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


METHODS
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.


CONCLUSIONS
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.
Wang
Tracy S
TS
Department of Surgery, Section of Endocrine Surgery, Yale University School of Medicine, New Haven, Connecticut, USA. trawang@alumni.brown.edu
Ocal
Idris Tolgay
IT
Salem
Ronald R
RR
Elefteriades
John
J
Sosa
Julie A
JA
D000311:Adrenal Glands / Q000098:blood supply*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D007890:Leiomyosarcoma / Q000175:diagnosis / Q000601:surgery*; D018810:Magnetic Resonance Angiography; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology*; D035583:Rare Diseases; D018570:Risk Assessment; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000175:diagnosis / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000379:methods; D014682:Vena Cava, Inferior