Background: Head and neck synovial sarcoma (HNSS) are rare types of sarcomas of uncertain etiology. They appear predominantly
in young adults, with a peak incidence in the third decade. They are considered as a high-grade sarcoma with poor prognosis. Aims:
to study the clinicopathological features of all cases with HNSS, pathological subtypes, management protocols & the outcome of
such treatments as regard progression-free survival (PFS) & overall survival (OS). Materials & Methods: a retrospective analysis of all
cases presented to National Cancer Institute-Cairo University from January 2011 until December 2019. 16 cases were included. Data
collected then analyzed. Results: Mean age was 30.6 years with male to female ratio of 1.3:1. Anatomically; 5 cases were in the neck
soft tissues, 3 cases were in the cheek, 2 cases were in each of hypopharynx, oropharynx & temporal regions, one case in thyroid
gland and submandibular gland. Mean tumor size was 7.38 cm. Surgical resection was done in all patients; postoperatively, 4 cases
(25%) had R0 margins with no further treatment; twelve cases had (R1/R2) resection. Five patients (~31%) developed metastasis
and 3 patients (~19%) developed both local and distant metastases; lungs were commonest site (~94%). Median follow-up was 57.2
months (range, 6.76–100 months). Overall survival at 1, 3, and 5 years were 95.1%, 71.4% & 42.9% respectively while the
corresponding PFS were 68.6%, 30.7% & 16%. Conclusion: Surgery is the primary treatment for localized tumors.
Radiotherapy/chemotherapy is options. Initial tumor size & margins of resection was the main predictive of survival.
Keywords: synovial sarcoma, surgical resection, localized tumors, chemotherapy