Woo Chang Gok, Choi Seo-Youn, Kwak Jeong Ja, Chin Susie, Kim Hee Kyung
Department of Pathology, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju, Republic of Korea.
Department of Radiology, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Republic of Korea.
Neuro Endocrinol Lett. 2017 May;38(2):83-86.
Clear cell neuroendocrine tumor (NET) of the pancreas is found in von Hippel-Lindau (VHL) disease, multiple endocrine neoplasia type I (MEN I), and sporadic form. Clear cell NETs are often misdiagnosed as metastatic renal cell carcinoma. A 47-year-old woman with VHL was found to have a mass in the pancreatic tail and two masses in the right kidney with two cysts. A distal pancreatectomy and right radical nephrectomy were performed. The pancreatic lesion was a well-circumscribed, golden-yellow solid mass, which was lobulated by septal fibrosis. Microscopically, the tumor consisted of entirely of clear cells with prominent nucleoli. The tumor cell nests were separated by collagen fibrosis. Immunohistochemical studies were positive for the neuroendocrine markers and vimentin. Synchronous kidney tumors were clear cell renal cell carcinoma and cystic renal cell carcinoma. Those with syndrome are younger than those without syndrome. Sporadic tumors have larger size and higher grade than those of VHL and MEN I. Stromal sclerosis is frequently observed in VHL, compared with the other two groups. In the absence of a documented genetic profile and family history, awareness of these features should help us to diagnose clear cell pancreatic NETs resembling metastatic renal cell carcinoma.
胰腺透明细胞神经内分泌肿瘤（NET）见于冯·希佩尔-林道（VHL）病、I型多发性内分泌腺瘤病（MEN I）以及散发性病例。透明细胞NET常被误诊为转移性肾细胞癌。一名47岁患有VHL病的女性被发现胰腺尾部有一肿块，右肾有两个肿块及两个囊肿。实施了远端胰腺切除术和右根治性肾切除术。胰腺病变为边界清楚的金黄色实性肿块，被间隔纤维化分成小叶状。显微镜下，肿瘤完全由具有明显核仁的透明细胞组成。肿瘤细胞巢被胶原纤维分隔。免疫组化研究显示神经内分泌标志物和波形蛋白呈阳性。同时存在的肾肿瘤为透明细胞肾细胞癌和囊性肾细胞癌。患有综合征的患者比未患综合征的患者年龄小。散发性肿瘤比VHL病和MEN I的肿瘤体积更大、分级更高。与其他两组相比，VHL病中经常观察到间质硬化。在没有记录在案的基因谱和家族史的情况下，了解这些特征应有助于我们诊断类似转移性肾细胞癌的胰腺透明细胞NET。
