Leiomyosarcoma of the inferior vena cava extending into the right atrium and ventricle

Abstract

Case Report: A 38 year old female presented to the ER with worsening symptoms of shortness of breath on exertion and dysphagia. On physical exam she was tachypneic with decreased air entry in all lung fields. On admission she was normotensive, afebrile with no other acute complaints. She had bilateral jugular venous distention with massive hepatomegaly. She had a resected astrocytoma when she was 8 and had residual seizure disorder. CT angiogram revealed a large mass extending from her IVC into her right atrium and ventricle. MRI of the abdomen noted a large heterogeneous mass within right hepatic lobe extending into left hepatic lobe compressing her IVC. TTE confirmed this mass at the junction of the IVC and right atrium prolapsed into the right ventricle and an echogenic, mobile structure on septal leaflet of tricuspid valve. She was subsequently started on a heparin drip for possible thrombus formation. IR performed an ultrasound guided percutaneous biopsy which revealed spindle cell sarcoma, immunohistochemical staining was positive for smooth muscle actin, desmin, CD 34 and Ki-67 up to 35% positive indicative of high grade tumor. Based on radiographic and pathologic specimens she was diagnosed with Stage IV poorly differentiated spindle cell leiomyosarcoma originating from the IVC. After review of images, surgical and oncology teams deemed the tumor unresectable and offered palliative chemotherapy. She recieved IV steroids and supplemental oxygen, but per patients desires she was discharged to hospice care and unfortunately passed away within 2 weeks of discharge. Primary leiomyosarcomas are rare, malignant slow growing-tumors with poor prognosis that have approximately 400 case reportsworldwide. The diagnostic workup for leiomyosarcomas of the IVC consists of thorough history and physical, radiologic imaging including CT, MRI, abdominal ultrasound and echocardiogram. However, the definitive diagnosis is biopsy and helps guide therapeutic or surgical interventions. Differentials of such an entity include thrombus, benign cardiac tumors such as myxomas, or leiomyosarcomas. Based on previous case reports there are no professional guidelines on the management of IVC leiomyosarcomas, but the consensus among surgeons and best prognosis is aggressive surgical removal in combination with chemotherapy and radiation.