Case Report: Primary vascular sarcoma presenting as massive pulmonary embolism salvaged by veno-arterial...

Acute massive pulmonary embolism (massive PE) caused by primary vascular-derived sarcoma is extremely rare in clinical practice. Massive PE results in severe shock or cardiac arrest, with high mortality and adverse clinical progression. We report the case of a young woman with a primary vascular-derived sarcoma-related massive PE, diagnosed through aspiration biopsy performed during angiography. The patient was successfully treated using veno-arterial extracorporeal membrane oxygenation (VA-ECMO) combined with interventional thrombectomy, thrombolysis, and anticoagulation.

A 34-year-old woman experienced 2 days of right thigh pain and acute dyspnea, culminating in transient syncope (3 h). On admission, she was hemodynamically unstable, with markedly elevated D-dimer levels. Computed tomography angiography confirmed a massive PE and extensive deep vein thrombosis involving the right iliac and femoral veins. Within the first hour after admission, she experienced cardiorespiratory arrest, requiring immediate initiation of VA-ECMO. Combined therapy (interventional thrombectomy, thrombolysis, and anticoagulation) stabilized cardiopulmonary function, allowing successful weaning from VA-ECMO. Venography revealed a filling defect from the right common iliac vein into the inferior vena cava. Pathological examination of an aspirated thrombus specimen revealed the presence of atypical cells. Immunohistochemistry supported the diagnosis of primary vascular-derived sarcoma (angiosarcoma). Following clinical stabilization, she was discharged on therapeutic rivaroxaban and scheduled for systemic chemotherapy for angiosarcoma.

This case illustrates that VA-ECMO, combined with interventional thrombectomy, thrombolysis, and anticoagulation, is an effective salvage therapy for angiosarcoma-associated massive PE, particularly in patients with hemodynamic instability. It highlights the need to suspect occult malignancy, including vascular sarcomas, in young patients with unprovoked massive PE. Moreover, aspiration biopsy during angiography, when clinically indicated, can serve as a critical diagnostic tool.