Sharma Ayush, Mukewar Saurabh, Vege Santhi Swaroop
From the Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN.
Pancreas. 2017 Aug;46(7):948-952. doi: 10.1097/MPA.0000000000000875.
Little is known about the association between pancreatic cystic lesions (PCLs) with von Hippel-Lindau disease (VHLD). In this study, we describe the clinical presentation, type of PCLs, and risk for malignancy in PCLs in VHLD.
Patients given a diagnosis of both VHLD and PCLs were identified from electronic medical records at Mayo Clinic, Rochester, MN, from January 1, 2000, to January 1, 2016. Various demographic, clinical, and radiologic variables were recorded.
Forty-eight patients were identified with PCLs and VHLD. Most were female (73%), and mean (standard deviation) age was 35.9 (14.2) years. Most (92%) were asymptomatic. PCLs included simple cysts (n = 34, 69%), serous cystadenoma (n = 14, 29%), branch duct intraductal papillary neoplasm (n = 5, 10%), and cystic neuroendocrine tumors (n = 2, 4%). Eight (19%) had mixed PCLs. Cyst aspiration was performed in 8 (53%) patients who underwent EUS, and all were negative for malignancy. At a median follow-up of 84 months, no PCL-associated cancers were seen.
Simple cyst is the most common type of PCL found in VHL disease; however, other PCLs were also seen with no malignancy potential. Branch duct intraductal papillary mucinous neoplasms were present in 10%, and this association was not hitherto reported.
胰腺囊性病变（PCLs）与冯·希佩尔-林道病（VHLD）之间的关联鲜为人知。在本研究中，我们描述了VHLD患者中PCLs的临床表现、类型以及发生恶性肿瘤的风险。
从明尼苏达州罗切斯特市梅奥诊所2000年1月1日至2016年1月1日的电子病历中识别出同时诊断为VHLD和PCLs的患者。记录了各种人口统计学、临床和放射学变量。
共识别出48例患有PCLs和VHLD的患者。大多数为女性（73%），平均（标准差）年龄为35.9（14.2）岁。大多数（92%）无症状。PCLs包括单纯囊肿（n = 34，69%）、浆液性囊腺瘤（n = 14，29%）、分支导管内乳头状瘤（n = 5，10%）和囊性神经内分泌肿瘤（n = 2，4%）。8例（19%）为混合性PCLs。8例（53%）接受超声内镜检查（EUS）的患者进行了囊肿抽吸，所有结果均为恶性阴性。中位随访84个月，未发现与PCL相关的癌症。
单纯囊肿是VHL病中最常见的PCL类型；然而，也发现了其他无恶性潜能的PCL。10%的患者存在分支导管内乳头状黏液性肿瘤，此前尚未有此关联的报道。
