J Korean Pediatr Soc.  2003 Dec;46(12):1266-1270.

A Case of Nasal T/NK-cell Lymphoma

• Lee JB
• Jeon IS
• Im HJ
• Oh YH
• Kim JH

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.

Keyword

Nasal T/NK-cell lymphoma; Non-Hodgkin Lymphoma; Nasal cavity

MeSH Terms

Adolescent
Adult
Classification
Cytogenetics
Genes, T-Cell Receptor
Humans
Immunophenotyping
Killer Cells, Natural
Lymphoma*
Lymphoma, Non-Hodgkin
Male
Nasal Cavity
Nasal Septum
Nasopharynx
Palate
Population Characteristics
Rare Diseases
T-Lymphocytes
Turbinates