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- W2894710428 abstract "SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Sarcomas are malignant tumors arising from mesenchymal cells and originate from bone, cartilage, fat, muscle, hematopoietic or endothelial cells. Angiosarcomas are a rare form of sarcoma that comprises only 2% of sarcomas, arise from endothelial cells and occur anywhere in the body. Angiosarcomas can rarely affect the heart and great vessels when this occurs patients may present with dyspnea, chest pain, cardiac tamponade, heart failure, and pulmonary hypertension. CASE PRESENTATION: A 59-year-old man presented to the emergency department complaining of chest pain, uncontrollable cough, and dyspnea. His past medical history was significant for pulmonary artery angiosarcoma (PAS) diagnosed 6 months prior. At the time of diagnosis, he presented with similar symptoms and initially thought to have a pulmonary embolism upon initial imaging and treatment was started with anticoagulation. Consultation with cardiothoracic surgery, interventional radiology, pulmonary and oncology were obtained. A transcatheter biopsy of the pulmonary artery (PA) showed high-grade sarcoma consistent with angiosarcoma. At that time, he was deemed a non-surgical candidate and treatment was initiated on palliative chemotherapy as an outpatient. The patient received two cycles of therapy. He then presented to the emergency department with tachycardia and hypoxemia. A CT-PE protocol was done and showed complete occlusion of the left main PA and interval increase in size of the tumor with extension into the right main PA resulting in near occlusion of both PAs. The patient was offered a palliative endovascular stent to be placed to the right PA. This was completed without incident and his symptoms largely improved. DISCUSSION: Sarcoma of primary pulmonary origin have been rarely described, however a small proportion have been confirmed as PAS. Prognosis is poor, and the mainstay of treatment is surgical resection, but, many patients are not candidates because of the rapid progression of the disease and advanced stage at the time of diagnosis. PA stent placement is used in patients with congenital heart disease causing PA stenosis, most commonly in pediatric patients. PA stent placement has also been used in strictures secondary to lung transplantation, PE, fibrosing mediastinitis and compression due to malignancy. Few case reports describe endovascular stent placement in patients with non-operative primary PAS. This case illustrates the natural history angiosarcomas present as the endovascular tumor extends through the bilateral PAs and we emphasize the important role endovascular stent placement has on improving quality of life for this rare malignancy. CONCLUSIONS: PAS is a rare malignancy. Diagnosis is commonly achieved late in the disease course rendering surgical removal impossible. With severe occlusion of one or both PAs, stent placement with adjuvant chemotherapy is a reasonable approach as palliative treatment. Reference #1: Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma. Lancet Oncol 2010;11:983-91 Reference #2: Fatima J., Duncan A.A., Maleszewski J., Kalra M., Oderich G., Gloviczki P. Primary angiosarcoma of the aorta, great vessels and the heart. J Vasc Surg. 2013;57(3):756–764. Reference #3: Desmarais P, Laskine M, Caporuscio C. Primary pulmonary artery angiosarcoma mimicking pulmonary embolism in a 66-year old man with dyspnea. CMAJ. 2016 Dec 6;188(17-18) DISCLOSURES: No relevant relationships by Mohamed Abdalla, source=Web Response No relevant relationships by Patricio De Hoyos, source=Web Response Institutional research grant relationship with Boehringer-Ingelheim Please note: $20001 - $100000 Added 11/27/2017 by Anoop Nambiar, source=Web Response, value=Grant/Research Support Speaker/Speaker's Bureau relationship with Boehringer-Ingelheim Please note: $5001 - $20000 Added 11/27/2017 by Anoop Nambiar, source=Web Response, value=Honoraria Institutional research grant relationship with Roche-Genentech Please note: >$100000 Added 11/27/2017 by Anoop Nambiar, source=Web Response, value=Grant/Research Support" @default.
- W2894710428 created "2018-10-12" @default.
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- W2894710428 date "2018-10-01" @default.
- W2894710428 modified "2023-09-25" @default.
- W2894710428 title "IF THE STENT FITS, PLACE IT: A NOVEL APPROACH TO THE TREATMENT OF NON-OPERATIVE PULMONARY ARTERY ANGIOSARCOMA" @default.
- W2894710428 doi "https://doi.org/10.1016/j.chest.2018.08.909" @default.
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