C44-05 The Great Collapse: A Rare and Devastating Presentation of Cystic Lung Disease Secondary to Pulmonary Angiosarcoma Complicated by Recurrent Spontaneous Bilateral Pneumothorax

Abstract Introduction Angiosarcoma is a rare and aggressive soft-tissue malignancy that commonly occurs on the face or scalp of elderly men, constituting less than 1% of all sarcomas with a five-year survival rate of less than 15%. It metastasizes early and often to the lungs as solid nodules, or less frequently, as thin-walled cystic lesions. While cystic pulmonary angiosarcoma is rare, spontaneous pneumothorax is a frequent complication with a high risk of recurrence and increased mortality. Case Description We report the case of a 48-year-old man with a history of progressive cutaneous angiosarcoma of the scalp and face despite chemo- and radiotherapy, who presented to the emergency department with shortness of breath and left rib discomfort. One month prior, imaging revealed five new cavitary lesions in the right lung which were concerning for metastatic disease versus infection, for which he completed an empiric course of fluconazole and antibiotics. CT imaging on admission showed new moderate left pneumothorax and scattered cavitary lesions bilaterally. A left chest tube was placed, and a cavitary lesion biopsy confirmed metastatic pulmonary angiosarcoma. Recovery was complicated by a persistent air leak due to a bronchopleural fistula. Talc pleurodesis was attempted but failed. Endobronchial valve was placed by interventional pulmonology with reduction of the air leak, after which the patient went home. A few weeks later, the patient developed frank hemoptysis. Repeat imaging showed new moderate right pneumothorax and stable left pneumothorax, and he was again hospitalized for placement of a right pigtail chest tube. A blood patch was performed and failed. After two weeks with the chest tube in place, the bronchopleural fistula sealed, and the patient was able to go home. Despite improvement with chest tube placement, the patient was readmitted multiple times with recurrent bilateral pneumothorax. Discussion Angiosarcoma is a rare cause of cystic lung disease, thought to be a result of the “ball-valve” effect from tumor infiltration into the walls of small airways causing air sac distension, excavation of pre-existing solid nodules, or direct proliferation of tumor cells to form blood-filled cysts. The results of this can be devastating. Moreover, pulmonary angiosarcoma-associated cystic lung disease requires a high index of suspicion for diagnosis and is often difficult to treat, with pulmonary complications such as pneumothorax being managed with talc pleurodesis, blood patch, endobronchial valve, and long-term chest tube. Finally, even with aggressive treatment, overall morbidity and mortality remain high. This abstract is funded by: None