Chronic acquired polyneuropathy as presentation of skull based solitary bone plasmacytoma

Abstract

Plasmacytomas can be solitary or multiple (osteosclerotic myeloma). Neuropathy resembling chronic inflammatory demyelinating polyradiculoneuropathy or POEMS syndrome has rarely been reported in literature in association of cranial vault plasmacytoma. A female patient with severe demyelinating sensori-motor polyneuropathy with conduction block, was found to have solitary skeletal lesion in the left frontal bone which confirmed to be a plasma cell neoplasm on biopsy. She had significant improvement in neurological signs and symptoms following local radiation and chemotherapy over next 3 months. Solitary bone plasmacytoma (SBP) is rare, accounting for 2-5% of all plasma cell neoplasms. The axial skeleton, especially vertebral body, is the most common site and cranial vault is one of the least commonly reported. Radiation therapy (RT) is the treatment of choice for SBP. There is no evidence that plasma exchange, IVIg or other immunosuppressive agents are effective when used alone in treatment of SBP-associated neuropathy.