Giant-sized primary clear cell chondrosarcoma of the thoracolumbar spine: A very rare case report

Abstract

Background: Clear cell chondrosarcoma (CCC), a rare subtype of chondrosarcoma, is generally a low-grade tumor that arises commonly in the epiphyses of the proximal femur or humerus. The spine is an uncommon site of CCC that most commonly involves the thoracic spine with the lumbar spine as the second most common predilection.

Methods: We report an extremely rare case of a 22-year-old female with a giant-sized CCC in the thoracolumbar region that severely compressed the spinal cord on that level. Tumor removal, spinal cord decompression, and stabilization were done, followed by high-dose radiotherapy.

Results: Histopathology demonstrates CCC with high cellularity, some hemorrhagic areas, and scanty mitotic figures. The patient had a significant back pain improvement. There were some recoveries of neurological function by the time of the last follow-up.

Conclusions: This report highlights the unusual location of this rare tumor, the unusual size, and moreover, the age at presentation. Despite its low-grade behavior, CCC needs to be treated as a true malignancy with wide resection whenever feasible. Adjuvant radiotherapy should also be considered as part of the surgical treatment because of the risk of de-differentiation, recurrence, and malignancy, especially in unresectable cases or cases with incomplete resection.

Keywords: Chondrosarcoma; Clear cell chondrosarcoma; Giant-sized tumor; Thoracolumbar spine.

Fig. 1

Thoracolumbar plain radiograph. (A) AP view showed a winking owl sign, without reduction in vertebral height nor increase in width. (B) Lateral view showed a lytic lesion over posterior aspect and loss of vertebral body trabeculation.

Fig. 2

Sagittal view of CT scan and MRI (A–D) and axial view CT scan and MRI (E–F). (A) The 2-D image demonstrates spinal mass over the T12 extending cranially to infero-posterior aspect of T11 and caudally up to L2. (B) The 3-D image showed bony destruction particularly on the posterior aspect of T12-L1. Contrast-enhanced T1-w (C) and T2-w (D) MR image of the lesion, showing paravertebral multi-focal soft tissue mass from T11-L2 and spinal canal compression at that level. The mass had an intense heterogenous enhancement in both T1-w and T2-w image, with predominantly high signal intensity on T2-w image. (E) The axial CT image demonstrates a soft tissue mass without calcified matrix that obliterate the spinal canal. (F) The axial T2-w demonstrates a heterogenous high intensity mass with hemorrhagic component (fluid-fluid level) that obliterate the spinal canal.

Fig. 3

Tissue sample demonstrating sheets of round to ovoid cells with clear cytoplasm. (A) 4x; (B) 10x; (C) 40x.