WJCS  Vol.4 No.4 , April 2014
A Rare Retroperitoneal Mass: Leiomyosarcoma of the Inferior Vena Cava
ABSTRACT
Leiomyosarcoma of the inferior vena cava (IVC) is a rare soft tissue tumor, mesenchymal in origin that arises from smooth muscles of tunica media; it accounts for about 0.5% of all soft tissue sarcomas, and it is the commonest vascular leiomyosarcoma. The tumor progression is slow, and it is asymptomatic until advanced stage in which involvement of surrounding structures even when the symptoms present are nonspecific. Presentation of Case: A 60 years old lady presented with upper abdominal pain for 3 months duration. Past surgical history was significant for Hysterectomy 15 years ago. On examination: soft abdomen, palpable non pulsating right hypochondrial mass. Ultrasound of the abdomen showed tumor of the head of pancreas. CT scan showed large retroperitoneal tumor extending from the head of pancreas to IVC. Trans abdominal CT guided FNAC showed retroperitoneal sarcoma while Immunohistochemistry (IHC) was proved to be Leiomyosarcoma of the IVC. Discussion: Leiomyosarcoma of inferior vena cava (IVC) is a rare soft tissue tumor, mesenchymal in origin that arises from smooth muscles of tunica media; it accounts for about 0.5% of all soft tissue sarcomas, and it is the commonest vascular leiomyosarcoma. The type of surgical management is a matter of debate and includes resection alone, primary repair/cavoplasty, or replacement with a graft. Reconstruction of the IVC is not always required especially in chronic occlusions. Conclusion: Despite all the advanced modalities, surgery remains the most effective method for treatment of Leiomyosarcoma.

Cite this paper
Baram, A. , Tayeb, Z. and Bakir, D. (2014) A Rare Retroperitoneal Mass: Leiomyosarcoma of the Inferior Vena Cava. World Journal of Cardiovascular Surgery, 4, 51-55. doi: 10.4236/wjcs.2014.44009.
References
[1]   Alexander, A., Rehders, A., Raffel, A., Poremba, C., Knoefel, W.T. and Eisenberger, C.F. (2009) Leiomyosarcoma of the Inferior Vena Cava: Radical Surgery and Vascular Reconstruction. World Journal of Surgical Oncology, 7, 56. http://dx.doi.org/10.1186/1477-7819-7-56

[2]   Ahluwalia, A., Saggar, K., Sandhu, P. and Gupta, K. (2002) Primary Leiomyosarcoma of Inferior Venacava: An Unusual Entity. Indian Journal of Radiology and Imaging, 12, 515-516.

[3]   Hartman, D.S., Hayes, W.S., Choyke, P.L. and Tibbets, G.P. (1992) From the Archives of the AFIP. Leiomyosarcoma of the Retroperitoneumand Inferior Vena Cava: Radiologic-Pathologic Correlation. Radio Graphics, 12, 1203-1220. http://dx.doi.org/10.1148/radiographics.12.6.1439022

[4]   Kieffer, E., Alaoui, M., Piette, J.C., Cacoub, P. and Chiche, L. (2006) Leiomyosarcoma of the Inferior Vena Cava: Experience in 22 Cases. Annals of Surgery, 244, 289-295.
http://dx.doi.org/10.1097/01.sla.0000229964.71743.db

[5]   Huguet, C., Ferri, M. and Gavelli, A. (1995) Resection of the Suprarenal Inferior Vena Cava. The Role of Prosthetic Replacement. Archives of Surgery, 130, 793-797.
http://dx.doi.org/10.1001/archsurg.1995.01430070115025

[6]   Ito, H., Hornick, J.L., Bertagnolli, M.M., George, S., Morgan, J.A., Baldini, E.H., Wagner, A.J., et al. (2007) Leiomyosarcoma of the Inferior Vena Cava: Survival after Aggressive Management. Annals of Surgical Oncology, 14, 3534-3541. http://dx.doi.org/10.1245/s10434-007-9552-z

[7]   Bailey, R.V., Stribling, J., Weitzner, S. and Hardy, J.D. (1976) Leiomyosarcoma of the Inferior Vena Cava: Report of a Case and Review of Literature. Annals of Surgery, 184, 169-173.
http://dx.doi.org/10.1097/00000658-197608000-00006

[8]   Singh-Panghaal, S., Karcnik, T.J., Wachsberg, R.H. and Baker, S.R. (1997) Inferior Vena Cava Leiomyosarcoma: Diagnosis and Biopsy with Color Doppler Sonography. Journal of Clinical Ultrasound, 25, 275-278. http://dx.doi.org/10.1002/(SICI)1097-0096(199706)25:5<275::AID-JCU10>3.0.CO;2-I

[9]   Gowda, R.M., Gowda, M.R., Mehta, N.J., Osborne, R., Bixon, R., Vasavada, B.C. and Sacchi, T.J. (2004) Right Atrial Extension of Primary Venous Leiomyosarcoma: Pulmonary Embolism and Budd-Chiari Syndrome at Presentation—A Case Report. Angiology, 55, 213-216.
http://dx.doi.org/10.1177/000331970405500215

[10]   Ceyhan, M., Danaci, M., Elmali, M. and Ozmen, Z. (2007) Leiomyosarcoma of the Inferior Vena Cava. Diagn Interv Radiol, 13, 140-143.

[11]   Reddy, V.P., et al. (2010) Leiomyosarcoma of the Inferior Vena Cava: A Case Report and Review of the Literature. Cases Journal, 3, 71.

 
 
Top