Neurological Surgery 脳神経外科48巻9号

2020年09月発行

症例

髄膜腫と鑑別困難であったErdheim-Chester diseaseの1例

著者： 吉山智美¹ 棟方理² 前島亜希子³ 梅嵜有砂¹ 山家弘雄¹ 西山徹¹ 中條敬人¹ 田中優子¹ 松本浩明¹ 寺田友昭¹

所属機関： ¹昭和大学藤が丘病院脳神経外科 ²国立がん研究センター中央病院希少がんセンター血液腫瘍科 ³国立がん研究センター中央病院病理診断科

ページ範囲：P.809 - P.818

Ⅰ．はじめに
　Erdheim-Chester disease（ECD）は，非ランゲルハンス細胞性組織球症の中でも稀な疾患である．1972年に提唱され，2004年まで世界で100例にも満たない報告数であった．しかし近年，報告数の増加とともに，2013年の米国サンディエゴでのシンポジウム以来，世界的に研究が進められBRAF^V600E遺伝子変異との関係も明らかになっている．
　今回，大脳鎌およびテント部，鞍上部に多発性に発生したECDの手術症例を経験したので，文献的考察を加えて報告する．

参考文献

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2) Alimohamadi M, Hartmann C, Paterno V, Samii M：Erdheim-Chester disease mimicking an intracranial trigeminal schwannoma：case report. J Neurosurg Pediatr 15：493-498, 2015

3) Arnaud L, Hervier B, Néel A, Hamidou MA, Kahn JE, Wechsler B, Pérez-Pastor G, Blomberg B, Fuzibet JG, Dubourguet F, Marinho A, Magnette C, Noel V, Pavic M, Casper J, Beucher AB, Costedoat-Chalumeau N, Aaron L, Salvatierra J, Graux C, Cacoub P, Delcey V, Dechant C, Bindi P, Herbaut C, Graziani G, Amoura Z, Haroche J：CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease：a multicenter survival analysis of 53 patients. Blood 117：2778-2782, 2011

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6) Diamond EL, Dagna L, Hyman DM, Cavalli G, Janku F, Estrada-Veras J, Ferrarini M, Abdel-Wahab O, Heaney ML, Scheel PJ, Feeley NK, Ferrero E, McClain KL, Vaglio A, Colby T, Arnaud L, Haroche J：Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood 124：483-492, 2014

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-mutated Erdheim-Chester disease. J Clin Oncol 33：411-418, 2015

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mutation. Blood 124：1119-1126, 2014

12) Huang LC, Topping KL, Gratzinger D, Brown RA, Martin BA, Silva RA, Kossler AL：Orbital and chorioretinal manifestations of Erdheim-Chester disease treated with vemurafenib. Am J Ophthalmol Case Rep 11：158-163, 2018

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18) Ohara Y, Kato S, Yamashita D, Satou A, Shimoyama Y, Hamaie C, Sato M, Ban N, Yamamoto K, Yamada T, Kawai H, Ohshima K, Nakamura S, Toyokuni S：An autopsy case report：differences in radiological images correlate with histology in Erdheim-Chester disease. Pathol Int 68：374-381, 2018

19) Rushing EJ, Bouffard JP, Neal CJ, Koeller K, Martin J, Ozdemirli M, Mena H, Ecklund JM：Erdheim-Chester disease mimicking a primary brain tumor. Case report. J Neurosurg 100：1115-1118, 2004

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21) Wagner KM, Mandel JJ, Goodman JC, Gopinath S, Patel AJ：Intracranial Erdheim-Chester disease mimicking parafalcine meningioma：report of two cases and review of the literature. World Neurosurg 110：365-370, 2018