An Unusual Case of Primary Pulmonary Synovial Sarcoma

Background : An extremely rare intrathoracic tumour that typically affects young adults is primary pulmonary synovial sarcoma. It manifests as a big intrathoracic mass without involvement of the bone or mediastinal region. Tumour biopsy, immunostaining and histologic analysis can assist in determining the kind of tumour. Case Presentation : We present a case of a 25 year old male with complaints of breathlessness and chest pain for a month. On radiological imaging he was found to have mass in upper and middle zones of right hemithorax. For confirmation of diagnosis patient underwent ultrasound guided biopsy of the lesion and histopathologhical examination (HPE) of the lesion. The histopathological features were suggestive of a rare type of lung malignancy. For further diagnosis regarding type of malignancy, immunohistochemical staining of the lesion was done with the help of with specific immunological markers, which confirmed the diagnosis of monophasic pulmonary sarcoma. On PET (positron emission tomography) CT (computed tomography) scan there were no features suggestive of distant metastasis especially in extremities which confirmed the diagnosis of primary pulmonary synovial sarcoma. Patient was started on chemotherapy regimen of Ifosfamide and Doxorubicin after oncologist’s opinion but was lost to follow up after 3 ^rd cycle of chemotherapy. Conclusions : Primary pulmonary synovial sarcoma is an extremely uncommon tumor in young adults as lung metastases from other sources mainly extremities are more common. Diagnosis of such rare tumour requires histopathological examination along with immunohistochemical staining of the lung lesions. A multimodal strategy of treatment involving resection, chemotherapy and radiation is used for the treatment of such tumours.