Vertebral synovial chondromatosis

Report of two cases and review of the literature

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✓ Synovial chondromatosis is an uncommon disorder characterized by the formation of multiple cartilaginous nodules within the synovium, most commonly affecting large joints. Its involvement with the spine is rare; only six cases have been reported. The authors describe two patients with synovial chondromatosis involving the cervical spine. In the first case, synovial chondromatosis arose from the left C1–2 facet joint. This patient underwent a two-stage procedure including a posterior approach for tumor resection and occipitocervical fusion as well as a transmandibular circumglossal approach to the anterior craniocervical junction to complete the tumor removal. Interestingly, on histopathological examination, scattered foci of low-grade chondrosarcoma were intermixed within the synovial chondromatosis. To the authors' knowledge, this is the first report of secondary low-grade chondrosarcoma arising in vertebral synovial chondromatosis. In the second case, synovial chondromatosis involved the left C4–5 facet joint. Tumor resection and cervical fusion were performed via a posterior approach.

In this report, the authors describe the clinical presentation, radiographic findings, operative details, histopathological features, and clinicoradiological follow-up data obtained in these two patients and review the literature pertaining to this rare entity.

Article Information

Address reprint requests to: Ziya L. Gokaslan, M.D., Department of Neurosurgery, Johns Hopkins University School of Medicine, Meyer 7–109, 600 North Wolfe Street, Baltimore, Maryland 21287. email: zgokasll@jhmi.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Preoperative axial CT scans obtained through the upper cervical spine, demonstrating a mass with speckled calcification centered around the left C1–2 articulation with extension into the spinal canal (A–C) and prevertebral and left paracervical soft tissue (C and D).

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    Case 1. Preoperative cervical T1-weighted (A), T2-weighted (B), and Gd-enhanced T1-weighted (C) left parasagittal MR images demonstrating isointensity, hyperintensity, and partial enhancement, respectively, of the C1–2 mass. The anterior aspect of the thecal sac is compressed without significant spinal cord compression.

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    Case 1. A–D: Axial Gd-enhanced T1-weighted MR images revealing the partially enhancing mass involving the left C1–2 articulation with prevertebral and left paracervical soft-tissue and intraspinal extension. The mass extends laterally into the adjacent left parapharyngeal space, displacing the left carotid artery sheath laterally (B and C). Tumor is also present between the anterior arch of C-1 and the anterior aspect of the odontoid process, posterior to the odontoid process, and along the left lateral aspect of the spinal cord down to C-3.

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    Case 1. Postoperative anteroposterior (A) and lateral (B) cervical radiographs obtained after the second procedure, demonstrating the occipitocervical instrumentation.

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    Case 1. Photomicrographic studies showing well-defined extraosseous nodules of cartilage that contain individual clones of chondrocytes consistent with synovial chondromatosis (A); chondrocyte atypia with bi- and trinucleation (B); and cancellous bone with permeation of marrow spaces by cartilage, a pattern diagnostic of chondrosarcoma (C). H & E, original magnification × 10 (A and C); original magnification × 40 (B).

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    Case 1. Postoperative axial CT scans obtained through the upper cervical spine, demonstrating very small residual foci of calcification around the remaining anterior arch of C-1 (A and B) and the left paravertebral region (C and D).

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    Case 2. Preoperative cervical T1-weighted (A), T2-weighted (B), and Gd-enhanced T1-weighted (C) left parasagittal MR images. The mass extends from the level of the C-3 VB to the superior aspect of C-5. It is isointense on T1-weighted images, heterogeneous on T2-weighted images, and enhances following Gd administration. Ventral dural enhancement is noted superior and inferior to the mass (C).

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    Case 2. Axial Gd-enhanced MR image demonstrating the partially enhancing extradural mass displacing the spinal cord to the right.

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