METASTATIC PULMONARY ANGIOSARCOMA PRESENTING WITH DIFFUSE ALVEOLAR HEMORRHAGE: A RARE COMPLICATION OF A RARE DISEASE

Abstract

SESSION TITLE: Lung Cancer: Expect the Unexpected SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/22/2019 3:45 PM - 4:45 PM INTRODUCTION: Angiosarcoma is an aggressive, malignant tumor of the vascular and lymphatic endothelium. Due to a tendency to cause hemorrhagic necrosis, the prognosis is poor. It represents a small portion of all soft-tissue sarcomas and typically develops in the head, neck or breast, with rare metastasis to the lung and liver. Metastatic pulmonary angiosarcoma is classically seen on CT as multiple solid lesions or thin-walled cysts. Metastasis predisposes the surrounding vasculature to rupture, leading to hemorrhage as a characteristic finding. Diffuse alveolar hemorrhage (DAH) is the consequence of destruction of the alveolar-capillary wall. We present a case of an elderly male presenting with DAH and ultimately diagnosed with metastatic angiosarcoma. CASE PRESENTATION: A 73-year-old male with a history of thyroid cancer and recent admission for blood loss anemia, secondary to diverticulitis and hemorrhoids, presented with progressive shortness of breath and fatigue. Initially, he was treated for new onset heart failure due to clinical exam and typical chest X-ray findings. There was initially a response to diuresis but this was short-lived. He failed to improve and thoracic ultrasound demonstrated bilateral pleural effusions. Thoracentesis was performed and its analysis was significant for exudative effusions with an abundance of red blood cells. He developed hemorrhage from multiple sites, including hematospermia, hematuria and hematochezia, but vascular, hematologic and rheumatologic workup was negative. CT thorax demonstrated bilateral lung/pleural nodules and ground glass opacities consistent with possible pulmonary edema. Sequential bronchoalveolar lavage was performed during bronchoscopy and demonstrated progressively bloodier output consistent with DAH. Given his declining respiratory status, he received a course of steroids and plasma exchange therapy. He had bilateral chest tubes placed due to progressive effusions. Multiple transfusions were administered due to ongoing blood loss. CT-guided biopsy of a dominant pleural nodule revealed the presence of an angiosarcoma. His course was complicated by sigmoid colon perforation at the site of known diverticulosis. He transitioned to comfort care after this terminal event. DISCUSSION: Metastatic angiosarcoma is typically associated with hemorrhagic necrosis of surrounding tissue. This condition rarely affects the lungs. Patients may present with dyspnea/hemoptysis and can rapidly deteriorate into respiratory failure, often requiring mechanical ventilation. DAH is confirmed with serial lavages to document progressively bloodier return. In the presence of DAH/hemoptysis, workup of vasculitis/rheumatologic disorders should be considered. The primary treatment of DAH is steroid therapy. Various immunosuppressive regimens have shown benefit in selected cases. Plasma exchange and IVIg have shown benefits in DAH as well, particularly if the etiology is rheumatologic. There is not currently a standard treatment for pulmonary angiosarcoma as it is a rare disorder. Various approaches that have shown benefit include immunotherapy, radiotherapy and surgical resection. CONCLUSIONS: Metastatic pulmonary angiosarcoma is a rare, aggressive malignancy that caused diffuse alveolar hemorrhage in this patient. Despite receiving steroids and plasma exchange therapy, this patient had rapid decline. Reference #1: Rojas-Solano JR, Ugalde-Gamboa L, Machuzak M. Robotic Bronchoscopy for Diagnosis of Suspected Lung Cancer: A Feasibility Study. J Bronchology Interv Pulmonol. 2018;25(3):168-175. DISCLOSURES: No relevant relationships by Ronak Chhaya, source=Web Response No relevant relationships by Gustavo Cumbo-Nacheli, source=Web Response No relevant relationships by John Egan, source=Web Response