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World J Surg Oncol. 2007 Oct 3;5:109.

Leiomyosarcoma of the adrenal vein: a novel approach to surgical resection.

Author information

1
Department of Surgery, Section of Endocrine Surgery, Yale University School of Medicine, New Haven, Connecticut, USA. trawang@alumni.brown.edu

Abstract

BACKGROUND:

Leiomyosarcomas typically originate within smooth muscle cells. Leiomyosarcomas arising from the adrenal vein are rare malignancies associated with delayed diagnosis and poor prognosis. The most common vascular site of origin is the inferior vena cava.

CASE PRESENTATION:

This is a 64-year old woman who presented with a 13 x 6.5 x 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.

CONCLUSION:

The diagnosis of leiomyosarcomas of the adrenal vein is one of exclusion and involves preoperative radiological imaging and biochemical evaluation to exclude other functional tumors of the adrenal gland. Aggressive surgical resection is associated with improved survival and may be best achieved via collaboration among different surgical subspecialties.

PMID:
17910774
PMCID:
PMC2117013
DOI:
10.1186/1477-7819-5-109
[Indexed for MEDLINE]
Free PMC Article
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