Primary Intracranial Synovial Sarcoma.

Area of Science:

• Neuro-oncology
• Surgical Pathology
• Medical Genetics

Background:

• Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain origins, typically affecting areas near the knee.
• Intracranial metastasis from synovial sarcoma is rare; primary intracranial presentation has not been previously documented.
• A 21-year-old male with a history of skull lesion resection presented with neurological deficits.

Purpose of the Study:

• To report the first documented case of primary intracranial synovial sarcoma.
• To highlight the potential for intracranial hemorrhage in this rare tumor.
• To emphasize the need for prompt diagnosis and management of intracranial lesions.

Main Methods:

• Case report of a patient with neurological symptoms and a hemorrhagic brain lesion.
• Histopathological examination and Fluorescence In Situ Hybridization (FISH) analysis to identify synovial sarcoma and the characteristic t(X;18) translocation.
• Diagnostic imaging including CT and PET scans.
• Treatment involved surgical decompression, stereotactic radiotherapy, and adjuvant chemotherapy.

Main Results:

• Histopathology confirmed synovial sarcoma with the t(X;18)(p11.2;q11.2) translocation.
• The patient's PET scan showed no evidence of extracranial metastatic disease.
• Following treatment, the patient remained disease-free at 2-year follow-up.

Conclusions:

• This case represents the first instance of primary intracranial synovial sarcoma.
• Intracranial synovial sarcoma may present with hemorrhagic complications requiring urgent surgical decompression.
• Early detection and multimodal treatment are crucial for managing this rare and aggressive brain tumor.