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A Case of Nasal T/NK-cell Lymphoma

Journal of the Korean Pediatric Society 2003;46(12):1266-1270.
Published online December 15, 2003.
A Case of Nasal T/NK-cell Lymphoma
Jung Bok Lee1, In-sang Jeon1, Ho Joon Im1, Young Ha Oh2, Ji Hye Kim3
1Department of Pediatrics, Gachon Medical School, Gil Medical Center, Inchon, Korea
2Department of Pathology, Gachon Medical School, Gil Medical Center, Inchon, Korea
3Department of Diagnostic Radiology, Gachon Medical School, Gil Medical Center, Inchon, Korea
비강 T/NK 세포형 림프종 1례
이정복1, 전인상1, 임호준1, 오영하2, 김지혜3
1가천의과대학교 길병원 소아과학교실
2가천의과대학교 길병원 해부병리학교실
3가천의과대학교 길병원 진단방사선과학교실
Correspondence: 
In-sang Jeon, Email: isjeon@ghil.com
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.
Key Words: Nasal T/NK-cell lymphoma, Non-Hodgkin Lymphoma, Nasal cavity


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