两阶段切除伴有冯·希佩尔-林道病患者的双侧嗜铬细胞瘤和胰腺神经内分泌肿瘤：一例报告

Two-stage resection of a bilateral pheochromocytoma and pancreatic neuroendocrine tumor in a patient with von Hippel-Lindau disease: A case report.

作者信息

Endo Yutaka, Kitago Minoru, Miyajima Akira, Kurihara Isao, Kameyama Kaori, Shinoda Masahiro, Yagi Hiroshi, Abe Yuta, Hibi Taizo, Takagi Chisato, Nakano Yutaka, Koizumi Wataru, Itano Osamu, Kitagawa Yuko

机构信息

Department of Surgery, Keio University School of Medicine, Tokyo, Japan.

出版信息

Int J Surg Case Rep. 2018;44:139-142. doi: 10.1016/j.ijscr.2018.02.020. Epub 2018 Feb 16.

DOI:10.1016/j.ijscr.2018.02.020

PMID:29501019

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5910515/

Abstract

INTRODUCTION

von Hippel-Lindau disease (vHL disease) is a hereditary disease in which tumors and cysts develop in many organs, in association with central nervous system hemangioblastomas, pheochromocytomas, and pancreatic tumors. We herein report a case of vHL disease (type 2A) associated with bilateral pheochromocytomas, pancreatic neuroendocrine tumors (PNET), and cerebellar hemangioblastomas treated via pancreatectomy after adrenalectomy.

CASE PRESENTATION

A 51-year-old woman presented with a cerebellar tumor, bilateral hypernephroma, and pancreatic tumor detected during a medical checkup. 18F-fluorodeoxyglucose positron emission tomography-computed tomography revealed a bilateral adrenal gland tumor and a tumor in the head of the pancreas, while an abdominal computed tomography examination revealed a 30-mm tumor with strong enhancement in the head of the pancreas. Cranial magnetic resonance imaging showed a hemangioblastoma in the cerebellum. Therefore, a diagnosis of vHL disease (type 2A) was made. Her family medical history included renal cell carcinoma in her father and bilateral adrenal pheochromocytoma and spinal hemangioblastoma in her brother. A detailed examination of endocrine function showed that the adrenal mass was capable of producing catecholamine. Treatment of the pheochromocytoma was prioritized, and therefore, laparoscopic left adrenalectomy and subtotal resection of the right adrenal gland were performed. Once the postoperative steroid levels were replenished, subtotal stomach-preserving pancreatoduodenectomy was performed for the PNET. After a good postoperative course, the patient was discharged in remission on the 11th day following surgery. Histopathological examination findings indicated NET G2 (MIB-1 index 10-15%) pT3N0M0 Stage II A and microcystic serous cystadenoma throughout the resected specimen. The patient is scheduled to undergo treatment for the cerebellar hemangioblastoma.

CONCLUSION

A two-staged resection is a safe and effective treatment option for bilateral pheochromocytoma and PNET associated with vHL disease.

摘要

引言

冯·希佩尔-林道病（vHL病）是一种遗传性疾病，许多器官会出现肿瘤和囊肿，并伴有中枢神经系统血管母细胞瘤、嗜铬细胞瘤和胰腺肿瘤。我们在此报告一例2A型vHL病，伴有双侧嗜铬细胞瘤、胰腺神经内分泌肿瘤（PNET）和小脑血管母细胞瘤，在肾上腺切除术后通过胰腺切除术进行治疗。

病例介绍

一名51岁女性在体检时发现小脑肿瘤、双侧肾肿瘤和胰腺肿瘤。18F-氟脱氧葡萄糖正电子发射断层扫描-计算机断层扫描显示双侧肾上腺肿瘤和胰头肿瘤，而腹部计算机断层扫描检查显示胰头有一个30毫米的肿瘤，强化明显。头颅磁共振成像显示小脑有血管母细胞瘤。因此，诊断为2A型vHL病。她的家族病史包括父亲患肾细胞癌，哥哥患双侧肾上腺嗜铬细胞瘤和脊髓血管母细胞瘤。详细的内分泌功能检查显示肾上腺肿块能够产生儿茶酚胺。优先治疗嗜铬细胞瘤，因此，进行了腹腔镜左肾上腺切除术和右肾上腺次全切除术。术后补充类固醇水平后，对PNET进行了保留胃的胰十二指肠次全切除术。术后恢复良好，患者在术后第11天缓解出院。组织病理学检查结果显示为NET G2（MIB-1指数10-15%）pT3N0M0 II A期，整个切除标本为微囊性浆液性囊腺瘤。患者计划接受小脑血管母细胞瘤的治疗。