臨床外科70巻12号

2015年11月発行

特集 同時性・異時性の重複がんを見落とさない—がん診療における他臓器への目配り 同時性・異時性重複がんの実態と診療戦略

膵・消化管NET

著者： 工藤篤¹ 伴大輔¹ 上田浩樹¹ 千代延記道¹ 水野裕貴¹ 大畠慶映¹ 赤星径一¹ 大庭篤志¹ 伊藤浩光¹ 光法雄介¹ 松村聡¹ 藍原有弘¹ 落合高徳¹ 田中真二² 田邉稔¹

所属機関： ¹東京医科歯科大学肝胆膵外科学分野 ²東京医科歯科大学分子腫瘍医学分野

ページ範囲：P.1369 - P.1374

【ポイント】
◆多発性内分泌腫瘍症1型（MEN-1型），von Hippel-Lindau（VHL）病，神経線維腫症1型（NF-1），結節性硬化症（TSC）などは神経内分泌腫瘍（NET）関連疾患と呼ばれる．
◆NET関連疾患は常染色体優性遺伝で，良性・悪性腫瘍が時間的・空間的に多発し，遺伝子診断は早期発見・早期治療のサーベイランスに役立つだけでなく，重複癌や他疾患治療の優先順位に関係する．
◆NET関連疾患における膵・消化管NETの治療方針は，散発性NETと異なる．

参考文献

1）Sakurai A, Suzuki S, Kosugi S, et al：Multiple endocrine neoplasia type 1 in Japan：establishment and analysis of a multicentre database. Clin Endocrinol（Oxf）76：533-539, 2012

2）Ito T, Sasano H, Tanaka M, et al：Epidemiological study of gastroenteropancreatic neuroendocrine tumors in Japan. J Gastroenterol 45：234-243, 2010

3）Oberg K, Eriksson B：Endocrine tumours of the pancreas. Best Pract Res Clin Gastroenterol 19：753-781, 2005

4）Relles D, Baek J, Witkiewicz A, et al：Periampullary and duodenal neoplasms in neurofibromatosis type 1：two cases and an updated 20-year review of the literature yielding 76 cases. J Gastrointest Surg 14：1052-1061, 2010

5）Verhoef S, van Diemen-Steenvoorde R, Akkersdijk WL, et al：Malignant pancreatic tumour within the spectrum of tuberous sclerosis complex in childhood. Eur J Pediatr 158：284-287, 1999

6）Rindi G：Clinicopathologic aspects of gastric neuroendocrine tumors. Am J Surg Pathol 19（Suppl 1）：S20-29, 1995

7）Sakurai A, Shirahama S, Fujimori M, et al：Novel MEN1 gene mutations in familial multiple endocrine neoplasia type 1. J Hum Genet 43：199-201, 1998

8）Brandi ML, Gagel RF, Angeli A, et al：Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86：5658-5671, 2001

9）Sakurai A, Yamazaki M, Suzuki S, et al：Clinical features of insulinoma in patients with multiple endocrine neoplasia type 1：analysis of the database of the MEN Consortium of Japan. Endocr J 59：859-866, 2012

10）Gauger PG, Doherty GM, Broome JT, et al：Completion pancreatectomy and duodenectomy for recurrent MEN-1 pancreaticoduodenal endocrine neoplasms. Surgery 146：801-806；discussion 807-808, 2009

11）Kouvaraki MA, Shapiro SE, Cote GJ, et al：Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg 30：643-653, 2006

12）Fraker DL, Norton JA, Alexander HR, et al：Surgery in Zollinger-Ellison syndrome alters the natural history of gastrinoma. Ann Surg 220：320-328；discussion 328-330, 1994

13）Norton JA, Fraker DL, Alexander HR, et al：Surgery increases survival in patients with gastrinoma. Ann Surg 244：410-419, 2006

14）Bartsch DK, Fendrich V, Langer P, et al：Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. Ann Surg 242：757-764, discussion 764-766, 2005

15）Gibril F, Venzon DJ, Ojeaburu JV, et al：Prospective study of the natural history of gastrinoma in patients with MEN1：definition of an aggressive and a nonaggressive form. J Clin Endocrinol Metab 86：5282-5293, 2001

16）Maeda H, Hanazaki K：Pancreatogenic diabetes after pancreatic resection. Pancreatology 11：268-276, 2011

17）Frilling A, Modlin IM, Kidd M, et al：Recommendations for management of patients with neuroendocrine liver metastases. Lancet Oncol 15：e8-21, 2014

18）Tory K, Brauch H, Linehan M, et al：Specific genetic change in tumors associated with von Hippel-Lindau disease. J Natl Cancer Inst 81：1097-1101, 1989

19）Latif F, Tory K, Gnarra J, et al：Identification of the von Hippel-Lindau disease tumor suppressor gene. Science 260：1317-1320, 1993

20）Cascon A, Escobar B, Montero-Conde C, et al：Loss of the actin regulator HSPC300 results in clear cell renal cell carcinoma protection in Von Hippel-Lindau patients. Hum Mutat 28：613-621, 2007

21）Blansfield JA, Choyke L, Morita SY, et al：Clinical, genetic and radiographic analysis of 108 patients with von Hippel-Lindau disease（VHL）manifested by pancreatic neuroendocrine neoplasms（PNETs）. Surgery 142：814-818；discussion 818 e1-2, 2007

22）Maher ER, Yates JR, Harries R, et al：Clinical features and natural history of von Hippel-Lindau disease. Q J Med 77：1151-1163, 1990

23）Walther MM, Choyke PL, Glenn G, et al：Renal cancer in families with hereditary renal cancer：prospective analysis of a tumor size threshold for renal parenchymal sparing surgery. J Urol 161：1475-1479, 1999