Pulmonary Artery Intimal Sarcoma Successfully Treated With Surgery and Adjuvant Chemotherapy: A Case Report

1. IntroductionPulmonary artery intimal sarcoma (PAIS) is a rare disease with a probably underestimated incidence at 0.001%–0.03% because it frequently mimics pulmonary vascular diseases, including acute pulmonary embolism 1-3. The literature reported only a few hundred PAIS cases, most of which were published as case reports4, 5. The mean age at diagnosis is 50 years and the range of age is estimated as 13–86 years 5. Several studies indicate the dismal prognosis of PAIS, especially among patients with delayed diagnosis 4-6. Standard therapies remain unestablished, but surgical resection is still the mainstay of treatment5. The effect of adjuvant chemotherapy and radiotherapy on the overall survival rate of patients has no consensus. However, the addition of chemotherapy and radiotherapy postoperatively may improve the outcome of patients with PAIS 6. Here, we report a 53-year-old female patient with PAIS who was successfully treated with surgery followed by weekly paclitaxel therapy.