Undifferentiated Intimal Sarcoma of the Inferior Vena Cava with Extension to the Right Atrium and Renal Vasculature

Aasim M Afzal,Jamil Alsahhar,Varsha Podduturi,Jeffrey M Schussler

doi:10.1155/2015/812374

Abstract

Primary sarcomas of the great vessels (aorta, pulmonary artery, and inferior vena cava (IVC)) are exceedingly rare. We report a rare case of an undifferentiated intimal sarcoma of the IVC with extension to the right atrium, adrenal, and renal veins. The patient underwent extensive resection, reconstruction of the IVC, and subsequent adjuvant chemotherapy. Patient has tolerated chemotherapy and, at 17 months after resection, the patient remains free of tumor recurrence. Undifferentiated intimal sarcomas remain a rare entity with only five cases of venous undifferentiated intimal sarcomas reported in the literature, two of which occurred in the IVC. Intimal sarcomas tend to carry a poor prognosis with the limited literature available on treatment approaches. Our objective is to highlight this rare entity and possible treatment approach which we utilized. Primary sarcomas of IVC need to be included as part of a complete differential diagnosis in patients with atrial masses or recurrent pulmonary emboli.

Highlights

Primary sarcomas of the great vessels (aorta, pulmonary artery (PA), and inferior vena cava (IVC)) are seldom seen
We highlight the case of a 52-year-old black female diagnosed with an undifferentiated intimal sarcoma, who underwent extensive resection with reconstruction of IVC followed by chemotherapy over the past year and a half
Sarcomas of the IVC have a female predominance and are typically present in the fifth decade [1]. These tumors are most often derived from the medial smooth muscle and are usually leiomyosarcomas, but intimal sarcomas, leiomyomas, synovial sarcoma, angiosarcoma, and rhabdomyosarcoma have been reported [1,2,3,4]

Summary

Introduction

Primary sarcomas of the great vessels (aorta, pulmonary artery (PA), and inferior vena cava (IVC)) are seldom seen. IVC sarcomas have a female predominance and are typically present in the fifth decade [1]. These sarcomas are typically leiomyosarcoma or angiosarcoma which carry a better prognosis than undifferentiated sarcoma [1]. We highlight the case of a 52-year-old black female diagnosed with an undifferentiated intimal sarcoma, who underwent extensive resection with reconstruction of IVC followed by chemotherapy over the past year and a half

Case Presentation

Discussion

Conflict of Interests