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POEMS syndrome associated with plasmacytoma of the clivus: "Time discovers the truth"
J Thomas1, B V Maramattom2, J Varghese3
1Department of Rheumatology, Lourdes Hospital, Kochi, Kerala, India.
Insights
POEMS syndrome, a rare disorder, involves neuropathy and organ issues. This case highlights a rare association between POEMS syndrome and a clival plasmacytoma, expanding understanding of this condition.
Area of Science:
- Neurology
- Oncology
- Hematology
Background:
- POEMS syndrome is a rare multisystem disorder associated with a monoclonal plasma cell disorder.
- Key features include peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes.
- Solitary plasmacytomas typically occur in bone but can be extramedullary.
Observation:
- This report details a rare case of POEMS syndrome.
- The case presented with a plasmacytoma located in the clivus, an unusual site.
Findings:
- The patient exhibited a rare co-occurrence of POEMS syndrome and a clival plasmacytoma.
- This finding expands the known spectrum of POEMS syndrome manifestations.
Implications:
- Understanding rare presentations like clival plasmacytoma is crucial for accurate POEMS syndrome diagnosis.
- This case may inform diagnostic criteria and treatment strategies for POEMS syndrome.
- Further research into extramedullary plasmacytomas in POEMS syndrome is warranted.
Abstract:
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following features: Osteosclerotic myeloma, Castleman's disease (angiofollicular lymph node hyperplasia), increased levels of serum vascular endothelial growth factor (VEGF), organomegaly, endocrinopathy, edema, typical skin changes, and papilledema. [1] Solitary plasmacytomas most frequently occur in the bone but can also be found in soft tissues (extramedullary plasmacytoma). [2] We report a case that showed a rare association of POEMS syndrome with a plasmacytoma of the clivus.
