Aggressive Cytotoxic Lymphomas of the Auricle: An Under-recognized Presentation

Primary cutaneous acral CD8+ T-cell lymphoproliferative disorder has been recognized by the World Health Organization as a distinct entity characterized by dermal-based, nonulcerated nodules typically involving the auricle and following an indolent course. By contrast, cytotoxic cutaneous lymphomas presenting with tumoral lesions of the auricle can follow a locally aggressive or metastatic course. In this study, we discuss the presentation, diagnosis, molecular characteristics, and treatment options for these under-recognized, aggressive cytotoxic lymphomas of the auricle. Six cases of cytotoxic lymphoma NOS and 4 cases of CD4+ primary cutaneous γδ T-cell lymphoma that presented as auricular tumors were included in this series. Patches at other sites resembling mycosis fungoides with variable immunophenotypes were observed in 7 cases. Despite multimodal therapy, 7 of 10 patients died within median 14 months of presentation. Necrotizing and locally progressive tumors of the auricle may represent an ominous sign of aggressive treatment-refractory cytotoxic lymphomas characterized by swift mortality.