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LETTER TO EDITOR Table of Contents   
Year : 2003  |  Volume : 13  |  Issue : 4  |  Page : 455-456
Intramural leiomyosarcoma of the inferior venacava


Department of Radiology, Lady Hardinge Medical College & Associated Smt.Sucheta Kriplani Hospital & Kalawati Saran Children's Hospital, New Delhi, India

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How to cite this article:
Madan R, Narula M K, Anand R, Pathania O P. Intramural leiomyosarcoma of the inferior venacava. Indian J Radiol Imaging 2003;13:455-6

How to cite this URL:
Madan R, Narula M K, Anand R, Pathania O P. Intramural leiomyosarcoma of the inferior venacava. Indian J Radiol Imaging [serial online] 2003 [cited 2019 Sep 18];13:455-6. Available from: http://www.ijri.org/text.asp?2003/13/4/455/28732
Sir,

We read with interest the article "Primary Leiomyosarcoma of Inferior Venacava: an unusual entity" in November 2002 issue of IJRI. Primary sarcomas of the great vessels are rare, and are still the subject of case reports. We would like to share our experience of a case of leiomyosarcoma of the inferior venacava and highlight important differences between the two cases.

A 45 year old female presented with right lumbar pain and bilateral pedal edema. Ultrasound revealed a 6.5x5x4 cms lobulated hypoechoic mass inseparable from the posterior wall of the middle segment of the IVC, narrowing the caval lumen and displacing the pancreas and duodenum anteriorly.

CT showed a heterogeneously enhancing soft tissue mass inseparable from the posterior wall of the IVC, involving the infrahepatic segment and the ostia of bilateral renal veins. The renal veins were displaced anteriorly and the right renal artery was seen encased by the mass. There was no evidence of any collateral circulation as the caval lumen was narrowed but not obliterated. The pancreas and 2nd, 3rd parts of duodenum were displaced anteriorly by the mass with loss of fascial planes between them. There was no evidence of hematogenous metastasis to the liver or lymphatic spread within the abdomen.

As the retroperitoneal mass with necrosis was inseparable from the posterior aspect of the IVC, the possibility of a malignant mass arising from the IVC was considered and confirmed on histopathology.

Exploratory laparotomy revealed a lobulated retroperitoneal mass arising from the posterior wall of IVC reducing the lumen to one third but leaving the intima intact. The tumor was also involving the renal vein ostia which however were still patent; the right renal artery was encased by the mass and dense adhesions were present between mass and duodenum Approximately 10 cms of the IVC along with the mass including 2 cms each on either side of the gross tumor and distal segments of both renal veins opening into the IVC were excised. IVC was reconstructed end to end with reimplantation of both renal veins. Patient was kept under follow up and monitored with serial US and color Doppler examination. The patient expired 6 months later due to sudden death at home.

Sarcomas of great vessels are classified according to the location of the sarcoma in relation to the vessel wall, i.e., luminal or mural [1],[2]. Sarcomas of the inferior vena cava are distinct from sarcomas arising in the aorta and pulmonary artery in that they arise chiefly in middle-aged women and are mural leiomyosarcomas[1],[2] ; while aortic and pulmonary artery sarcomas occur in younger patients with no sex predilection and are luminal leiomyosarcomas[1]. Vascularity of leiomyosarcomas differ depending on site of origin - retroperitoneal leiomyosarcomas are hypovascular while those arising from small bowel are hypervascular[3]

The feasibility of surgical resection is determined preoperatively by CT and MRI and is highly dependent on the location of the tumor[4]. Those involving the upper segment present with  Budd-Chiari syndrome More Details , hepatomegaly, jaundice and ascites and have the worst prognosis[1],[2]; those in the middle segment involving the region between the renal and hepatic veins present with right upper quadrant pain mimicking biliary tract disease, and distal lesions have the best long-term outcome and present typically with IVC syndrome[1]. Metastases develop in half of cases to the lung, kidney, pleura, chest wall, liver, and bone.

Imaging features observed in our case were at variance with the published case. As compared to luminal heterogeneous mass described by the authors in their case report , we observed a typical mural mass which was only partially obliterating the caval lumen- there was no evidence of a collateral circulation and the hepatic veins were normal. The renal vein ostia were involved by the mass but no tumor mass or bland thrombus was seen extending into the renal veins; the right renal artery was seen encased within the mass.

Important differential diagnoses to be considered are primary right adrenal tumors with extension along the adrenal veins into the IVC; pancreatic carcinoma and nonHodgkin's lymphoma; exophytic duodenal leiomyosarcomas; angiosarcoma and a bland thrombus within the IVC.

 
   References Top

1.Burke A.P., Virmani R Sarcomas of great vessels Cancer 1993; 71:1761-73Luminal/mural; site  Back to cited text no. 1    
2.Leu J H , Makek M Intramural Venous Leiomyosarcomas Cancer 1986; 57: 1395-1400  Back to cited text no. 2    
3.Nyman U, Hellekant Ch, Jonsson K, Angiography in leiomyosarcoma of the inferior venacava British Journal of Radiology 1979; 52: 273-275  Back to cited text no. 3    
4.Hines 0 J, Nelson S, Quinones-Bald rich W J, Eilber R F Leiomyosarcoma of the Inferior Venacava prognosis and comparison with leiomyosarcoma of other anatomic sites Cancer 1999; 85:1077-1083 pre- staging,  Back to cited text no. 4    

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Correspondence Address:
R Madan
Department of Radiology, Lady Hardinge Medical College & Associated Smt.Sucheta Kriplani Hospital & Kalawati Saran Children's Hospital, New Delhi 110001
India
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Source of Support: None, Conflict of Interest: None


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