Extramedullary facial plasmacytomas with anaplastic features: a diagnostic dilemma with implications for treatment

Extramedullary plasmacytomas (EMPs) are rare plasma-cell tumors of the soft tissue that occur predominantly in the nasal sinuses and oropharynx. Subcutaneous and cutaneous plasmacytomas of the face are distinctly unusual. The authors report a case of rapidly expanding EMP involving the lip and contralateral nasolabial fold of a native Alaskan man with a 25-year history of recurring solitary bone plasmacytomas (SBP). An incisional biopsy revealed sheets of monotypic plasmablasts with anaplastic features. The pathologic and clinical findings were most consistent with a Richter transformation from a low-grade to a high-grade malignancy, or anaplastic myeloma (AM). With combined chemotherapy and radiation therapy, he achieved a complete response. The clinical and laboratory features of this most unusual plasma-cell dyscrasia are reviewed with an emphasis placed on diagnosis and treatment.