Neurological Surgery 脳神経外科42巻7号

2014年07月発行

症例

小脳虫部髄芽腫に対する放射線療法25年後に発生した前頭葉CNS primitive neuroectodermal tumorの1例

著者： 佐藤裕之¹ 澁谷航平¹ 小泉孝幸¹ 加藤俊一¹ 遠藤深¹

所属機関： ¹竹田綜合病院脳神経外科

ページ範囲：P.641 - P.650

Ⅰ．はじめに

　中枢神経系primitive neuroectodermal tumor（CNS PNET）は未分化な神経上皮性腫瘍からなり，神経細胞，グリア，上皮細胞などへの多様な潜在的分化能をもつ腫瘍である．WHO gradeⅣであり，組織学的に類似した髄芽腫とともに胎児性脳腫瘍である．発生母地としては，Rorke²⁰⁾は胎生期に盛んに分裂増殖している脳室周囲細胞であるgerminal matrix cellが起源であると推定し報告している．1973年にHartとEarle¹¹⁾によって，①幼少期に好発，②臨床的に悪性，③大脳に発生，④肉眼的に囊胞状，出血性で境界明瞭，⑤顕微鏡的に悪性，全体的に未分化で一部グリア細胞や神経細胞への分化傾向を認め，⑥著明な間質成分という特徴を有し，90～95%以上が未分化の細胞からなる腫瘍として提唱された．

　一方，髄芽腫治療後長期生存例が増えるにつれて，二次新生物，とりわけ二次性脳腫瘍の報告は増えてきている．

　今回われわれは，髄芽腫に対する放射線治療25年後に発症した放射線誘発性が疑われる二次性脳腫瘍としてのCNS PNET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

1) Behdad A, Perry A：Central nervous system primitive neuroectodermal tumors：a clinicopathologic and genetic study of 33 cases. Brain Pathol 20：441-450, 2010

2) British Neuro-Oncology Society. “Guidelines on the diagnosis and management of Adult PNETs”. Rare brain and CNS Tumors Guidelines. June 2011, pp16-17　http://www.bnos.org.uk/documents/rare_tumours_guidelines/Adult%20PNET%20guidelines.pdf（accessed 2014-06-16）

3) Brüstle O, Ohgaki H, Schmitt HP, Walter GF, Ostertag H, Kleihues P：Primitive neuroectodermal tumors after prophylactic central nervous system irradiation in children. Association with an activated K-ras gene. Cancer 69：2385-2392, 1992

4) Butturini AM, Jacob M, Aguajo J, Vander-Walde NA, Villablanca J, Jubran R, Erdreich-Epstein A, Marachelian A, Dhall G, Finlay JL：High-dose chemotherapy and autologous hematopoietic progenitor cell rescue in children with recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors：the impact of prior radiotherapy on outcome. Cancer 115：2956-2963, 2009

5) Cahan WG, Woodard HQ：Sarcoma arising in irradiated bone；report of 11 cases. Cancer 1：3-29, 1948

6) Chan M, Herrera SR, Neckrysh S, Wallace A, Valyi-Nagy T, Charbel FT：Primitive neuroectodermal tumor after radiation therapy for craniopharyngioma. Neurosurg Focus 30:E3, 2011

7) Chen AY, Lee H, Hartman J, Greco C, Ryu JK, O' Donnell R, Boggan J：Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma. AJNR Am J Neuroradiol 26：160-162, 2005

8) Fangusaro J, Finlay J, Sposto R, Ji L, Saly M, Zacharoulis S, Asgharzadeh S, Abromowitch M, Olshefski R, Halpern S, Dubowy R, Comito M, Diez B, Kellie S, Hukin J, Rosenblum M, Dunkel I, Miller DC, Allen J, Gardner S：Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue（AuHCR）in young children with newly diagnosed supratentorial primitive neuroectodermal tumors（sPNETs）:report of the Head Start I and II experience. Pediatr Blood Cancer 50：312-318, 2008

9) Geyer JR, Sposto R, Jennings M, Boyett JM, Axtell RA, Breiger D, Broxson E, Donahue B, Finlay JL, Goldwein JW, Heier LA, Johnson D, Mazewski C, Miller DC, Packer R, Puccetti D, Radcliffe J, Tao ML, Shiminski-Maher T；Children's Cancer Group：Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors：a report from the Children's Cancer Group. J Clin Oncol 23：7621-7631, 2005

10) Hader WJ, Drovini-Zis K, Maguire JA：Primitive neuroectodermal tumors in the central nervous system following cranial irradiation：a report of four cases. Cancer 97：1072-1076, 2003

11) Hart MH, Earle KM：Primitive neuroectodermal tumor of the brain in children. Cancer 32：890-896, 1973

12) Kim DG, Lee DY, Paek SH, Chi JG, Choe G, Jung HW：Supratentorial primitive neuroectodermal tumors in adults. J Neurooncol 60：43-52, 2002

13) MacKenzie JM, Franks AJ, van Hille PT, Cameron MM：The evolution of an oligodendroglioma into a primitive neuroectodermal tumour. Neuropathol Appl Neurobiol 14：71-79, 1988

14) McBride SM, Daganzo SM, Banerjee A, Gupta N, Lamborn KR, Prados MD, Berger MS, Wara WM, Haas-Kogan DA：Radiation is an important component of multimodality therapy for pediatric non-pineal supratentorial primitive neuroectodermal tumors. Int J Radiat Oncol Biol Phys 72：1319-1323, 2008

15) National Comprehensive Cancer Network（NCCN). “Central Nervous System Cancers（Version1. 2014）”. NCCN Clinical Practice Guidelines in Oncology. 2014, AMED1-3　http://www.nccn.org/professionals/physician_gls/pdf/cns.pdf（accessed 2014-06-16）

16) Nicholson HS, Kretschmar CS, Krailo M, Bernstein M, Kadota R, Fort D, Friedman H, Harris MB, Tedeschi-Blok N, Mazewski C, Sato J, Reaman GH：Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors：a report from the Children's Oncology Group. Cancer 110：1542-1550, 2007

17) Packer RJ, Zhou T, Holmes E, Vezina G, Gajjar A：Survival and secondary tumors in children with medulloblastoma receiving radiotherapy and adjuvant chemotherapy：results of Children's Oncology Group trial A9961. Neuro Oncol 15：97-103, 2013

or radiation induced? Report of three cases and review of literature. Br J Neurosurg 22：402-408, 2008

19) Papadopoulos EK, Fountas KN, Brotis AG, Paterakis KN：A supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in a 42-year-old man：a case report and review of the literature. J Med Case Rep 7：86, 2013

20) Rorke LB：The cerebellar medullo-blastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neural 42：1-15, 1983

21) Rutkowski S, Bode U, Deinlein F, Ottensmeier H, Warmuth-Metz M, Soerensen N, Graf N, Emser A, Pietsch T, Wolff JE, Kortmann RD, Kuehl J：Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352：978-986, 2005

22) Sobowale OA, McCabe M, Pal P, Soh C, Karabatsou K：Radiotherapy-induced supratentorial primitive neuroectodermal tumour in a 17-year-old female：a case report and review of the literature. Acta Neurochir（Wien）153：413-417, 2011