Limits...
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.

Show MeSH

Related in: MedlinePlus

Tumor (A) shows a rim of fibrous tissue (B) separating it from adjacent adrenal cortex (C).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC2117013&req=5

Figure 4: Tumor (A) shows a rim of fibrous tissue (B) separating it from adjacent adrenal cortex (C).

Mentions: On pathological examination, the specimen measured 14.2 × 9.5 × 5.5 cm. Cross-sections of the tumor revealed a firm, nodular and trabeculated tan-white mass with focal areas of cystic degeneration and hemorrhage (Figure 3). The tumor was surrounded by a thin rim of fibrous tissue and abutted, but did not penetrate, grossly identifiable adrenal gland (Figure 4A). On microscopic evaluation, the tumor was markedly cellular, composed of spindle cells arranged in intersecting fascicles. Tumor cells showed moderate atypia with hyperchromasia, nuclear enlargement and occasional giant cells. Three to five mitoses per 10 high power field were identified, with occasional atypical mitotic figures (Figure 5). Areas of coagulative necrosis were present. Immunohistochemically, neoplastic cells were negative for c-kit, S100 and HMB45 (Dako®), but showed strong reactivity for desmin and smooth muscle actin (Dako®) (Figure 4C). This finding was consistent with leiomyosarcoma, most likely arising from adrenal vein; however, on multiple sections, no residual vascular wall was detected. Primary adrenal leiomyosarcoma was considered unlikely due to clear-cut separation of tumor from the morphologically unremarkable adrenal gland by a thin rim of fibrous tissue (Figure 4A).


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)

Tumor (A) shows a rim of fibrous tissue (B) separating it from adjacent adrenal cortex (C).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: Tumor (A) shows a rim of fibrous tissue (B) separating it from adjacent adrenal cortex (C).
Mentions: On pathological examination, the specimen measured 14.2 × 9.5 × 5.5 cm. Cross-sections of the tumor revealed a firm, nodular and trabeculated tan-white mass with focal areas of cystic degeneration and hemorrhage (Figure 3). The tumor was surrounded by a thin rim of fibrous tissue and abutted, but did not penetrate, grossly identifiable adrenal gland (Figure 4A). On microscopic evaluation, the tumor was markedly cellular, composed of spindle cells arranged in intersecting fascicles. Tumor cells showed moderate atypia with hyperchromasia, nuclear enlargement and occasional giant cells. Three to five mitoses per 10 high power field were identified, with occasional atypical mitotic figures (Figure 5). Areas of coagulative necrosis were present. Immunohistochemically, neoplastic cells were negative for c-kit, S100 and HMB45 (Dako®), but showed strong reactivity for desmin and smooth muscle actin (Dako®) (Figure 4C). This finding was consistent with leiomyosarcoma, most likely arising from adrenal vein; however, on multiple sections, no residual vascular wall was detected. Primary adrenal leiomyosarcoma was considered unlikely due to clear-cut separation of tumor from the morphologically unremarkable adrenal gland by a thin rim of fibrous tissue (Figure 4A).

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