Although rare, synovial sarcoma is the most common malignant non-rhabdomyosarcomatous soft tissue sarcoma in children and adolescents. Synovial sarcoma typically involves the soft tissues of the extremities, especially near large joints, but it can occur anywhere in the body in locations far from joint spaces. Although this tumor typically affects adults in their fourth decade of life, nearly half of the reported cases have been children and adolescents. We report a rare case of head and neck synovial sarcoma presented with tonsilar hemorrhage and painful facial contracture. Cervical computed tomography (CT) scan with contrast injection showed an asymmetrical respiratory tract image. Significant thickening of right (anterior) parapharyngeal soft tissue was revealed indicating a soft tissue mass. The mass was completely resected in a surgical procedure. In pathological examination of the mass, biphasic synovial sarcoma was reported. At first, we treated the patient with VIE (Vincristin, Ifosfamide, Etoposide),but because of the severe neutropenia and hemorrhagic cystitis, we changed the protocol to VAC (Vincristin, Actinomycine, Cyclophosphamide). The girl is 6 years old now and in a good condition without any pain or bleeding. Also, she has a normal cervical CT scan after more than two years. She is followed up every week and receives monthly Vincristin.

Keywords: synovial sarcoma, pediatrics

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