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.
Aboulafia
D M
DM
Section of Hematology/Oncology, Virginia Mason Medical Center, Seattle, Washington 98111, USA.
Lee
R M
RM
Hafermann
M
M
Chu
F W
FW
Fenske
M
M
D000328:Adult; D001859:Bone Neoplasms / Q000473:pathology*; D002471:Cell Transformation, Neoplastic; D003131:Combined Modality Therapy; D005153:Facial Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D009101:Multiple Myeloma / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D009364:Neoplasm Recurrence, Local / Q000473:pathology*; D010954:Plasmacytoma / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy