gms | German Medical Science

Introduction: Synovial sarcomas usually occur in extremity junctions. Only 5-10% of head and heck sarcomas are synovial and are localized in the larynx, hypopharynx, tongue or in the parapharyngeal space. Infratemporal or suboccipital manifestations with intracranial extension in the posterior cranial fossa are extremely rare.

Method: A 15 years old female presented with nuchal pain after light trauma. The CT revealed a large tumor with osteolysis of the right occipital bone, the MRI showed a soft tissue tumor in the posterior cranial fossa with compression of the cerebellum and extension to the clivus and the foramen magnum. After histological diagnosis of sarcoma, the tumor has been removed macroscopically in toto but without safe margins via a transmastoidal, transsigmoidal, infratemporal approach. The final histology revealed a synovial sarcoma. Thus, an aggressive radio-chemotherapy followed.

Result: Preservation of cranial nerves was attempted and only the ipsilateral hypoglossal nerve was sacrificed. The patient is after 10 months free of pain and still free of disease.

Discussion: There is only limited experience in treatment and outcome of the very rare synovial sarcomas of the skull base. Since prognosis of synovial sarcomas of other localizations is very poor, an aggressive combined therapy is suggested. However, surgical treatment should not result in additional functional disturbances that impair quality of life.