Primary pleural synovial sarcoma in an adolescent: a case report

Primary pleural synovial sarcoma (SS) is an extremely rare entity, especially in the group of children and adolescents. Due to its rarity in this location and variable histogenesis, the diagnosis of SS is a clinical challenge. We report a new case of primary pleural SS in an adolescent. A 14-year-old boy initially referred to another facility for spontaneous right-sided chest pain and dyspnea, and was transferred to our hospital five days later for recurrent hemorrhagic pleural effusion in the right chest cavity. The items in routine haematological investigations were within normal limits, except for the lower level of eosinophil. CT scan revealed a heterogeneously enhancing solid mass occupied the upper two-thirds of the right hemithorax and massive pleural effusion in the lower half of the hemithorax. There was no evidence of hilar or mediastinal lymphadenopathy. Subsequently, an open thoracotomy was performed and the tumour was diagnosed as biphasic SS based on morphological and immunohistochemical analysis. The patient was discharged after surgical resection without complications and adjuvant chemotherapy was arranged. Currently, the patient is clinically well 6 months after surgery, with no evidence of recurrent disease. Though pleural SS is a rare anatomic subset of SS, it should be considered in the differential diagnosis of intrathoracic mass without hilar or mediastinal lymphadenopathy in adolescents, especially the cases with recurrent hemorrhagic pleural effusion.