R EVIEW A RTICLES 膵 消化管神経内分泌腫瘍 (NET) 最近の動向 100 Obendorfer cancer like lesion Neuroendocrine tumor NET NET adenoma-carcinoma sequence NET NET 1 NET は稀な

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1 膵 消化管神経内分泌腫瘍 () 最近の動向 100 Obendorfer cancer like lesion Neuroendocrine tumor adenoma-carcinoma sequence 1 は稀な疾患か? Yao Work Japan midgut 1/31/10 2 WHO 2000 cancer like lesions 3 国際的な病理分類の改定 膵 消化管 N E T は良性腫瘍か? adenomacarcinoma sequence 39

2 6060 SASI test SRS, 4 機能性膵 消化管 の早期発見と治癒的治療は可能か? mm 1980 SRS SASI test -11 SRS SASI test SRS MEN 1 10, 11 VIP VIP 5 incidentaloma cm cm 12 6 非機能性膵 消化管 の治療はどうするのが良いか? MEN 1 患者に発生する膵 消化管 MEN 1 SASI test MEN 1 60 VIPoma MEN 1 Mayo Clinic EU 20 40

3 WHO MEN 1 (PD) No. E Z S MEN 1 PD PD Gastrinoma IRG pg/mll SASI GIF CT secretin test pg/mll N mm DU GU perf JU 1990 GX 1982 JX DU, GU T Nov T IPDA X PD IPDA PD, PX St ParX 1989 Mar 1990 Mar 1991 Mar 1991 Mar 1991 Nov 583 PD 1997 Aug X 1997 Oct D D D T, P GIF; IRG; N; DU; GU; JU; perf; GX; JX; ; St ParX; ; IPDA; PX; D; P; Dsmt; MEN 1 No. E Z S MEN 1 Gastrinoma pg/ml SASI GIF CT pg/ml N mm GU 1984 JU 1995 GX 1984 T 3,180 DX, DP, St ParX 1996.Sep 50 D TParX 2004 Jul (gluc) T2006 Oct1310 secretin test GU 400 St Par X 1984.Apr 1974 GX JU 1975 JX DX 1996.Apr D post Par X DX 2004.Nov D PX 1997.Feb DU St Par X 1999.Jul DX 2001.Jan D post Par X DX 2007.Jan D PPPD 1993.Jan GU StPar X 1993.Apr Dsmt DX 2001.Apr D ParX ,240 Dsmt 44 D St ParX 2003 May (gluc) DX, DP 2003.Jul Ins DP GU DU D 2 6, T 49,500 Dsmt Dsmt TParX, TX 1989 Gsmt X 1989, 1995 PH 1 15 Gsmt NMets X 2009 Feb GIF; IRG; N; postop; GU; JU; DU; GX; JX; ; Ins; ParX; ; Dsmt; PX; N; DX; StParx; TParx; TX; PPPD; DP; D; gluc; glucagonomamets; P(H); ; 41

4 MEN 1 No. E Z S MEN 1 PPTD PPTD Gastrinoma PPTD pg/ml SASI GIF CT pg/ml mm secretin test 54, DU 1997.Dec -, 18,200 IPDA #13 #6 St ParX LN PPTD, DP 2003.Apr 216 D 2003.Nov ;gluc IRG 900 post ParX 820 TParX, TX 2004.Apr , 110 D post ParX IPDA PPTD, DP 2004.Jul uncus.tail DU 2004.Mar - 3,050, Dsmt TParX, TX 710 IPDA PPTD, DP 2003.Aug 57 D Aug post ParX Dsmt #13 LN IPDA, P PPTD DPA <3mmTParX, TX 2007.Apr 59 D 2007.Sep D-EUS diff JU perf 2007.Jan Dsmt 13,900 post ParX #17 LN TParX, TX Dsmt PPTD 2001.Dec N 255 D Nov IRG 371 D-EUS DU perf 2006.Jan JU perf 2006.Jul Ileus 2007.May JU perf 2008.Jul Es bleeding DU JU perf JU perf GX 720 JX, 646 IPDA n.d. TParX, TX PPTD, TG 2008.Aug 42 D post ParX 2002.Oct 1,630 DGA, SPA ParX,PPTD 17mm 2008.Aug 450 D, PT 2006.Jan JX 2008.Nov GX, JX 1,000 DGA, SPA DP, 2009 Jul 94 Pb mm 3, 3,10, >5mm GIF; IRG;, N: post ParX; DU; JU; perf; Es; GX; JX; ; ; IPDA; DPA; SPA; Dsmt; D-EUS; LN; st ParX; TParX; TX; DP; PX; D; gluc; glucagonommet; MD Anderson MEN 1, cm QOL, 7 肝転移などの遠隔転移巣をどのように治療するか? 90, 14 RFARadio-frequency ablation therapy TAE, streptozocin 5-FU Sandostatin LAR 16 mtor

5 8 P 9 膵 消化管 診療ガイドライン作成 終わりに Obendorfer S. Karzinoide Tumoren des Dunndarms. Frankf Z Path 1907; 1: Soga J. The term `Carcinoid` is a misnomer: the evidence of local invasion. J Exp Clin Cancer Res 2009;28:15 Yao JC, Hassan M, Phan A, et al. One hundred years after carcinoid : epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063 Ito T, Sasano H, Tanaka M, et al. Epidemiological study of gastroenteropancreatic neuroendocrine tumors in Japan. J Gastroenterol. 2010;45: Bettini R, Boninsegna L, Mantovani W, et al. Prognostic factors at diagnosis and value of WHO classification in a mono-institutional series of 180 non-functioning pancreatic endocrine tumors. Ann Oncol. 2008;19:903 Klöppel G. Tumor biology and histopathology of neuroendocrine tumors. Best Pract Res Clin Endocrinol Metab. 2007;21:15 Imamura M, Takahashi K, Adachi H, et al. Usefulness of selective arterial secretin injection test for localization of gastrinoma in the Zollinger-Ellison syndrome. Ann Surg. 1987;205:230 K r e n n i n g E P, K w e k k e b o o m D J, O e i H Y, e t a l. S o m a t o s t a t i n - r e c e p t o r s c i n t i g r a p h y i n gastroenteropancreatic tumors. An overview of European results. Ann N Y Acad Sci. 1994;733:416 Imamura M. Recent standardization of treatment strategy for patients with pancreatic neuroendocrine tumors. World J Gastroenterol 2010;16: Imamura M, Takahashi K, Isobe Y, et al. Curative resection of multiple gastrinomas aided by selective arterial secretin injection test and intraoperative secretin test. Ann Surg. 1989;210:710 11Doppman JL, Miller DL, Chang R, Shawker TH, Gorden P, Norton JA. Insulinomas: localization with selective arterial injection of calcium. Radiology. 1991;178:237 12Falconi M, Zer bi A, Crippa S, et al. Parenchymapreser ving resections for small nonfunctioning pancreatic endocrine tumors. Ann Surg Oncol. 2010; 17: Imamura M, Komoto I, Ota S, et al. Biochemically curative surgery for gastrinoma in multiple endocrine neoplasia type 1 patients. World J Gastroenterol. 2011;17: Chen H, Hardacre JM, Uzar A, Cameron JL. Isolated liver metastases from neuroendocrine tumors; does resection prolong survival? J Am Coll Surg. 1998;187:88 15Livraghi T, Solbiati L, Meloni MF, et al. Treatment of focal liver tumors with percutaneous radio-frequency ablation: complications encountered in a multicenter study. Radiology. 2003;226:441 16Oberg K, Ferone D, Kalsas G, et al. ES consensus guidelines for the standards of care in neuroendocrine tumors; biotherapy. Neuroendocrinology 2009;90:209 17Ekeblad S, Sundin A, Janson ET, et al. Temozolomide as monotherapy is effective in treatment of advanced malignant neuroendocrine tumors. Clin Cancer Res. 2007;13: Yao JC, Phan AT, Chang DZ, et al. Efficacy of RAD001 evelolimusand octreotide LAR in advanced low- to intermediate grade neuroendocrine tumors: results of a phase 2 study. J Clin Oncol 2008;26:

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ガストリノーマとは はじめに Zollinger と Ellison は 1955 年に難治性消化性潰瘍 胃酸分泌亢進を伴う膵島細胞腫瘍をはじめて報告 1) その後 Zollinger-Ellison 症候群と命名されました 2) のちに 本腫瘍の病態がガストリン産生腫瘍であることが明らかとなり 3 ガストリノーマとは はじめに Zollinger と Ellison は 1955 年に難治性消化性潰瘍 胃酸分泌亢進を伴う膵島細胞腫瘍をはじめて報告 1) その後 Zollinger-Ellison 症候群と命名されました 2) のちに 本腫瘍の病態がガストリン産生腫瘍であることが明らかとなり 3) ガストリノーマと呼ばれるようになりました なお Zollinger-Ellison 症候群による潰瘍は

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