Pulmonary Artery Intimal Sarcoma Diagnosed as Pulmonary Embolism

Background: Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy of vasculature that carries a very poor prognosis that is often misdiagnosed as a pulmonary embolism due to overlapping clinical and radiological features. It is a very aggressive malignancy with surgery the mainstay of initial management. Chemotherapy is normally given postoperatively, although it is not clear if chemo and radiotherapy bring any improvement, some data report an increase in survival in patients who received multimodality therapy vs single therapy.

Case: A 48 year-old female was initially diagnosed with a pulmonary embolism based on clinical presentation and imaging. She was treated vigorously but continued to show no improvement after two weeks. She underwent an open thrombectomy and a large concurrent pulmonary artery intimal sarcoma (PAIS) was found. The mass was adherent to the intima and extended into the left and right pulmonary arteries. The tumor was not fully resected and tissue results showed poorly differentiated sarcoma. A treatment plan was then initiated to include systemic chemotherapy. She developed metastatic disease despite receiving multimodal therapy and died with within 2 years from the initial diagnosis.

Conclusion: Persistent symptoms in patients diagnosed and treated adequately for pulmonary artery thrombosis should indicate a possible comorbid condition including sarcoma. This is particularly the case in the older age group with imaging that suggests central embolism despite treatment.