日本消化器外科学会教育集会

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1 IPMT MCT Epithelial tumors Exocrine tumors Serous cystic tumors Mucinous cystic tumors Intraductal tumors Atypical hyperplasica(ah)and carcinoma in situ(cis) Invasive ductal carcinoma Acinar cell tumors Endocrine tumors Combined tumors Epithelial tumors of uncertain differentiation Unclassifiable Miscellaneous 2 Non-epithelial tumors Hemangioma Lymphangioma Leiomyosarcoma Malignant fibrous histiocytoma Malignant lymphoma Paraganglioma Others Courvoisier CEA CA19-9 DUPAN2, Span

2 52 ( ) 80 CT CT a, b Thin sectional helical CT phase helical CT Time-attenuation curve (TAC) CT MRCP ERCP ERCP ERP a b FDG-PET Angiography encasement pharmacoportography EUS-FNA (Combination of pathological diagnosis and analysis for mutant K-ras gene) a 47 CT low density mass b 72

3 a b ras 81 -ras 88 / PTCD metallic stent stent QOL gemcitabine Zollinger Ellison 13 Zollinger-Ellison syndrome 14 VIP (vasoactive intestinal peptide) WDHA MEN (multiple endocrine neoplasmia) MEN MEN 20 hypervascular 73

4 21 VIPoma 21 MEN Whipple Fajans IRI( U/ml)/BS( /dl) 0.3 C-peptide ( ) IRI ( ) CT50 71 PTPVS EUS CT CT CT MRI T T Dynamic MRI PTPVS(percutaneous transhepatic portal sampling) Ingemansson 23 cm a, b Ca ASVS 24 IRI 74

5 PTPVS (a) (b) a ASVS PTPVS IRI IRI150 U/ml IRI octreotide g 26 b OUS MEN 75

6 a 21 CT isodensity mass b 27 Zollinger-Ellison mm MEN MEN 29 ph 200pg/mL 1000pg/mL BAO 15mEq/ /BAO/MAO G 31 / FDG-PET 30 Imamura SASI test SASI test RIA 30 76

7 cm pg/mL 34 gluconeogenesis / cm VIP (Watery Diarrhea) Hypokalemia Achlorhydria VIP 200pg/ml cm Vipoma 21 CT isodensity mass a b MRI TT a, b Gd ( c) 28 3l VIPWDHAVerner- Morrison VIP (vasoactive intestinal polypeptide) VIP a MRI T1 77

8 b T2 c Gd VIPoma 100 MEN PP Cushing ACTH ; 57(11): MRCP 2001; 84(6): ; 20(11): Helical CT1998; 36(1):79-89 FDG-PET 2001; 24(9): Tada M, Komatsu Y, Kawabe T et al: Quantitative analysis of K-ras gene mutation in pancreastic tissue obtained by endoscopic ultrasonography-guided fine needle aspiration : clinical utility for diagnosis of pancreastic tumor. Am J Gastroenterol 2002; 97: Oettle H, Riess H : Gemcitabine in combination with 5-Fluorouracil with or 78

9 without folinic acid in the treatment of pancreastic cancer. Cancer 2002; 95: McGinn CJ, Lawrence TS, Zalupski MM: On the development of gemcitabine-based chemoradiotherapy regimens in pancreatic cancer. Cancer 2002; 95: Nicholls AG: Simple adenoma of pancreas arising from island of Langerhans. J Med Res 1902; 8: Wilder RM, Alan FN, Power MH, et al. Carcinoma of the island of the pancreas; hyperinsulinism and hypoglycemia. JAMA 1927; 89: Zolliger RM, Ellison EH:Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 1955; 132: GregoryRA, Tracy HJ, Freench JM et al. Extraction of a gastrin-like substance from a pancreatic tumor in a case of Zolliger- Ellison syndrome. Lancet 1960; 1: Bloom SR, Polak JM, Pearse AGE : Vasoactive intestinal peptide and watery- diarrhea syndrome. Lancet 1973; 2 (7819) : Verner JV, Morrison AB : Endocrine pancreatic islet disease with diarrhea. Report of a case due to diffuse hyperplasia of non beta islet tissue with a review of 54 additional cases. Arch Intern Med 1974; 133: Prinz RA, Dorsch TR, Lawrence AM : Clinical aspect of glucagons-producing islet cell tumors. Am J Gastroenterol 1981; 76 : Larsson LI, Hirsch MA, Holst JJ et al.: Pancreatic somatostatinoma, clinical features and physiological implications. Lancet 1977; 1: MEN1 1999; 38(6): Chandrasekharappa SC, Guru SC, Manickam P et al.: Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science 1997; 276 (5311) : ; 51: ; 17 (1): Ingemansson S, Lunderquist A, Lunderquist I et al.: Portal and pancreastic vein cateterization with radioimmunologic determination of insulin. Surg Gynecol Obstet 1975; 141: Doppman JL, Miller DL, Chang R, et al. Insulinomas: Localization with selective intraarterial injection of calcium. Radiology 1991; 178: Tsagarakis S, Jasjarekus J, Malagari C, et al.: Regionalization of occult pancreastic insulinomas with the arterial stimulation venous sampling (ASVS) technique. Clin Endocrinol 1997; 47: Debas HT, Gittes G : Somatostatin analogue therapy in functioning neuroendocrine gut tumors. Digestion 1993; 54: ; 38 (6) : Ikeda S, Matsumoto S, Maeshiro K, et al.: Segmental pancreatectomy for the diagnosis and treatment of small lesions in the neck or body of the pancreas. Hepato- Gastroenterol 1995; 42:

10 1996; 17(1): ; 36(6): Imamura M, Adachi H, Takahashi K, et al.: Gastrin release from gastrinoma cells stimulated with secretin. Dig Dis Sci 1982; 27: Imamura M, Minematsu S, Suzuki T, et al.: Usefulness of selective arterial sectetin injection test for localization of gastrinoma in the Zollinger-Ellison syndrome. Ann Surg 1987; 205: Soga J, Yakuwa Y: Glucagonomas/diabetico- dermatogenic syndrome (DDS): A statistical evaluation of 407 reported cases. J Hepatobiliary Pancreat Surg 1998; 5: ; 38(6): ; 9: Delcore R, Friesen SR:Gastrointestinal neuroendocrine tumors. J Am Coll Surg 1994; 178: Ajani JA, Carrasco H, Charnsangavej C et al : Islet cell tumors metastatic to the liver: Effective palliation by sequential hepatic artery embolization. Ann Int Med 1988; 108: WDHA 1997; 12: 'Dorisio MS : Neuropeptides and gastrointestinal immunity. Am J Med 1986; 81: ; 38(6):

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

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 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 1986 2005 Work Japan 10 3.73.1 10 2.21.0 midgut 1/31/10 2 WHO 2000 cancer like lesions

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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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