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This is a 64-year old woman who presented with a 13 × 6.5 × 6.6 cm heterogeneous mass arising in the region of the right adrenal gland and extending into the inferior vena cava (IVC) and the right atrium. Biochemical evaluation excluded a functional tumor of the adrenal gland, and multiple tumor markers were negative. We present the novel use of deep hypothermic circulatory arrest (DHCA) in the resection of an adrenal vein leiomyosarcoma extending into the right atrium. The patient remains free of disease ten months after surgery. DHCA afforded a bloodless operative field for optimal resection of disease from within the IVC.
Leiomyosarcoma is a soft-tissue tumor that differentiates from smooth muscle. Primary leiomyosarcomas of vascular origin are relatively rare and frequently arise within the IVC [1-3]. Rarely, leiomyosarcomas may arise from the renal or adrenal vein. We report the case of a leiomyosarcoma of the adrenal vein and a novel approach to surgical resection involving the use of deep hypothermic circulatory arrest (DHCA).
(Figure 1).
Figure 1. Sagittal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.
A leiomyosarcoma of the adrenal vein was first reported in 1981, in a 50-year old patient with a 12 cm leiomyosarcoma arising from the left adrenal vein [4]. Since that time, there have been only eight other reported cases. We report a ninth case, using the novel approach of surgical resection under DHCA
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