Wait and see: a case of EBV + cutaneous extranodal NK/T-type lymphoma with indolent behaviour

Virchows Arch. 2023 Oct 23. doi: 10.1007/s00428-023-03681-9. Online ahead of print.

Abstract

Extranodal NK/T-cell lymphoma (ENKTL) is a rare lymphoma subtype associated with Epstein-Barr virus (EBV) infection, portending a poor prognosis despite systemic chemotherapy. We present the unusual case of an 85-year-old man receiving ibrutinib for mantle cell lymphoma, who developed a erythematous, subcutaneous nodule on the forehead, featuring a proliferation of pleomorphic CD8 + /CD56 - /EBV + cells. Given the negative staging and comorbidities, a watchful waiting strategy was performed, experiencing a benign course with self-resolution and complete remission over a 4-year follow-up. The literature on primary cutaneous ENKTL has been discussed, with particular attention to clinical and histological prognostic factors.

Keywords: Epstein-Barr virus; Lymphoma extranodal NK-T-cell; Prognosis; Skin neoplasms.