A 59-year-old male presenting with recurrent hemoptysis was diagnosed with primary pulmonary angiosarcoma via cryobiopsy and passed away 7 months after his initial presentation.
Case Report (n=1)
Primary pulmonary angiosarcoma is a rare, aggressive malignancy that can mimic pulmonary embolism and requires high clinical suspicion and swift biopsy for diagnosis.
Abstract Introduction Pulmonary angiosarcomas are a rare form of malignant vascular tumor usually arising from distant metastases, though a rarer subtype can present as primary lung neoplasms. They carry a poor prognosis and are difficult to diagnose, given clinical similarities to common pulmonary pathology such as pulmonary emboli, infections, and other lung cancers. Here, we detail a case of primary pulmonary angiosarcoma presenting with recurrent hemoptysis. Case Report A 59-year-old male with remote smoking history was recently treated for three episodes of pulmonary emboli. He initially presented with hemoptysis, was found to have pulmonary embolism attributed to his work as a truck driver and was anticoagulated. One month later, he had worsened hemoptysis and clot burden, ultimately undergoing mechanical thrombectomy. Imaging noted new pulmonary nodules, hilar lymphadenopathy, and irregular right pulmonary artery pseudoaneurysm concerning for neoplastic vascular invasion. Two extensive bronchoscopic evaluations with transbronchial needle aspiration, forceps biopsy, and cryobiopsies of suspicious nodules yielded non-diagnostic samples showing only histiocytes and necrosis. AFB and GMS stains were also negative. PET/CT revealed bilateral lung nodules up to 1.9 cm and hilar lymphadenopathy, but no FDG avidity or distant metastases. The patient returned two months later with large-volume hemoptysis and respiratory failure requiring intubation. His CT imaging now showed a right pulmonary mass contiguous with the pulmonary artery and a new left perivascular mass. Bronchoscopy revealed a mass extending from distal trachea to right lower lobe. Debulking was limited by bleeding. Cryobiopsy of the right endobronchial lesion revealed a high-grade neoplasm with atypia, multinucleated giant cells, tumor necrosis, mitotic figures, and associated vasculature. Immunohistochemistry demonstrated c-Myc overexpression suggesting diagnosis of angiosarcoma, though MDM2 FISH was negative. He underwent paclitaxel induction but developed worsening hemoptysis and dyspnea, prompting him to pursue outpatient palliative radiation instead. He passed away at home shortly after, just seven months after his initial presentation. Discussion Primary pulmonary angiosarcomas can elude early detection via non-specific respiratory symptoms, lack of definitive imaging features, and low pre-test probability. Diagnosis requires combined consideration of clinical presentation, radiologic findings, and immunohistochemical pathology. Here, overexpression of c-Myc was suggestive of angiosarcoma, while negative MDM2 FISH suggested against. However, aggressive malignancies such as this often lose signature markers, making it necessary to consider supporting evidence like the tumor’s hypervascularity on bronchoscopic and radiologic evaluation. Given rapid progression, imaging suggesting vascular involvement should prompt swift biopsy. Still, bleeding from vascular tumors can complicate sampling, requiring meticulous pre-procedural planning. This abstract is funded by: None
Fong et al. (Fri,) conducted a case report in Primary pulmonary angiosarcoma (n=1). A 59-year-old male presenting with recurrent hemoptysis was diagnosed with primary pulmonary angiosarcoma via cryobiopsy and passed away 7 months after his initial presentation.