Leiomyosarcoma of the adrenal vein: a novel approach to surgical resection. World J Surg Oncol 2007 Oct 03;5:109
Date
10/04/2007Pubmed ID
17910774Pubmed Central ID
PMC2117013DOI
10.1186/1477-7819-5-109Scopus ID
2-s2.0-36949002041 (requires institutional sign-in at Scopus site) 17 CitationsAbstract
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.
Author List
Wang TS, Ocal IT, Salem RR, Elefteriades J, Sosa JAAuthor
Tracy S. Wang MD, MPH Professor in the Surgery department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Adrenal GlandsFemale
Follow-Up Studies
Humans
Immunohistochemistry
Leiomyosarcoma
Magnetic Resonance Angiography
Middle Aged
Neoplasm Invasiveness
Rare Diseases
Risk Assessment
Tomography, X-Ray Computed
Treatment Outcome
Vascular Neoplasms
Vascular Surgical Procedures
Vena Cava, Inferior