ABSTRACT
Cerebral involvement is an uncommon complication of multiple myeloma. We report on a 64-year-old man hospitalized for a partial seizure. MRI showed two intracerebral lesions, which proved to be plasmacytomas. After complete staging, we retained the diagnosis of immunoglobulin G lambda-type multiple myeloma with CNS involvement. Cytogenetic analysis of plasma cells detected a deletion in the p53 gene at 17p13.1. Despite cranial radiotherapy and systemic chemotherapy, the patient's disease progressed rapidly and he died five months after diagnosis. What makes this case unusual is that overt multiple myeloma had been absent before cerebral involvement was discovered. It confirms the extremely poor prognosis of patients with CNS myeloma even in the presence of aggressive treatment. Cytogenetic abnormalities could be a marker of chromosomal and genetic instability, conferring to multiple myeloma a more aggressive profile.
Subject(s)
Brain Neoplasms/pathology , Multiple Myeloma/genetics , Multiple Myeloma/pathology , Plasmacytoma/pathology , Brain Neoplasms/genetics , Brain Neoplasms/therapy , Diabetes Mellitus, Type 2/pathology , Fatal Outcome , Gene Deletion , Genes, p53 , Humans , Immunohistochemistry , In Situ Hybridization , Magnetic Resonance Imaging , Male , Middle Aged , Multiple Myeloma/therapy , Plasmacytoma/genetics , Plasmacytoma/therapyABSTRACT
Primary indolent leptomeningeal lymphoma is a rare entity, and corresponds in most cases to mucosa-associated lymphoid tissue (MALT) type lymphoma. We are reporting a case of a 75 years old woman, who presented with a 2-year history of behavioral disorder, progressive memory loss and aphasia. Neuroimaging showed a mass infiltrating the frontal circumvolutions and the roof of the orbit. The biopsy revealed an infiltration of the dura by an indolent lymphoma, characteristic of a MALT-type lymphoma. Complete staging work-up did not show any evidence of systemic involvement. A treatment with systemic methotrexate, combined with intrathecal chemotherapy and followed by radiotherapy (30,6 Gy) of the primary site, was conducted. The 3-year follow-up confirms the persistent remission, the patient remaining well and free of symptoms. The review of the literature highlights the importance to recognize the indolent PLML as a distinct clinical entity, which exhibits a rather good prognosis following a relatively non-toxic therapy.
