Presentation
Acute onset bilateral neck pain.
Patient Data
Large well-circumscribed lobulated soft tissue mass extending from C2 to C5 level. It extends through left C3/4 and C4/5 exit foramina and compresses on the left posterior aspect of the hypopharynx.
There is encasement of the left vertebral artery from C2 to C5 level with slight narrowing.
The component within the spinal canal compresses the spinal cord, which is displaced posteriorly, and to the right side most marked at C3 and C4 level. There is no associated cord edema.
The mass is T1 isointense to hypointense, T2/STIR hyperintense with minimal heterogeneous enhancement associated with paravertebral component.
HISTOLOGY
MACROSCOPIC DESCRIPTION: Cervical spine tumor: Multiple fragments of white / tan tissue. 50 x 40 x 17 mm in aggregate.
MICROSCOPIC DESCRIPTION: Sections show large vacuolated epithelioid cells which are in cords and nests and are surrounded by septa containing inflammatory cells and foamy cells. Areas of myxoid matrix are also seen. Many of the cells have a glassy cytoplasm and have a chondroid type of appearance. There is some nuclear atypia seen but no evidence of significant increase in mitoses. The immunostains show positive staining with EMA (Epithelial Membrane Antigen) and S100. Brachyury stain is positive, consistent with a chordoma. The overall features favor a chordoma.
Case Discussion
This tumor demonstrates typical features of chordoma: low T1 signal intensity, high T2 signal intensity, T1+C heterogeneous enhancement. Vertebral body chordomas account for 15-30% of total incidence 1.
The patient, in this case, went on to have surgical resection and then radiotherapy, as is typical management.
Case Contributors: Dr Blake Milton and Dr Udit Nindra
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