Abstract:

Extranodal NK/T-cell lymphoma (NKTCL) is a rarely occurring non-Hodgkin lymphoma with predilection for the nasal cavity and higher incidence in East Asia and Latin America. Cutaneous involvement, rarely occurring and often aggressive in behavior, may present as nodular mass-forming lesions with or without ulceration. Histologically, lesions are characterized by an atypical dermal lymphoid infiltrate with angioinvasion and associated necrosis. Fortuitously, Epstein-Barr virus (EBV) infection, implicated in the pathogenesis of this entity, serves as a useful diagnostic marker (i.e. EBER in situ hybridization). Furthermore, CD30 expression in NKTCL raises the possibility of potential targeted therapeutic strategies (i.e. brentuximab). Here we present a 54-year-old-male who initially presented with two 2 cm ulcerations on the right lower leg which progressed despite antibiotic therapy. With suspicion for an atypical process, biopsy was decidedly performed. Histologic examination demonstrated dense lymphoid infiltrates exhibiting epidermotropism, angiocentricity and angioinvasion extending into the deep dermis. Immunohistochemical staining demonstrated expression of CD2, CD3, CD8, TIA-1, perforin and granzyme-B, consistent with a cytotoxic T-cell phenotype. Additionally, CD56 was positive, confirming the presence of a coexistent NK cell phenotype. Additional testing demonstrated significant CD30 expression and molecular analysis was positive for TCR gene rearrangement. These findings, in conjunction with EBER in situ hybridization positivity, confirmed a diagnosis of extranodal NKTCL. It is our aim to increase awareness of this rarely occurring lymphoma with cutaneous involvement. Ancillary testing with CD30 may also provide patients with options for targeted therapy.

Financial Disclosure:
No current or relevant financial relationships exist.

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Jessica Muldoon, MD