Intimal sarcoma is a rare type of mesenchymal tumor that arises from the intima of large and small vessels. Most commonly it arises from the pulmonary artey or the thoracic aorta. We report a case diagnosed with right pulmonary artery intimal sarcoma after CT guided biopsy. The patient underwent surgical excision of the tumor successfully without residual and no post-operative complications, then received adjuvant chemotherapy. Patient was disease free till her last follow up in 12/2020. Conclusion: As there is no standard of care for the intimal sarcoma, it was treated by extrapolating data from sarcoma guidelines. Complete surgical excision will remain the best prognostic factor for the survival.
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