Skull base plasmacytoma: A unique case of POEMS syndrome with a plasmacytoma causing craniocervical instability

Hannah Gilder, Meghan E. Murphy, Mohammed Ali Alvi, Panagiotis Kerezoudis, Daniel Shepherd, Patrick R. Maloney, Michael J Yaszemski, Jonathan M. Morris, Angela Dispenzieri, Jane M. Matsumoto, Mohamad Bydon

Research output: Contribution to journalArticlepeer-review

Abstract

Introduction Plasmacytomas, considered to be the solitary counterparts of multiple myeloma, are neoplastic monoclonal plasma cell proliferations within soft tissue or bone. Plasmacytomas often present as a collection of findings known as POEMS-syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein spike, and Skin changes). Case description We present a report of a 47 yo male diagnosed with POEMS-syndrome secondary to a skull base plasmacytoma. The mass resulted in marked instability of the cranio-cervical junction due to bony erosion. Following an induction course of chemotherapy, he showed clinical improvement with a marked reduction in tumor size and underwent an autologous peripheral blood stem cell transplant for systemic treatment of his POEMS-syndrome. Following completion of systemic treatment, he then underwent a definitive occipital-cervical fusion without complications. His neurologic exam upon dismissal was stable with subjective improvement in left upper extremity strength. Postoperative radiographs confirmed spinal alignment and pathological examination of a small biopsy from C1 revealed benign fibrous tissue. Conclusion To the best of our knowledge, this is the first report of a skull-base plasmacytoma associated with POEMS-syndrome, causing cranio-cervical instability. The approach of systemic therapy combined with temporary external fixation, followed by definitive occipital cervical fusion resulted in a good outcome for this patient.

Original languageEnglish (US)
Pages (from-to)254-257
Number of pages4
JournalJournal of Clinical Neuroscience
Volume47
DOIs
StatePublished - Jan 1 2018

Keywords

  • Craniocervical mass
  • Plasmacytoma
  • POEMS syndrome
  • Spinal oncology
  • Spine surgery

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Physiology (medical)

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