Extramedullary Plasmacytoma

Extramedullary plasmacytoma is a plasma cell tumor that arises outside the bone marrow. Extramedullary plasmacytoma accounts for up to 3% of plasma cell malignancies.15 The median age of patients is about 60

years, and the majority of patients are male.15 Approximately 80% of tumors involve the upper respiratory tract, including the sinuses and nasal cavity, larynx, and nasopharynx. Epistaxis, rhinorrhea, and nasal obstruction are the most frequent symptoms.

In a review of 714 cases of extramedullary plasma-cytoma, 82% involved the upper respiratory tract. Seventy-four percent of plasmacytomas involving the upper aerodigestive tract occurred in male patients. Extramedullary plasmacytomas in the 155 cases not involving the upper aerodigestive region were in the gastrointestinal tract (40%), urogenital region (25%), skin (17%), lung (10%), breast (4%,) conjunctiva (2%), and retroperitoneum (1%).20

In a review of the literature, Anghel et al.21 described 17 patients with a primary solitary testicular plasmacy-toma at diagnosis. The median age was 53.5 years. Nine patients died of progressive disease, six were alive without evidence of disease, and two were alive with disease. Twenty-five patients with plasmacytoma localized to the lymph nodes were reported.22 Cervical nodes were involved in 56%, abdominal in 16%, and mediastinal in 12%. Bone marrow examinations and metastatic bone surveys showed no evidence of multiple myeloma. Ten patients had localized disease, and none had development of multiple myeloma.

Pulmonary involvement may occur. Koss et al.23 reported two patients with extramedullary plasmacy-toma involving the mediastinal lymph nodes and three with parenchymal involvement. When the authors considered their 5 cases in combination with 14 others from the literature, the overall 2-year survival rate was 66%, and the 5-year survival rate was 40%.23

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