Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
August 2017
Next Case of the Month coming September 5...
POEMS Syndrome
- Background:
- Clinical syndrome comprised of the constellation of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes
- The etiology is unknown, but proinflammatory cytokines seem to be involved, including increased VEGF in blood, as well as IL-6, TNF-α, and Interleukin-1β.
- The prognosis and response to treatment of POEMS have even been tracked using VEGF levels, which are minor criteria for diagnosis of the disease.
- This case was atypical in that initial images suggested lumbar metastatic disease, a primary intracranial CNS malignancy.
- Clinical Presentation:
- The major criteria include polyneuropathy and a plasma cell dyscrasia, typically monoclonal gammopathy.
- Patients typically present with neurologic symptoms, with varying degrees of sensorimotor deficit, such as weakness, paralysis, and paresthesia.
- Key Diagnostic Features:
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Most common in the setting of osteosclerotic myeloma—typically single lesions—found in the axial skeleton, flat bones, and long bones
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A mixed lytic-/blastic-type of osseous involvement may also be seen. Predominantly lytic lesions are atypical in this entity.
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T2WI and FLAIR have shown to be very useful, demonstrating diffuse thickening of the dura.
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Postcontrast T1WI demonstrates thickened, enhancing dura and spinal nerves.
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Additional findings may mimic CNS malignancy like a “drop” metastasis appearance, such as that observed in this patient.
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- Differential Diagnoses:
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The differential is mainly similar to that of dural enhancement: meningitis, metastasis, intracranial hypotension, neurosarcoidosis, postoperative trauma
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- Treatment:
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Therapy includes radiation and chemotherapy
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No consensus on systemic therapy, but studies have shown success with lenalidomide and other “IMiD” drugs, as well as melphalan, and dexamethasone
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Proteasome inhibitors have also been used.
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