Primary pulmonary angiosarcoma mimicking acute interstitial pneumonia - a diagnostic challenge: A case report

Angiosarcoma is a malignant endothelial cell tumor of vascular or lymphatic origin and accounts for approximately 2% of all soft tissue neoplasms. Primary pulmonary angiosarcoma (PPA) is extremely rare, with <30 cases reported in the literature. Moreover, because of non clinical manifestation and low positive diagnostic rate of traditional, noninvasive or minimally invasive diagnostic methods, the early diagnosis of this neoplasm is really challenging.

A 73-year-old woman initially presented with anemia and hemoptysis, which has occurred over a period of 4 weeks before admission. Subsequently, the patient experienced progressive weakness, anxiety attacks, and loss of appetite.

The bronchoscopy and chest X-ray failed to detect the cause of the symptoms. The patient was initially treated for acute interstitial pneumonia. Subsequent video-assisted thoracic surgery with parietal pleura sampling identified the presence of neoplastic tissue of unknown primary origin.

Transfusion of red blood cell concentrate and high-flow oxygen therapy were administered. In view of poor performance status and unfavorable prognosis, a decision was made not to escalate the therapy. The patient died 21 days after admission. An autopsy was ordered to determine the exact cause of death and revealed multiple nodular lesions scattered in both lung parenchyma and on the pleural surface. The final diagnosis of poorly differentiated epithelioid PPA was made on the basis of histopathological and immunohistochemical postmortem examination.

Presented case cautions that PPA should be considered in patient with hemoptysis, negative bronchoscopy and non-characteristic radiological findings. When the disease is suspected, biopsy and microscopic examination should be implemented to confirm the diagnosis as soon as possible and apply timely treatment. Moreover, due to the small number of PPA cases there is no standard diagnostic and treatment protocol available. Thus, further investigations and sharing experiences, may aid better understanding of this malignancy and lead to improvement in patients' survival.