SADDLE SARCOMA: AN UNUSUAL IMITATOR OF PULMONARY EMBOLISM

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: We are reporting the case of a 51-year-old male initially thought to have pulmonary thrombosis, who after significant diagnostic studies and therapeutic interventions was found to have intimal sarcoma of his main pulmonary arteries bilaterally. CASE PRESENTATION: 51-year-old male without significant past medical history presents with dyspnea on exertion, 20-pound weight loss, and hemoptysis over the past 6 months. On admission, CT pulmonary angiogram was obtained showing evidence of saddle pulmonary embolism and a pulmonary wedge infarct. He was subsequently placed on a heparin drip for his extensive thrombus. Three days into admission the patient developed new onset chest pain, tachycardia, and hemoptysis. On further evaluation, echocardiogram was obtained showing a large pericardial effusion with tamponade physiology. A pericardiocentesis was performed with 600 cc of dark, sanguineous fluid removed. Cytology later returned without malignant cells. Repeat CTA displayed evidence of significant clot burden and new findings concerning for right heart strain. Interventional radiology was then consulted to perform thrombectomy (EKOS) with minimal thrombus retrieved following procedure. Cardiothoracic surgery was consulted and after reviewing the patient’s findings, were concerned for malignancy. Pathology was sent from the yield of the thrombectomy which confirmed high grade pleomorphic sarcoma occluding both main pulmonary arteries. Palliative care, oncology, and radiation oncology consulted given these new findings. Patient declined palliative care and expressed his wishes to remain full code. He was not a candidate for surgery or radiation given the location and stage (T2N0M0, stage IIIA) of his malignancy at time of diagnosis. He was evaluated by oncology who recently started him on doxorubicin and ifosfamide, although his prognosis at this time remains poor. DISCUSSION: Pleomorphic sarcoma arising from the intimal layer of the vasculature is an extremely rare finding. It most commonly arises from the pulmonary artery or thoracic aorta and has a mean age at presentation of 66. Most patients present with metastasis and 80% of patients will be deceased within a year of diagnosis. Treatment options are limited and are usually palliative measures as most patients are not candidates for radiation or surgery given the location and stage at diagnosis. Systemic chemotherapy can be offered to patients, however it has not been shown to lead to significant increases in mortality. CONCLUSIONS: This is a rare case of pulmonary artery intimal sarcoma in a patient who was previously suspected to have a saddle pulmonary embolus. After undergoing heparin therapy and EKOS procedure without evidence of improvement, pathology was sent leading to his diagnosis. While rare, this diagnosis should be considered if a presumed pulmonary artery clot does not resolve with standard therapy as early detection is crucial. Reference #1: Cantaloube M, Moureau-Zabotto L, Mescam L, et al. Metastatic Intimal Sarcoma of the Pulmonary Artery Sensitive to Carboplatin-Vinorelbine Chemotherapy: Case Report and Literature Review. Case Rep Oncol. 2018;11(1):21-28. Published 2018 Jan 5. https://doi.org/10.1159/000485740 Reference #2: García-Cabezas S, Centeno-Haro M, Espejo-Pérez S, et al. Intimal sarcoma of the pulmonary artery with multiple lung metastases: Long-term survival case. World J Clin Oncol. 2017;8(4):366-370. DISCLOSURES: No relevant relationships by Thomas Brimer, source=Web Response