Neurological recovery from pentaplegia after resection for sacral plasmacytoma presenting with POEMS syndrome

Case report

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The authors describe a case of a 52-year-old woman in whom tetraplegia developed with neurological respiratory failure due to POEMS syndrome associated with a solitary sacral plasmacytoma. Resection was finally performed after her condition proved resistant to radiation and chemotherapy. The patient showed a dramatic recovery and was ambulatory without tumor recurrence after 5 years and 6 months of follow-up. To the authors' knowledge, there are only 3 reported cases in the literature of bilateral phrenic nerve palsy leading to respiratory failure treated by chemotherapy. This is the first report describing neurological recovery after surgery for pentaplegia due to POEMS syndrome associated with solitary sacral plasmacytoma.

Abbreviations used in this paper: POEMS = polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes; VB = vertebral body.

Article Information

Address correspondence to: Yoshihisa Kotani, M.D., Department of Orthopaedic Surgery, Hokkaido University Graduate School of Medicine, Kita-15, Nishi-7, Kitaku, Sapporo 060-8638, Japan. email: y-kotani@med.hokudai.ac.jp.

© AANS, except where prohibited by US copyright law.

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Figures

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    Imaging studies of the solitary sacral plasmacytoma. A: The lesion demonstrated mixed osteolytic and osteoblastic changes. B: A CT scan demonstrated the destruction of the anterior and posterior sacrum with spinal canal involvement. C: Sagittal T1-weighted image demonstrated both low and high intensity signal. D: The tumor showed strong enhancement on the Gd-enhanced image. E: Sagittal T2-weighted image demonstrating mixed low and high intensity of the mass. F: The tumor involved the neural tissue, sacrum, and right ilium.

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    A: The malleable jelly-like tumor at S1–3 (arrow) and the posterior part of the sacral tumor were resected en bloc. The tumor surrounding the dura mater and nerve root, as well as the ventral part at the VB, was resected piecemeal with an ultrasonic aspirator. B: The tumor was completely removed and the dura and nerve root were exposed. No spinal reconstruction procedure was performed due to the patient's immunosuppressed state.

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    A: The posterior part of the tumor was resected en bloc. The malleable jelly-like tumor was demonstrated. B and C: Photomicrographs demonstrating cytological atypia such as multinuclear changes and dense proliferation of plasma cells. H & E, original magnification × 40 (B) and × 400 (C).

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    A and B: The CT images obtained at the final follow-up of 5 years and 6 months demonstrated bone remodeling at the resection site. There was no apparent tumor recurrence.

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