급성 골수성 백혈병의 최초 증상으로 안구 돌출을 초래한 양측 안와-부비동 과립세포육종: 증례 보고

Sino-orbital Granulocytic Sarcoma Causing Bilateral Proptosis As an Initial Manifestation of Acute Myelogenous Leukemia (AML): A Case Report

  • 김희선 (고려대학교 의과대학 안산병원 영상의학과) ;
  • 제보경 (고려대학교 의과대학 안산병원 영상의학과) ;
  • 이영흔 (고려대학교 의과대학 안산병원 영상의학과) ;
  • 김백현 (고려대학교 의과대학 안산병원 영상의학과)
  • Kim, Hee-Sun (Department of Radiology, Ansan Hospital, College of Medicine, Korea University) ;
  • Je, Bo-Kyung (Department of Radiology, Ansan Hospital, College of Medicine, Korea University) ;
  • Lee, Young-Hen (Department of Radiology, Ansan Hospital, College of Medicine, Korea University) ;
  • Kim, Baek-Hyun (Department of Radiology, Ansan Hospital, College of Medicine, Korea University)
  • 투고 : 2010.02.21
  • 심사 : 2010.04.27
  • 발행 : 2010.06.30

초록

과립세포육종은 골수성 백혈병의 발현 형태의 하나로써, 과립세포들의 원시 전구체가 골수가 아닌 연조직에 침착하여 생긴 고형 종괴를 지칭한다. 저자들은 다른 전신 증상 없이 양쪽 안구의 돌출을 주소로 내원하여 광범위한 양쪽 안와-부비동 과립세포육종으로 진단된 22개월 남아의 CT 및 MR 소견을 보고한다. 종괴는 양측 안와 외하벽을 침범하여 양쪽 안구를 돌출시켰으며, 부비동, 상악골, 측두골, 광대뼈, 나비뼈, 벌집뼈, 입천장뼈를 포함한 거의 모든 머리뼈바닥의 골수에 침윤하고 주변에 연부 조직 종괴를 형성하였고, 해면정맥동의 아래 1/2을 침범하고 측두골 안쪽의 경질막이 비후된 소견을 동반하였다. 환아는 골수를 이용한 염색체 검사를 통해 8번과 21번 염색체의 상호전좌 t(8;21) (q22;q22)를 동반한 급성 골수성 백혈병으로 진단되었다.

Granulocytic sarcoma is a manifestation of myelogenous leukemia, which means a solid mass consisting of primitive precursors of the granulocytic series of white blood cells. We present CT and MR imaging findings of bilateral sino-orbital granulocytic sarcoma in a 22-month-old boy. The mass involved bilateral orbital fossa which resulted in bilateral proptosis. Moreover, the mass extended to the almost skull base including paranasal sinuses, maxilla, temporal bone, zygomatic bone, sphenoid bone, ethmoid, and palatine bone. The adjacent dura was continuously thickened and the lower half of cavernous sinus was also involved. The patient was diagnosed as AML (M5) with t(8,21) translocation through a chromosome study from the bone marrow.

키워드

참고문헌

  1. Pui MH, Fletcher BD, Langston JW. Granulocytic sarcoma in childhood leukemia: imaging features. Radiology 1994;190(3): 698-702 https://doi.org/10.1148/radiology.190.3.8115614
  2. Pui CH. Childhood leukaemias. Cambridge: cambridge university press. 1999:288-312 443-481
  3. Binder C, Tiemann M, Haase D, Humpe A, Kneba M. Isolated meningeal chloroma (granulocytic sarcoma) - a case report and review of the literature. Ann Hematol 2000;79(8):459-462 https://doi.org/10.1007/s002770000165
  4. Bulas RB, Laine FJ, Das Narla L. Bilateral orbital granulocytic sarcoma (chloroma) preceding the blast phase of acute myelogenous leukemia: CT findings. Pediatr Radiol 1995;25(6):488- 489 https://doi.org/10.1007/BF02019079
  5. Guermazi A, Feger C, Rousselot P, et al. Granulocytic sarcoma (chloroma): imaging findings in adults and children. AJR Am J Roentgenol 2002;178(2):319-325 https://doi.org/10.2214/ajr.178.2.1780319
  6. Chung EM, Murphey MD, Specht CS, Cube R, Smirniotopoulos JG. From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit. Radiographics 2008;28(4):1193-1214 https://doi.org/10.1148/rg.284085013
  7. Jakobiec FA. Granulocytic sarcoma. AJNR Am J Neuroradiol 1991;12(2):263-264
  8. Stein-Wexler R, Wootton-Gorges SL, West DC. Orbital granulocytic sarcoma: an unusual presentation of acute myelocytic leukemia. Pediatr Radiol 2003;33(2):136-139 https://doi.org/10.1007/s00247-002-0834-0
  9. Uyesugi WY, Watabe J, Petermann G. Orbital and facial granulocytic sarcoma (chloroma): a case report. Pediatr Radiol 2000;30(4):276-278 https://doi.org/10.1007/s002470050740