Primary cutaneous histiocyte and neutrophil‐rich CD30 + and CD56 + anaplastic large‐cell lymphoma with prominent angioinvasion and nerve involvement in the forehead and scalp of an immunocompetent woman

Background:  Cutaneous lymphomas co‐expressing CD56 and CD30 are very rare. They share a clinicopathological overlap with natural killer‐ (NK)/T‐cell lymphomas and anaplastic large‐cell lymphomas (ALCLs), two entities with widely disparate clinical behavior. Methods:  We present a case of an immunocompetent 57‐year‐old Caucasian woman with a rapidly growing, angiodestructive and neuroinvasive primary cutaneous ALCL (PCALCL). The neoplastic population of large anaplastic CD30 + and CD56 + T cells was masked by a massive admixture of histiocytes and neutrophils. The partially ulcerated and pus‐secreting tumor involved the forehead and scalp and was assessed as clinical stage IAE. Results:  After chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone), the patient achieved a complete remission. Additionally, high‐dose chemotherapy with autologous peripheral blood stem‐cell transplantation was administered as a consolidation of complete remission, in which she has remained for 6 years. Conclusions:  This is the first CD30 + and CD56 + primary skin lymphoma to be reported on the head. The presented case carries a remarkable combination of clinicopathological features of PCALCL and NK‐/T‐cell lymphoma.