Abstract
Extranodal natural killer/T-cell lymphoma is a rare disease associated with latent EBV infection. Cutaneous involvement is the most common site after the nasal-pharyngeal area and maybe primary or secondary manifestation. We report a case of a 67-year-old woman presenting with multiple ulcerative skin lesions in the legs and buttocks. Physical examination revealed disseminated eruptive papules and nodules with central ulceration and necrosis. No lymphadenopathy is identified. Physical examination as well as radiologic evaluation, including thoracic, abdominal, and pelvic CT scan and head and neck MRI, yielded no definite involvement of other organ systems. Histopathological examination of the skin lesion biopsy showed diffuse dermal and subcutaneous infiltrate of small to large-sized tumor cells with irregular or oval nuclei and dense to vesicular chromatin. Tumor cells appeared remarkably angiocentric and angiodestructive accompanied by extensive necrosis. Marked increased mitotic figures were also seen. The overlying epidermis showed pseudoepitheliomatous hyperplasia. Tumor cells were positive for CD3 and CD56 while negative for CD20, CD4, CD8, and CD30 immunohistochemical stains. Ki-67 index was approximately 80%. Tumor cells were also extensively positive for EBER ISH. Final histopathological diagnosis was primary cutaneous extranodal NK-/T-Cell lymphoma, nasal type. The patient was treated with chemotherapy; however, she expired after three cycles. This case highlights clinical and histopathologic features of a rare case of primary cutaneous extranodal NK-/T-Cell lymphoma, nasal type with poor prognostic.
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