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

Wang TS, Ocal IT, Salem RR, Elefteriades J, Sosa JA - World J Surg Oncol (2007)

Bottom Line: Biochemical evaluation excluded a functional tumor of the adrenal gland, and multiple tumor markers were negative.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: 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.

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Sagittal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.
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Figure 1: Sagittal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.

Mentions: A 64-year old woman who presented with a 9-month history of persistent, non-productive cough, bilateral lower extremity edema, and multiple spider angiomata extending from umbilicus to ankles. The patient was otherwise healthy with no significant past medical, surgical, or family history. She denied recent weight loss, fevers, chills, nausea, vomiting, abdominal or back pain, and she had no difficulty with ambulation. Physical exam also was significant for hepatomegaly extending 4 fingerbreadths below the costal margin. There was no jaundice or virilization. Routine laboratory studies were unremarkable. Computed tomography (CT) scan revealed a 13 × 6.5 × 6.6 cm heterogeneous mass arising in the region of the right adrenal gland and extending into the IVC and the right atrium (Figure 1).


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

Wang TS, Ocal IT, Salem RR, Elefteriades J, Sosa JA - World J Surg Oncol (2007)

Sagittal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2117013&req=5

Figure 1: Sagittal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.
Mentions: A 64-year old woman who presented with a 9-month history of persistent, non-productive cough, bilateral lower extremity edema, and multiple spider angiomata extending from umbilicus to ankles. The patient was otherwise healthy with no significant past medical, surgical, or family history. She denied recent weight loss, fevers, chills, nausea, vomiting, abdominal or back pain, and she had no difficulty with ambulation. Physical exam also was significant for hepatomegaly extending 4 fingerbreadths below the costal margin. There was no jaundice or virilization. Routine laboratory studies were unremarkable. Computed tomography (CT) scan revealed a 13 × 6.5 × 6.6 cm heterogeneous mass arising in the region of the right adrenal gland and extending into the IVC and the right atrium (Figure 1).

Bottom Line: Biochemical evaluation excluded a functional tumor of the adrenal gland, and multiple tumor markers were negative.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: 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.

Show MeSH
Related in: MedlinePlus