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A5318 - Synovial Sarcoma Mimicking Pulmonary Embolism
Author Block: N. Bongu1, B. K. Dunn2, C. Brown1; 1Pulmonary and Critical Care, East Carolina University/Vidant Medical Center, Greenville, NC, United States, 2Pulmonary Critical Care, ECU Vidant Medical, Greenville, NC, United States.
Introduction:
Primary intravascular synovial sarcoma (PISS) is a very rare highly aggressive mesenchymal malignancy that usually originates from deep soft tissues of lower extremities. We report a case of a woman who was admitted to the hospital for pulmonary embolism (PE) which was later found to be synovial sarcoma of lower extremities with extensive pulmonary tumor emboli.
Case Description:
60 year old lady with past medical history of deep vein thrombosis/PE on warfarin presented to outside hospital after a syncopal event . Computed tomographic-pulmonary angiography (CTPA) done revealed near occlusive PE in the left and right pulmonary arteries with right heart strain. She was transferred to our intensive care unit (ICU) for further management . Vitals included blood pressure 110/72 mm Hg , temp 37.6 ,Heart rate 123 beats/minute
Lab data including complete blood count, renal and liver function tests were normal . International normalized ratio (INR) was 4 , tissue plasminogen activator (tPA) was with held given elevated INR .Intravenous heparin drip was started . Two hours later, patient developed bradycardia and pulse less electrical activity (PEA) arrest. Advanced cardiac life support protocol was initiated and tPA was administered.There were multiple episodes of PEA arrest despite intensive resuscitation efforts , cardio vascular surgery was consulted given her ongoing obstructive shock and high vasopressor requirements.
Patient received emergent VA(Veno arterial ) ECMO andembolectomy was performed that demonstrated 24 cm of whitish clot . ICU stay was complicated with acute renal failure requiring CRRT (Continuos renal replacement therapy) , hemoperitoneum from rupture of splenic hematoma and eventual splenectomy . There was significant improvement in neurological status however patient failed to wean from ECMO due to persistent pulmonary hypertension .Biopsy pathology was noted to be consistent with primitive neuroectodermal tumor. She underwent further imaging for identification of primary tumor . A 6 cm soft tissue mass was noted in left groin area. Given aggressive nature of the tumor and poor prognosis family pursued comfort care . Autopsy findings showed extensive malignant tumor emboli within the pulmonary arteries of both lungs . Immuno histochemical studies performed identified the malignant neoplasm as a synovial sarcoma.
Discussion : PISS are rare and only nine cases of them have been reported in literature .There are only five reported cases of them having PE , which was also described in our patient .PE in a patient on therapeutic anticoagulation always think of underlying malignancy .