Pancreatic involvement in von Hippel-Lindau disease
Pancreatic involvement in von Hippel-Lindau disease
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DOI：
10.1053/gast.2000.18143
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发表时间：
2000-10-01
期刊：
影响因子：
29.4
通讯作者：
Richard, S
中科院分区：
文献类型：
--
作者：
Hammel, PR;Vilgrain, V;Richard, S
背景和目标：血管性希佩尔-林道病（VHL）是一种影响多种器官的显性遗传性疾病，胰腺受累很少被研究。我们评估了VHL患者胰腺受累的患病率、病变类型、自然史和影响。在法国的一项前瞻性合作研究中，共对来自94个VHL疾病家族的158名连续患者进行了研究，所有患者均接受VHL病变的系统筛查，包括由经验丰富的放射科医生审查的胰腺计算机断层扫描（CT），还审查了临床数据、检查和治疗，结果：胰腺受累122例（77.2%），包括真性囊肿浆液性囊腺瘤91.1%（12.3%），神经内分泌肿瘤（12.3%），或合并病变胰腺是7.6%患者的唯一受累器官，有胰腺病变的患者发生嗜铬细胞瘤的比例比无胰腺病变的患者少（14/122 vs，16/36; P < 0.0001），并且胰腺神经内分泌肿瘤患者的静脉受累率低于无神经内分泌肿瘤患者（8/99 vs，6/20; P = 0.013），神经内分泌肿瘤患者无激素分泌过多症状。胰腺病变的一半的患者，但需要特定的治疗只有10（8.2%），当他们有症状或切除大型神经内分泌肿瘤，结论：胰腺受累是在大多数VHL疾病的患者。虽然症状罕见，但在选定的患者中，主要是神经内分泌肿瘤患者，需要对胰腺病变进行特异性治疗。
Background & Aims: Pancreatic involvement in von Hippel-Lindau (VHL) disease, agenetic disorder with a dominant mode of inheritance affecting various organs, has rarely been studied, We assessed the prevalence, type of lesions, natural history, and impact of pancreatic involvement In patients with VHL, Methods: A total of 158 consecutive patients from 94 families with VHL disease were studied in a prospective French collaborative study, All patients underwent systematic screening for VHL lesions, including computerized tomography (CT) scanning of the pancreas reviewed by an experienced radiologist, Clinical data, investigations, and treatments performed were also reviewed, Results: Pancreatic involvement was observed in 122 patients (77.2%) and included true cysts (91.1%),serous cystadenomas (12.3%), neuroendocrine tumors (12.3%), or combined lesions (11.5%), The pancreas was the only organ affected in 7.6% of patients, Patients with pancreatic lesions had fewer pheochromocytomas than those without (14/122 vs, 16/36; P < 0.0001), and patients with neuroendocrine pancreatic tumors had venal involvement less often than those without (8/99 vs, 6/20; P = 0.013), None of the patients With neuroendocrine tumors had symptoms of hormonal hypersecretion. Pancreatic lesions evolved in half of patients but required specific treatment in only 10 (8.2%) when they were symptomatic or for the resection of large neuroendocrine tumors, Conclusions: Pancreatic involvement is seen in most patients with VHL disease. Although symptoms are rare, specific treatment of pancreatic lesions is required in selected patients, mainly those with neuroendocrine tumors.