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Clear cell sarcoma (CCS) is a rare tumour that manifests in tendons and aponeuroses of the distal extremities and comprises roughly 1% of all soft tissue cancers. Only two cases of a primary CCS arising in the lung have been reported in the literature, and neither were located in the lung apex. We report a case of a primary CCS located in the lung apex that originally presented as ptosis, as well as arm pain and weakness. Distant metastasis was discovered on diagnosis, which led to irreversible paraplegia and death of the patient.

Primary clear cell sarcoma in the lung apex with spinal metastasis: a fatal outcome