Primary Intracranial Synovial Sarcoma

Abstract EN

Background.

Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin.

The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh.

Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma.

We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature.

Case Description.

A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia.

CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression.

Histopathology revealed synovial sarcoma.

FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation.

PET scan did not show other metastatic disease.

He underwent stereotactic radiotherapy and adjuvant chemotherapy.

At 2-year follow-up, he remained nonfocal without recurrence.

Conclusion.

We report the first known case of primary intracranial synovial sarcoma.

Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression.