Malignancy arising from the blood vessels is a very rare finding in daily clinical practice. In addition, the finding can often be misdiagnosed and ill-treated. These tumors usually go unnoticed unless it has metastasized to distant tissues. Among these rare tumors, leiomyosarcoma is the most common. It mostly arises in the inferior vena cava (IVC). Clinical signs and symptoms are very vague. Usually it is often misdiagnosed as an abscess cavity in the liver or primary hepatic malignancy (when present at level II) or as a thrombus in the IVC. Radiological investigations are the key to proper diagnosis. Depending upon the exact location, further treatment options vary. Generally, it is believed that level II and level III tumors are amenable to surgery followed by chemo or radiotherapy. We present a rare case of leiomyosarcoma of IVC at level II being diagnosed with proper radiological investigations and its management with further stress on offering chemo-radiotherapy after its surgical removal as compared to only surgery performed earlier. This case report will throw some light on the proper management of such rare tumors in terms of their exact diagnosis and treatment in order to prolong patient survival.
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