All issues > Volume 46(12); 2003

Case Report

J Korean Pediatr Soc. 2003;46(12):1266-1270. Published online December 15, 2003.

A Case of Nasal T/NK-cell Lymphoma

Jung Bok JB Lee¹, In-sang IS Jeon¹, Ho Joon HJ Im¹, Young Ha YH Oh², Ji Hye JH 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

Correspondence In-sang IS 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.

Keywords :Nasal T/NK-cell lymphoma, Non-Hodgkin Lymphoma, Nasal cavity