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CNS localization of multiple myeloma

Thursday 12 March 2009

Multiple myeloma is characterized by the presence of neoplastic proliferating plasma cells; the tumor is generally restricted to the bone marrow. In less than 1 % of the cases a CNS involvement is observed including localized intraparenchymal lesions, solitary cerebral plasmocytoma or CNS myelomatosis (= presence of monoclonal cells in the CSF).

The etiology of CNS infiltrating multiple myeloma is unknown and the hypothesis includes a continuous spread from eroded lytic skull lesions or hematogenous spread of tumor cells favored by the fact that most MM drugs do not reach the brain parenchyma.

The prognosis of patients with MM CNS manifestation is in general poor; Nieuwenhuizen reported a survival time between 0.1 and 25 months.

Histopathologically, localized intraparenchymal lesions of CNS MM are characterized by the presence of neoplastic, proliferating plasma cells which are occasionally multinucleated as observed in our cases.

Case studies

- case UPMC #599

See also

- multiple myeloma


- Chang H, Sloan S, Li D, Keith SA (2004) Multiple myeloma involving central nervous system: high frequency of chromosome 17p13.1 (p53) deletions. Br J Haematol 127: 280-284.

- Fassas AB, Muwalla F, Berryman T et al (2002) Myeloma of the central nervous system: association with high-risk chromosomal abnormalities, plasmablastic morphology and extramedullary manifestations. Br J Haematol 117: 103-108.

- Kuhlmann T, Remington L, Maruschak B, Owens T, Bruck W (2007) Nogo-A is a reliable oligodendroglial marker in adult human and mouse CNS and in demyelinated lesions. J Neuropathol Exp Neurol 66: 238-246.
Lupu VD, Saini N, Balish M (2005) CNS myelomatosis. Neurology 64: 1007.

- Nieuwenhuizen L, Biesma DH (2008) Central nervous system myelomatosis: review of the literature. Eur J Haematol 80: 1-9.

- Seftel MD, Maguire J, Voss N, Woodhurst WB, Dalal BI, Shepherd JD (2002) Intra-cerebral relapse following prolonged remission after autologous stem cell transplantation for multiple myeloma. Leuk Lymphoma 43: 2399-2403.

- Veinstein A, Brizard A, Randriamalala E, Babin P, Preud’homme JL, Guilhot F (1997) Central nervous system relapses after autologous stem cell transplantation for myeloma. Report of two cases. Hematol Cell Ther 39: 327-330.