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Published on: October 3, 2010
Case study of intracerebral plasmacytoma as an initial presentation of multiple myeloma
Aurelia Wavre1, Audrey S Baur, Michael Betz
1Department of Oncology, University Hospital CHUV , Lausanne, Switzerland.
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.
Insights
This case highlights an unusual presentation of multiple myeloma with initial cerebral involvement, emphasizing the poor prognosis of central nervous system (CNS) myeloma.
Area of Science:
- Neurology
- Oncology
- Hematology
Background:
- Multiple myeloma, a plasma cell malignancy, rarely presents with central nervous system (CNS) involvement.
- Early detection and treatment are crucial for managing multiple myeloma, but CNS complications pose significant challenges.
Observation:
- A 64-year-old man presented with a partial seizure, revealing two intracerebral plasmacytomas on MRI.
- The patient was diagnosed with immunoglobulin G lambda-type multiple myeloma with CNS involvement, notably without prior overt signs of the disease.
- Cytogenetic analysis identified a critical p53 gene deletion (17p13.1) in plasma cells.
Findings:
- Despite aggressive treatment including cranial radiotherapy and systemic chemotherapy, the patient experienced rapid disease progression.
- The case underscores the extremely poor prognosis associated with CNS myeloma, even with intensive therapeutic interventions.
- The presence of cytogenetic abnormalities, such as the p53 deletion, may indicate heightened genetic instability and a more aggressive myeloma phenotype.
Implications:
- This case emphasizes the importance of considering CNS involvement in atypical multiple myeloma presentations.
- The findings suggest that genetic markers like p53 deletion could predict treatment resistance and aggressive disease course in multiple myeloma.
- Further research into the role of cytogenetic abnormalities in CNS myeloma is warranted to improve patient outcomes.