William Remley1, Xiaojun Li1, Bhavin Patel1, Kyle Ruffing1
1Neurology, University of Florida
Background:
Progressive polyradiculoneuropathy that mimics CIDP may result from a paraneoplastic etiology in a constellation of symptoms known as POEMS syndrome. It should be considered when findings are atypical, and standard treatments for CIDP are unsuccessful. A high index of suspicion is required from neurologists to recognize the syndrome in its sea of medical problems. POEMS syndrome results from a plasma cell dyscrasia, commonly a plasmacytoma involving the bone marrow. Extramedullary plasmacytomas account for less than 4% of cases, most frequently in the upper aerodigestive tract. Isolated hepatic plasmacytomas are exceedingly rare and have not previously been reported in association with POEMS syndrome. Treatment involves targeting the plasma cell dyscrasia with surgery, radiation therapy, or chemotherapy.
Design/Methods:
NA
A 55-year-old man presented after four months of progressive ascending length-dependent sensorimotor weakness with 0/5 lower extremity strength and 3/5 upper extremity strength without response to steroids, IVIg, or plasmapheresis. EMG/NCS showed a primarily axonal length-dependent sensorimotor polyneuropathy. Investigation showed CSF albuminocytologic dissociation, polycythemia, hypogonadism, a monoclonal IgG lambda gammopathy, splenomegaly, anasarca, and elevated VEGF. Biopsy of a 3cm hepatic lesion was consistent with a plasmacytoma.
Results:
The patient met both mandatory criteria of polyneuropathy and plasma cell dyscrasia, major criteria of elevated VEGF and minor criteria including organomegaly, extravascular volume overload, endocrinopathy, polycythemia, and other classic symptoms including weight loss, venous thromboembolism. He was treated with chemotherapy and radiation therapy.
Conclusions:
Chronic progressive polyneuropathies that mimic CIDP and occur alongside plasma cell dyscrasias should raise suspicion for POEMS syndrome. The purpose of this case presentation is to report a unique extramedullary site of plasmacytoma in the liver, as our review of the literature shows only one other case of a hepatic plasmacytoma associated with polyneuropathy. This is the first reported case of an isolated hepatic plasmacytoma associated with POEMS syndrome.
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