Rhabdomyosarcoma is a well-known neoplasm in children that frequently occurs in the extremities, the head and neck region, and the genitourinary tract. To the best of our knowledge, pulmonary primary rhabdomyosarcoma in adults is exceedingly rare, and few resected cases have been reported. We report a case of pulmonary primary rhabdomyosarcoma that was surgically resected then treated with adjuvant chemotherapy (vincristine, actinomycin-D and cyclophosphamide). At 9 months after surgery, the patient is free from disease. Although the prognosis of rhabdomyosarcoma is unfavorable, surgical resection and adjuvant therapy could be a potential treatment strategy for pulmonary primary rhabdomyosarcoma.
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