Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A Case of Nasal T/NK-cell Lymphoma

비강 T/NK 세포형 림프종 1례

  • Lee, Jung Bok (Department of Pediatrics, Gachon Medical School, Gil Medical Center) ;
  • Jeon, In-sang (Department of Pediatrics, Gachon Medical School, Gil Medical Center) ;
  • Im, Ho Joon (Department of Pediatrics, Gachon Medical School, Gil Medical Center) ;
  • Oh, Young Ha (Department of Pathology, Gachon Medical School, Gil Medical Center) ;
  • Kim, Ji Hye (Department of Diagnostic Radiology, Gachon Medical School, Gil Medical Center)
  • 이정복 (가천의과대학교 길병원 소아과학교실) ;
  • 전인상 (가천의과대학교 길병원 소아과학교실) ;
  • 임호준 (가천의과대학교 길병원 소아과학교실) ;
  • 오영하 (가천의과대학교 길병원 해부병리학교실) ;
  • 김지혜 (가천의과대학교 길병원 진단방사선과학교실)
  • Received : 2003.07.19
  • Accepted : 2003.10.06
  • Published : 2003.12.15
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Abstract

The advance of the immunobiology clarifies the nature of non-Hodgkin's lymphoma(NHL). In addition the proceed in the immunophenotyping renders the classification of NHL. According to the Revised European American Lymphoma(REAL) classification, classified by the etiologic factors, molecular biological characteristics, immunophenotype, cytogenetics and histologic feature, nasal T/NK-cell lymphoma(=angiocentric lymphoma) belongs to the category of peripheral T-cell and natural killer cell lymphoma. Nasal T/NK-cell lymphoma is a distinct clinicopathologic entity characterized by progressive necrotic lesions in the nasal cavity, nasopharynx, and palate. The cellular origin of this tumor has been controversial. Although most nasal T/NK-cell lymphomas are of NK-cell lineage, being CD56+, negative for surface CD3(Leu4), and unassociated with rearrangements of the T-cell receptor genes, other minor variants have been reported. This lymphoma is a rare disease and usually experienced in adult. Recently, we experienced a rare type lymphoma, nasal T/NK-cell lymphoma, in 14 years old boy. His soft mass occupied the right nasal cavity including the nasal septum and turbinate. Pathologically this nasal mass showed the infiltration into the vascular wall, illustrating angiodestructive lesion. The cellular origin was NK-cell lineage, being CD56+ and negative to CD3. Now, we report the case with a brief review of related literatures.

저자들은 비강내 종괴로 내원한 14세 환아에서 종괴 생검 및 면역 표현형 검사로 비강 T/NK 세포형 림프종으로 진단하고 치료 중인 1례를 보고하는 바이다.

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