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A4104 - Primary Pulmonary Angiosarcoma: Rare and Deadly
Author Block: M. Naddour, M. Kalani, A. Fasanya, R. Thirumala; Allegheny Health Network, Pittsburgh, PA, United States.
Introduction: Primary pulmonary angiosarcoma is a rare vascular malignancy that carries a poor prognosis. We present a 66-year-old with primary pulmonary angiosarcoma. Case report: 66-year-old male, never smoker, presents to the hospital with dyspnea and hemoptysis. CT chest showed multiple pulmonary nodules. He was electively intubated, and underwent bronchoscopy, which was suggestive of diffuse alveolar hemorrhage. Patient was started on steroids for vasculitis and underwent embolization of left bronchial artery. Infectious and rheumatologic work-up was negative. He was believed to have ANCA-negative vasculitis. Patient was successfully extubated and started on cyclophosphamide. Repeat imaging showed increased size of right lung nodules, with ground glass halos concerning for hemorrhage. Patient underwent navigational bronchoscopy and cytology was concerning for malignancy. Cyclophosphamide was discontinued. He underwent surgical lung biopsy. Pathology was consistent with epithelioid angiosarcoma and strongly positive for CD31 and vimentin. PET-CT and brain MRI was negative for extrapulmonary involvement. Patient was diagnosed with primary pulmonary angiosarcoma, and chemotherapy was initiated. Discussion: Primary pulmonary angiosarcoma is a rare, aggressive vascular malignancy with a poor prognosis. Only 38 cases are reported in the literature. Patients usually present with hemoptysis, dyspnea and chest pain. Average age at diagnosis is 56 years and is more common in females. CT chest commonly exhibits pulmonary nodules. Biopsy is required for definitive diagnosis. Open-lung biopsy is usually required, as bronchoscopies have lower yield. On Histology, abnormal pleomorphic malignant endothelial cells are indicative of angiosarcoma. It typically expresses factor VII-related antigens, Fli-1, CD34 and CD31. Due to its rarity, standardized treatment is not established. Early diagnosis and surgical resection of localized disease has been reported to be the most effective treatment. The prognosis remains poor, with mortality within few months after clinical diagnosis. Conclusion: Primary pulmonary angiosarcoma is a rare vascular malignancy, and clinicians should consider this entity in the differential diagnosis.