beigelman
C
C
Chauveau
D
D
Resche
F
F
Bougerolles
E
E
Flejou
J F
JF
Bernades
P
P
Delchier
J C
JC
Richard
S
S
D000230:Adenocarcinoma / Q000209:etiology*; D000328:Adult; D018293:Cystadenoma, Serous / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D016145:Endoscopy, Digestive System; D017809:Fatal Outcome; D005260:Female; D018325:Hemangioblastoma / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010181:Pancreatic Cyst / Q000000981:diagnostic imaging / Q000209:etiology*; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000235:genetics
Variété des lésions pancréatiques observées au cours de la maladie de von Hippel-Lindau. Présentation de 8 cas.