Difficult diagnosis of atypical cystic pancreatic lesions in von Hippel-Lindau disease.
von Hippel-Lindau disease is a progressive, autosomal dominant disorder with multiorgan involvement. There are 2 types of pancreatic lesions from von Hippel-Lindau disease: cystic lesions and endocrine pancreatic tumors. Only the latter type is potentially malignant and may justify pancreatic resection. The differential diagnosis between these 2 types of lesions can be difficult. We report 3 patients with atypical cystic pancreatic lesions who underwent surgery for suspected malignant tumors. The pathological-radiological correlations and the diagnostic and management strategies are discussed.
Neuzillet
Cindy
C
Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, Clichy, France.
Vullierme
Marie-Pierre
MP
Couvelard
Anne
A
Sauvanet
Alain
A
Levy
Philippe
P
Richard
Stéphane
S
Ruszniewski
Philippe
P
Hammel
Pascal
P
D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007565:Jaundice / Q000209:etiology; D008279:Magnetic Resonance Imaging / Q000379:methods; D010179:Pancreas / Q000000981:diagnostic imaging / Q000473:pathology; D010182:Pancreatic Diseases / Q000175:diagnosis / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D010195:Pancreatitis / Q000209:etiology; D014057:Tomography, X-Ray Computed / Q000379:methods; D014463:Ultrasonography; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000601:surgery