Published online Aug 10, 2017. doi: 10.5306/wjco.v8.i4.366
Peer-review started: December 1, 2016
First decision: February 17, 2017
Revised: June 12, 2017
Accepted: July 21, 2017
Article in press: July 24, 2017
Published online: August 10, 2017
Pulmonary artery intimal sarcoma (PAIS) is a rare tumor with a very poor prognosis. Clinical and radiological findings usually mimic thromboembolic disease, leading to diagnostic delays. The treatment of choice is surgery, and adjuvant chemotherapy and radiotherapy have limited results. We report the case of a 48-year-old male patient, initially suspected with pulmonary thromboembolism. The angio-CT revealed a filling defect in the pulmonary artery trunk. The patient underwent surgery, resulting in with complete resection of the mass with a diagnosis of PAIS. The tumor progressed rapidly in the lung, requiring surgery of multiple lung metastases. The patient was treated with stereotactic body radiation therapy (SBRT) on two occasions for new pulmonary lesions. In the last followup (4 years after initial diagnosis), the patient was disease-free. In conclusion, SBRT proved to be an alternative treatment to metastasectomy, allowing palliative chemotherapy to be delayed or omitted, which may result in improved quality of life.
Core tip: Intimal sarcoma of the pulmonary artery is a rare tumor with a very poor prognosis. It has been described in a limited number of reports. This case is a uncommon patient with long-term survival despite having rapid metastatic progression, who maintains a complete remission after initial surgical treatment, completed after occurrence of progression with stereotactic body radiotherapy.