Primary intradural extramedullary spinal mesenchymal chondrosarcoma: Case report and literature review
Journal
BMC Musculoskeletal Disorders
Journal Volume
20
Journal Issue
1
Date Issued
2019
Author(s)
Abstract
Background: Mesenchymal chondrosarcoma (MCS) is a rare malignant variant of chondrosarcoma with a high tendency of recurrence and metastasis. Intradural extramedullary spinal MCS is exceedingly rare and usually found in pediatric patients. Herein, we present an elderly patient with primary intradural extramedullary spinal MCS. Relevant literatures are reviewed to disclose characteristics of intradural extramedullary spinal MCS. Case presentation: A 64-year-old female presented with urinary difficulty and tightness of upper back preceding progressive weakness of right lower extremity. Magnetic resonance imaging revealed an intradural extramedullary tumor at the level of 3rd thoracic vertebra. This patient underwent total tumor resection and then received adjuvant radiotherapy. Histopathological examination showed that the tumor composed of spindle and round cells with high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix. Along with immunohistochemical findings and the existence of HEY1-NCOA2 fusion transcript, the diagnosis of MCS was confirmed. Neurologic deficit recovered nearly completely after surgery. No evidence of local recurrence or distant metastasis was found 5 years after treatments. Including the current case, a total of 18 cases have been reported in the literature with only one case with local recurrence and one case of mortality. The current case was the eldest patient diagnosed with primary intraspinal MCS in the literature. Conclusions: MCS rarely appears in the intradural space of the spine. In contrast to classic MCS, treatment outcome of primary intradural extramedullary spinal MCS is usually excellent as total tumor resection is commonly achievable. Adjuvant radiotherapy may reduce local recurrence and chemotherapy may be associated with fewer recurrences especially for unresectable tumors. ? 2019 The Author(s).
SDGs
Other Subjects
basic helix loop helix transcription factor; cell cycle protein; HEY1 protein, human; NCOA2 protein, human; nuclear receptor coactivator 2; adjuvant radiotherapy; case report; chondrosarcoma; diagnostic imaging; dura mater; female; genetics; human; laminectomy; middle aged; nuclear magnetic resonance imaging; pathology; spinal cord tumor; spine fusion; surgery; treatment outcome; Basic Helix-Loop-Helix Transcription Factors; Cell Cycle Proteins; Chondrosarcoma, Mesenchymal; Dura Mater; Female; Humans; Laminectomy; Magnetic Resonance Imaging; Middle Aged; Nuclear Receptor Coactivator 2; Radiotherapy, Adjuvant; Spinal Cord Neoplasms; Spinal Fusion; Treatment Outcome
Publisher
BioMed Central Ltd.
Type
journal article