Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A Case of Atypical Teratoid/Rhabdoid Tumor Arising from the Supratentorial Area

천막상부에 발생한 비정형 기형/횡문근양 종양 1례

  • Jung, Kyeong Hun (Department of Pediatrics, College of Medicine, Inha University) ;
  • Kwon, Young Se (Department of Pediatrics, College of Medicine, Inha University) ;
  • Jun, Yong Hun (Department of Pediatrics, College of Medicine, Inha University) ;
  • Kim, Soon Ki (Department of Pediatrics, College of Medicine, Inha University) ;
  • Hong, Young Jin (Department of Pediatrics, College of Medicine, Inha University) ;
  • Son, Byong Kwan (Department of Pediatrics, College of Medicine, Inha University) ;
  • Kim, Eun Young (Department of Neurosurgery, College of Medicine, Inha University) ;
  • Park, In Suh (Department of Pathology,College of Medicine, Inha University)
  • 정경훈 (인하대학교 의과대학 소아과학교실) ;
  • 권영세 (인하대학교 의과대학 소아과학교실) ;
  • 전용훈 (인하대학교 의과대학 소아과학교실) ;
  • 김순기 (인하대학교 의과대학 소아과학교실) ;
  • 홍영진 (인하대학교 의과대학 소아과학교실) ;
  • 손병관 (인하대학교 의과대학 소아과학교실) ;
  • 김은영 (인하대학교 의과대학 신경외과학교실) ;
  • 박인서 (인하대학교 의과대학 해부병리학교실)
  • Received : 2004.08.21
  • Accepted : 2004.10.12
  • Published : 2005.02.15
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Abstract

Atypical teratoid/rhabdoid tumor may arise at any central nervous system location, but it is most commonly located in the cerebellum(60 percent). The incidence of this tumor remains unclear but it occurs most commonly in children less than 2 years of age. This highly malignant tumor shows a rapid progression and nonspecific radiologic findings. We report a case of primary intracranial atypical teratoid/rhabdoid tumor arising from the supratentorial area in early infancy. The diagnosis was made based on distinctive light microscopy and immunohistochemical findings. Despite aggressive surgical treatment with adjuvant chemotherapy, he died six months after his second operation.

저자들은 재태기간 36주 6일로 출생한 환아가 대천문 팽대와 활동량 감소를 보여 시행했던 방사선과적 검사에서 발견된 천막 상부에 위치한 종양에 대해 일차적으로 교모세포종으로 진단하였다가 치료 중 재발된 종양에 대해 이차 수술 후 조직검사소견과 면역조직화학검사로 비정형 기형/횡문근양 종양으로 진단한 매우 드믄 악성 뇌종양 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Keywords

References

  1. Lefkowitz IB, Rorke LB, Packer RJ, Sutton LN, Siegel KR, Katnick RJ. Atypical teratoid tumor of infancy : definition of an entity. Ann Neurol 1987;22:448-9
  2. Beigel JA, Rorke LB, Packer RJ, Emanuel BS. Monosomy 22 in rhabdoid or atypical tumors of the brain. J Neurosurg 1990;73:710-4 https://doi.org/10.3171/jns.1990.73.5.0710
  3. Burger PC, Yu IT, Tihan T, Friedman HS, Strother DR, Kepner JL, et al. Atypical teratoid/rhabdoid tumor of the central nervous system : a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma : a Pediatric Oncology Group study. Am J Surg Pathol 1998; 22:1083-92 https://doi.org/10.1097/00000478-199809000-00007
  4. Beckwith JB, Palmer NF. Histopathology and prognosis of Wilm's tumor. Results from the First National Wilm's Tumor Study. Cancer 1978;41:1937-48 https://doi.org/10.1002/1097-0142(197805)41:5<1937::AID-CNCR2820410538>3.0.CO;2-U
  5. Bonin JN, Rubinstein LJ, Palmer NF, Beckwith JB. The association of embryonal tumors originating in the kidney and in the brain. A report of seven cases. Cancer 1984;54:2137-46 https://doi.org/10.1002/1097-0142(19841115)54:10<2137::AID-CNCR2820541014>3.0.CO;2-D
  6. Rorke LB, Packer R, Biegel J. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. J Neurooncol 1995;24:21-8 https://doi.org/10.1007/BF01052653
  7. Rorke LB, Packer R, Biegel J. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood : definition of an entity. J Neurosurg 1996;85:56-65 https://doi.org/10.3171/jns.1996.85.1.0056
  8. Bhattacharjee M, Hicks J, Langford L, Dauser R, Strother D, Chintagumpala M, et al. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. Ultrastruct Pathol 1997;21:369-78 https://doi.org/10.3109/01913129709021935
  9. Ho DM, Hsu CY, Wong TT, Ting LT, Chiang H. Atypical teratoid/rhabdoid tumor of the central nervous system : a comparative study with primitive neuroectodermal tumor/medulloblastoma. Acta Neuropathol 2000;99:482-8 https://doi.org/10.1007/s004010051149
  10. Biegel JA, Allen CS, Kawasaki K, Shimizu N, Budarf ML, Bell CJ. Narrowing the critical region for a rhabdoid tumor locus in 22q11. Genes Chromosomes Cancer 1996;16:94-105 https://doi.org/10.1002/(SICI)1098-2264(199606)16:2<94::AID-GCC3>3.0.CO;2-Y
  11. Packer RJ, Biegel JA, Blaney S, Finlay J, Geyer JR, Heideman R, et al. Atypical teratoid/rhabdoid tumor of the central nervous system : report on workshop. J Pediatr Hematol Oncol 2002;24:337-342 https://doi.org/10.1097/00043426-200206000-00004