Abstract:

Extranodal NK/T-cell lymphoma of nasal type is a rare disease in the western world and is linked to the Epstein-Barr virus infection. The majority of the cases are located in the upper aerodigestive tract. Cutaneous extranodal NK/T-cell lymphoma commonly presents as multiple erythematous papules or plaques, sometimes as ulcerated lesions. The lymphoma cells are often heterogeneous in size and in the degree of cytologic atypia. The typical immunophenotype of the lymphomas is CD2+, CD3+, and CD56+. All the cases express EBV encoded small RNA. Extranodal NK/T-cell lymphoma of nasal type is frequently angiodestructive and with extensive tissue necrosis. Here we report a case of extranodal NK/T-cell lymphoma with both cutaneous and mucosal involvement and with rather bland cytology. A 47-year-old male presented with a palate ulcer and cutaneous plaques for 4 months. The skin lesions are erythematous papules with central necrosis on bilateral temples and cheeks. The skin lesion showed diffuse dermal lymphocyte infiltrates and is composed of small and medium-sized lymphocytes with only slightly irregular nuclei and is accompanied by heavy inflammation. The atypical lymphocytes express CD3, CD7, CD30, and CD56 with a high proliferation index. The tumor cells express Epstein-Barr virus-encoded small RNA and are negative for CD4, CD5, and CD8 expression. The palate lesion showed similar morphology and immunophenotype except with much prominent cytologic atypia. When a suspicious lymphoproliferative lesion involving the aerodigestive tract and skin, even with minimal cytologic atypia, extranodal NK/T-cell lymphoma should be included in the differential diagnosis.

Financial Disclosure:
No current or relevant financial relationships exist.

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Bing Han, MD