Neuzillet Cindy, Vullierme Marie-Pierre, Couvelard Anne, Sauvanet Alain, Levy Philippe, Richard Stéphane, Ruszniewski Philippe, Hammel Pascal
Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, Clichy, France.
J Comput Assist Tomogr. 2010 Jan;34(1):140-5. doi: 10.1097/RCT.0b013e3181b52b84.
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.
冯·希佩尔-林道病是一种累及多器官的进行性常染色体显性疾病。冯·希佩尔-林道病的胰腺病变有两种类型：囊性病变和胰腺内分泌肿瘤。只有后一种类型有潜在恶性可能，可能需要行胰腺切除术。这两种病变的鉴别诊断可能具有挑战性。我们报告3例患有非典型胰腺囊性病变的患者，他们因怀疑患有恶性肿瘤而接受了手术。本文讨论了病理与放射学的相关性以及诊断和管理策略。
