PAEDIATRIC-06 PEDIATRIC RECURRENT PRIMARY OCCIPITAL SYNOVIAL SARCOMA: A CASE REPORT

BACKGROUND: Synovial sarcoma is one of the malignant soft tissue sarcomas with an unknown histological origin. It has a unique t(X;18) (p11.2; q11.2) chromosomal translocation, resulting in the SYT-SSX fusion protein. The intracranial occurrence is mainly metastatic, and primary intracranial synovial sarcoma is extremely rare, with few case reports and case series in the literature.Surgical resection of the tumor followed by adjuvant radiotherapy and chemotherapy, regardless of the extent of tumor resection, has been shown to have a significant benefit. CASE PRESENTATION: Here we report the case of an eleven-year-old male patient who presented with a four-month history of swelling at the occipital area and intermittent headache. Imaging showed a destructive skull vault lesion with infiltration of the ipsilateral cerebellum. A subtotal resection of the tumor was achieved, and histopathology examination revealed a biphasic variant of synovial sarcoma. The patient disappeared from follow-up without receiving adjuvant radiotherapy and presented two years later with a swelling at the previous surgical site. After imaging was done, a recurrence was diagnosed, and he was reoperated with near total excision. CONCLUSION: Primary intracranial synovial sarcoma is an extremely rare malignant tumor. Surgical resection with adjuvant radiation should be the standard of care to prevent recurrence. In low-income countries, patients disappear from follow-up, making post-operative management difficult. Furthermore, radiation services should be made widely available to address issues with compliance.