von Recklinghausen病に十二指腸乳頭カルチノイドおよび多発性十二指腸GISTを合併した1例
症例は59歳，女性．29歳時にvon Recklinghausen病（VRD）と診断されている．血液検査で肝胆道系酵素の上昇を指摘され当院を受診した．上部消化管内視鏡検査にて十二指腸乳頭に不整形の隆起性病変を認め，生検の結果カルチノイドと診断され，幽門輪温存膵頭十二指腸切除術を施行した．十二指腸乳頭に30×25mm大の腫瘤を認めた以外に十二指腸壁に5個の壁在結節を認めた．病理組織検査にて十二指腸乳頭の腫瘤はソマトスタチン産生型のカルチノイド，壁在結節はいずれもGISTと診断された．#17aのリンパ節2個にカルチノイドの転移を認めたが，術後4年の時点で転移再発は認めていない．VRDにおいては神経...
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| Published in | 日本臨床外科学会雑誌 Vol. 69; no. 7; pp. 1668 - 1672 |
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| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本臨床外科学会
2008
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| Subjects | |
| Online Access | Get full text |
| ISSN | 1345-2843 1882-5133 |
| DOI | 10.3919/jjsa.69.1668 |
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| Abstract | 症例は59歳，女性．29歳時にvon Recklinghausen病（VRD）と診断されている．血液検査で肝胆道系酵素の上昇を指摘され当院を受診した．上部消化管内視鏡検査にて十二指腸乳頭に不整形の隆起性病変を認め，生検の結果カルチノイドと診断され，幽門輪温存膵頭十二指腸切除術を施行した．十二指腸乳頭に30×25mm大の腫瘤を認めた以外に十二指腸壁に5個の壁在結節を認めた．病理組織検査にて十二指腸乳頭の腫瘤はソマトスタチン産生型のカルチノイド，壁在結節はいずれもGISTと診断された．#17aのリンパ節2個にカルチノイドの転移を認めたが，術後4年の時点で転移再発は認めていない．VRDにおいては神経線維腫や神経原性腫瘍以外にさまざまな非神経原性腫瘍を合併しうることが報告されており，VRD患者の診療に当たっては消化管病変の合併の可能性に関して常に留意しておく必要があると考えられた． |
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| AbstractList | 症例は59歳，女性．29歳時にvon Recklinghausen病（VRD）と診断されている．血液検査で肝胆道系酵素の上昇を指摘され当院を受診した．上部消化管内視鏡検査にて十二指腸乳頭に不整形の隆起性病変を認め，生検の結果カルチノイドと診断され，幽門輪温存膵頭十二指腸切除術を施行した．十二指腸乳頭に30×25mm大の腫瘤を認めた以外に十二指腸壁に5個の壁在結節を認めた．病理組織検査にて十二指腸乳頭の腫瘤はソマトスタチン産生型のカルチノイド，壁在結節はいずれもGISTと診断された．#17aのリンパ節2個にカルチノイドの転移を認めたが，術後4年の時点で転移再発は認めていない．VRDにおいては神経線維腫や神経原性腫瘍以外にさまざまな非神経原性腫瘍を合併しうることが報告されており，VRD患者の診療に当たっては消化管病変の合併の可能性に関して常に留意しておく必要があると考えられた． |
| Author | 吉田, 泰夫 伊藤, 雅 池田, 博斉 小笠原, 敬三 河本, 和幸 朴, 泰範 |
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| References | 1) Riccardi VM : Von Recklinghausen neurofibromatosis. New Engl J of Med 305 : 1617-1626, 1981 8) Ricci JL : Carcinoid of the ampulla of Vater. Cancer 71 : 686-690, 1994 7) 岡村圭也，長川達哉，安保智典他:十二指腸乳頭部カルチノイド腫瘍および胆管原発カルチノイド腫瘍の診断と治療．胆と膵 28:173-180，2007 4) 鈴木 力，曽我 淳，岡本春彦他:消化器カルチノイド―消化管カルチノイドの外科治療―．G.I.Res 7:116-121，1999 11) Miettinen M, Fetsch JF, Sobin LH, et al : Gastrointestinal stromal tumors in patients with neurofibromatosis 1 : clinicapathologic and molecular genetic study of 45 cases. Am J Sug Pathol 30 : 90-96, 2006 6) Yoshida A, Hatanaka S, Ohi Y, et al : Von Recklinghausen's disease associated with somatostatin-rich duodenal carcinoid (somatostatinoma), medullary tyroid carcinoma and diffuse adrenal medullary hyperplasia. Acta Pathol Jpn 41 : 847-856, 1991 17) Suzuki S, Sato K, Katada E, et al : Periampullary somatostatinoma and multiple gastrointestinal stromal tumors associated with von Recklinghausen's disease. J Gastroenterol 39 : 1011-1012, 2004 15) Karatzas G, Kouraklis G, Karayiannakis A, et al : Ampullary carcinoid and jejunal stromal tumor associated with von Recklinghausen's disease presenting as gastrointestinal bleeding and jaundice. Eur L Surg Oncol 26 : 428-429, 2000 16) Usui M, Matsuda S, Suzuki H, et al : Somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease. J Gastroenterol 37 : 947-953, 2002 5) Hartel M, Wente MN, Sido B, et al : Carcinoid of the ampulla Vater. J of Gastroenterology and Hepatology 20 : 676-681, 2005 14) Martens O, Prenen H, Debiec-Rychter M, et al : Molecular pathogenesis of multiple gastrointestinal stromal tumors in NF1 patients. Hum Mol Genet 15 : 1015-1023, 2006 2) Xu G, O'Connell P, Viskochil D, et al : The neurofibromatosis type1 gene encodes a protein related to GAP. Cell 62 : 599-608, 1992 13) Stewart DR, Corless CL, Rubin BP, et al : Mitotic recombination as evidence of alternative pathogenesis of gastrointestinal stromal tumors in neurofibromatosis type1. J Med Genet 44 : e61, 2007 12) Andersson J, Sihto H, Mesi-Kindblom JM, et al : NF1-associated gastrointestinal stromal tumors have unique clinical, phenotypic, and genotypic characteristics. Am J Surg Pathol 29 : 1170-1176, 2005 9) Hatzithoklitos E, Buchler MW, Friess H, et al : Carcinoid of ampulla of Vater. Cancer 73 : 1580-1588, 1994 10) Zöller ME, Rembeck B, Odén A, et al : Malignant and benign tumors in patients with neurofibromatosis type 1 in a defined Swedish population. Cancer 79 : 2125-2131, 1997 3) Fuller CE, Williams GT : Gastrointestinal manifestations of type 1 neurofibromatosis (von Recklinghausen's disease). Histopathology 19 : 1-11, 1991 18) Kramer K, Siech M, Strater J, et al : GI hemorrhage with fuluminant shock induced by jejunal gastrointestinal stromal tumor (GIST) coincident with duodenal neuroendocrine carcinoma (NET) + neurofibromatosis (NF) —case report and review of literature. J Gastroenterol 43 : 281-288, 2005 |
| References_xml | – reference: 18) Kramer K, Siech M, Strater J, et al : GI hemorrhage with fuluminant shock induced by jejunal gastrointestinal stromal tumor (GIST) coincident with duodenal neuroendocrine carcinoma (NET) + neurofibromatosis (NF) —case report and review of literature. J Gastroenterol 43 : 281-288, 2005 – reference: 15) Karatzas G, Kouraklis G, Karayiannakis A, et al : Ampullary carcinoid and jejunal stromal tumor associated with von Recklinghausen's disease presenting as gastrointestinal bleeding and jaundice. Eur L Surg Oncol 26 : 428-429, 2000 – reference: 6) Yoshida A, Hatanaka S, Ohi Y, et al : Von Recklinghausen's disease associated with somatostatin-rich duodenal carcinoid (somatostatinoma), medullary tyroid carcinoma and diffuse adrenal medullary hyperplasia. Acta Pathol Jpn 41 : 847-856, 1991 – reference: 14) Martens O, Prenen H, Debiec-Rychter M, et al : Molecular pathogenesis of multiple gastrointestinal stromal tumors in NF1 patients. Hum Mol Genet 15 : 1015-1023, 2006 – reference: 13) Stewart DR, Corless CL, Rubin BP, et al : Mitotic recombination as evidence of alternative pathogenesis of gastrointestinal stromal tumors in neurofibromatosis type1. J Med Genet 44 : e61, 2007 – reference: 5) Hartel M, Wente MN, Sido B, et al : Carcinoid of the ampulla Vater. J of Gastroenterology and Hepatology 20 : 676-681, 2005 – reference: 8) Ricci JL : Carcinoid of the ampulla of Vater. Cancer 71 : 686-690, 1994 – reference: 4) 鈴木 力，曽我 淳，岡本春彦他:消化器カルチノイド―消化管カルチノイドの外科治療―．G.I.Res 7:116-121，1999 – reference: 11) Miettinen M, Fetsch JF, Sobin LH, et al : Gastrointestinal stromal tumors in patients with neurofibromatosis 1 : clinicapathologic and molecular genetic study of 45 cases. Am J Sug Pathol 30 : 90-96, 2006 – reference: 12) Andersson J, Sihto H, Mesi-Kindblom JM, et al : NF1-associated gastrointestinal stromal tumors have unique clinical, phenotypic, and genotypic characteristics. Am J Surg Pathol 29 : 1170-1176, 2005 – reference: 10) Zöller ME, Rembeck B, Odén A, et al : Malignant and benign tumors in patients with neurofibromatosis type 1 in a defined Swedish population. Cancer 79 : 2125-2131, 1997 – reference: 17) Suzuki S, Sato K, Katada E, et al : Periampullary somatostatinoma and multiple gastrointestinal stromal tumors associated with von Recklinghausen's disease. J Gastroenterol 39 : 1011-1012, 2004 – reference: 7) 岡村圭也，長川達哉，安保智典他:十二指腸乳頭部カルチノイド腫瘍および胆管原発カルチノイド腫瘍の診断と治療．胆と膵 28:173-180，2007 – reference: 9) Hatzithoklitos E, Buchler MW, Friess H, et al : Carcinoid of ampulla of Vater. Cancer 73 : 1580-1588, 1994 – reference: 3) Fuller CE, Williams GT : Gastrointestinal manifestations of type 1 neurofibromatosis (von Recklinghausen's disease). Histopathology 19 : 1-11, 1991 – reference: 16) Usui M, Matsuda S, Suzuki H, et al : Somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease. J Gastroenterol 37 : 947-953, 2002 – reference: 1) Riccardi VM : Von Recklinghausen neurofibromatosis. New Engl J of Med 305 : 1617-1626, 1981 – reference: 2) Xu G, O'Connell P, Viskochil D, et al : The neurofibromatosis type1 gene encodes a protein related to GAP. Cell 62 : 599-608, 1992 |
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| Snippet | 症例は59歳，女性．29歳時にvon Recklinghausen病（VRD）と診断されている．血液検査で肝胆道系酵素の上昇を指摘され当院を受診した．上部消化管内視鏡検査にて十二指腸乳... |
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| Title | von Recklinghausen病に十二指腸乳頭カルチノイドおよび多発性十二指腸GISTを合併した1例 |
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