Leiomyosarcoma of the inferior vena cava

Inferior vena cava leiomyosarcoma is a rare malignant mesenchymal tumor that originates from the smooth muscle cells of the venous media and is more frequent in females in the V-VI decade of life. Due to scarce and specific symptoms, diagnosis is not simple, and often metastases to the liver, lungs, and/or lymph nodes are already present. A 44-year-old male patient arrives at our institution presenting diffuse abdominal pain and a sense of weight associated with lumbar pain. He showed nothing relevant except for a moderate consumption of alcohol. Ultrasound examination of the abdomen revealed liver enlargement with hyperechoic nodularity and clear margins. Furthermore, the presence of a voluminous solid nodular formation was found, with an inhomogeneous echostructure and moderate vascularization on Doppler. Inferior vena cava leiomyosarcoma is a rare malignancy. The diagnosis is usually established after surgery, however, recurrences are common and the role of chemoradiation therapy remains to be defined. The only potential treatment is surgical resection with possible vessel reconstruction and en bloc removal of adjacent structures with 5 and 10-year survival rates of 49% and 29%, respectively.