Kogire M, Hosotani R, Kondo M, Itoh K, Doi R, Terachi T, Imamura M
Department of Surgery and Surgical Basic Science, Graduate School of Medicine, Kyoto University, Japan.
Surg Today. 2000;30(4):380-2. doi: 10.1007/s005950050605.
Multiple cysts and benign cystadenomas of the pancreas have been documented occasionally in von Hippel-Lindau syndrome (HLS); however, the malignant involvement of the pancreas in HLS is very rare. We report a case of HLS in which metastatic tumors from renal cell carcinoma (RCC) coexisted with multiple cysts in the pancreas. A 22-year-old woman with a history of HLS had undergone a partial resection of the left kidney for RCC 3 years earlier, at which time a solid mass in the pancreatic tail and multiple pancreatic cysts were also incidentally detected by computed tomography. Over the following 3 years, the mass enlarged slightly, thus raising suspicions that it might be a primary neoplasm of the pancreas. She was referred to the Department of Surgery and Surgical Basic Science to undergo surgery. In addition to the tumor in the pancreatic tail, however, further tumors in the pancreatic head were also disclosed by preoperative celiac arteriography and intraoperative palpation and ultrasonography. A distal pancreatectomy was performed, because the enucleation of all the tumors in the pancreatic head was technically impossible and because the patient declined a total pancreatectomy. A histologic examination of the mass in the pancreatic tail revealed metastatic RCC. This case emphasizes that metastatic disease should be included in the differential diagnosis when evaluating the pancreas in a patient with HLS.
在冯·希佩尔-林道综合征（HLS）中，偶尔会有胰腺多发囊肿和良性囊腺瘤的记录；然而，HLS中胰腺发生恶性病变的情况非常罕见。我们报告一例HLS病例，其中肾细胞癌（RCC）转移瘤与胰腺多发囊肿并存。一名有HLS病史的22岁女性在3年前因RCC接受了左肾部分切除术，当时通过计算机断层扫描偶然发现胰尾有一个实性肿块和多个胰腺囊肿。在接下来的3年里，肿块略有增大，因此怀疑它可能是胰腺原发性肿瘤。她被转诊至外科和外科基础科学部接受手术。然而，除了胰尾的肿瘤外，术前腹腔动脉造影以及术中触诊和超声检查还发现胰头有更多肿瘤。由于在技术上无法摘除胰头的所有肿瘤，且患者拒绝接受全胰切除术，因此进行了远端胰腺切除术。对胰尾肿块的组织学检查显示为转移性RCC。该病例强调，在评估HLS患者的胰腺时，鉴别诊断应包括转移性疾病。
