Malignancy: Meningeal Myeloma: A Case Report and Review of Literature

Purpose: Report and review of an uncommon complication of multiple myeloma. Methods: We report a case of leptomeningeal myeloma and using Medline, review previously reported cases. Results: We found 35 previously reported cases of meningeal myeloma. Of the 36 cases, including the present case, the male to female ratio was 2:1 with a median age of 58 years (range, 31 to 83 years). Forty five percent of the patients had circulating plasma cells and 90.9% had plasma cells in the cerebrospinal fluid. All but 4 cases were diagnosed antemortem. Thirty patients had a pre-existing diagnosis of multiple myeloma, while leptomeningeal disease was the presenting feature in six. Management included a combination of intrathecal chemotherapy and radiation therapy, radiation therapy alone or intrathecal chemotherapy alone in 14, 3, and 3 patients respectively. Thirteen patients also received systemic chemotherapy in addition to some form of local therapy. The median survival for the entire group was 8 weeks (range 4 days to 18 months). Even those who initially responded to local therapy relapsed in the central nervous system (CNS) or succumbed to systemic disease. Five of six patients in whom CNS disease was the presenting feature survived 4 weeks or less. Conclusions: Leptomeningeal involvement is a rare and fatal complication of multiple myeloma with a short survival despite aggressive intrathecal and systemic chemotherapy and radiation therapy.