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Year : 2002  |  Volume : 12  |  Issue : 4  |  Page : 515-516
Primary leiomyosarcoma of inferior vena cava : an unusual entity


Department of Radiodiagnosis, Dayanand Medical College & Hospital, Ludhiana-141001 (Punjab), India

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   Abstract 

Leiomyosarcoma of primary vascular origin is a rare tumor, which arises mainly from the inferior vena cava (IVC). The middle part of IVC is most often affected. There is a strong predilection for women. Clinical symptoms depend upon the size and location of tumor.

Keywords: Leiomyosarcoma/DI, Abdomen/US: Vena Cava, Inferior, Weight Loss/CO, Hepatic Vein Thrombosis, Magnetic Resonance Imaging, Human, Male, Adult, Case Report,

How to cite this article:
Ahluwalia A, Saggar K, Sandhu P, Gupta K. Primary leiomyosarcoma of inferior vena cava : an unusual entity. Indian J Radiol Imaging 2002;12:515-6

How to cite this URL:
Ahluwalia A, Saggar K, Sandhu P, Gupta K. Primary leiomyosarcoma of inferior vena cava : an unusual entity. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Oct 23];12:515-6. Available from: http://www.ijri.org/text.asp?2002/12/4/515/28528
Subsequently, contrast enhanced computed tomography (CECT) examination showed an intra luminal partially enhancing soft tissue attenuation mass in the IVC. The mass expanded the inferior vena cava up to approximately eight centimeter at the level of the liver. Enhancing tumor vessels were seen within the mass [Figure - 2]. No focal liver lesion was seen and the hepatic veins were well opacified with contrast. The mass was seen to extend from the level of the diaphragm to the junction of the common iliac veins [Figure - 3]. The provisional diagnosis was primary sarcoma of the IVC.

A CT guided FNAC of the mass was performed. Cytological examination revealed interlacing spindle shaped tumor cells with frequent mitosis consistent with leiomyosarcoma.


   Discussion Top


Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor originating from the smooth muscle of the media. Although rare, it is the most common primary malignancy in the IVC [1].

Vascular leiomyosarcomas are located mainly in the IVC, although they sometimes develop in smaller veins also. The tumor shows a striking preponderance for older women with a male to female ratio of 1: 5 [2].

Symptoms and resectability depend on the location and extension of the tumor as well as associated thrombosis. Therefore, it is useful to divide the IVC into three segments i.e. a lower segment below the renal veins, a middle segment from the renal vein up to the hepatic veins and an upper segment from the level of the hepatic veins to the right atrium. Leiomyosarcoma of the IVC most frequently occurs in the middle segment. The tumor may be purely extrinsic or intrinsic or may have both components. The tumor tends to grow slowly expanding along the tissue planes of least resistance. Infiltration of contiguous organs has not been reported. Hematogenous metastasis is common especially to the liver, lung and brain. At a later stage the tumor may spread through lymphatics [2],[3],[4].

In general, patients with leiomyosarcoma of the IVC have non specific complaints such as malaise, weight loss, abdominal and back pain. Infra renal leiomyosarcomas are often dormant for a protracted period and may cause only venous obstruction at a later stage. These slow growing tumors often develop adequate collateral circulation.

If the tumor is located in the middle segment, the renal vein can be involved and if occlusion occurs, the patient can present with nephrotic syndrome.

Tumors in the upper segment give rise to varying degrees of Budd Chiari syndrome due to hepatic vein thrombosis [5,6]. The tumor can also involve more than one segment giving rise to a combination of signs and symptoms.

Until the introduction of USG and CT, the IVC leiomyosarcoma was most commonly diagnosed during laparotomy or autopsy. With the development of imaging modalities like USG, CT and MRI, preoperative diagnosis is possible. USG shows a right sided lobulated retroperitoneal mass, which is hypoechoic and sometimes surrounded by a hyperechoic rim. It reveals the position of the tumor in the IVC, which sometimes cannot be determined by other means. CT scan clearly delineates the intravascular tumor, which is usually large lobulated and sometimes heterogeneous owing to haemorrhage and necrosis. The tumor is usually hypovascular, but may show peripheral enhancement following contrast injection [1,6]. Leiomyosarcoma with extravascular development may be much more difficult to differentiate from retroperitoneal tumors compressing or invading the IVC [7].

The differential diagnosis of an intra-luminal mass in the IVC includes leiomyosarcoma, angiosarcoma, tumor thrombus and bland thrombus [5].

The final diagnosis can be made by an ultra sound or CT guided biopsy. Surgical resectability is highly dependent on the location of the tumor. Complete resection of the tumor is often possible in the lower segment. In the middle segment, a more complicated en bloc resection along with the right kidney is usually needed if the renal vein is involved. If the tumor involves the upper segment, complete resection is usually not possible due to frequent extension into the hepatic veins and the right side of the heart [2],[8].



 
   References Top

1.Cacoub P, Piette JC, Wechsler B, et al . Leiomyosarcoma of inferior vena cava. Medicine 1991; 70: 293-306.  Back to cited text no. 1    
2.Van Rooij WJJ, Martens F, Verbeeten B, Dijkstra J. CT and MR imaging of leiomyosarcoma of the inferior vena cava. J Comput Assit Tomogr 1988; 12(3): 415-419.  Back to cited text no. 2    
3.Young R, Friedman AC, Hartman DS. Computed tomography of leiomyosarcoma of inferior vena cava. Radiology 1982; 145: 99-103.  Back to cited text no. 3  [PUBMED]  
4.Van Zanten TEG, Golding RP. CT and MR demonstration of leiomyosarcoma of inferior vena cava. J Comput Assit Tomogr 1987; 11(4): 670-674.  Back to cited text no. 4    
5.Gay SB, Sistronm CL, Pevarski DJ, Feldman PS. Inferior vena cava mass and Budd Chiari syndrome. Investigative Radiology 1993; 28(8): 774-776.  Back to cited text no. 5    
6.Hemant D, Kranti Kumar R, Amita J, Chawla R, Ranjit N. Primary leiomyosarcoma of IVC, a rare entity: Imaging features. Australasian Radiol 2001; Nov 45(4): 448-451.  Back to cited text no. 6    
7.Skooj SJ, Mcleod DG, Stutzman RE, Bloom DA. Leiomyosarcoma of the inferior vena cava presenting as suprarenal mass. J Urol 1983; 130: 760-772   Back to cited text no. 7    
8.Monig SP, Gawenda M, Erasmi H, Zieren J, Pichlmaier H. Diagnosis treatment and prognosis of leiomyosarcoma of the inferior vena cava. Eur J Surg 1995; 161: 231-235  Back to cited text no. 8  [PUBMED]  

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Correspondence Address:
A Ahluwalia
Department of Radiodiagnosis, Dayanand Medical College & Hospital, Ludhiana-141001 (Punjab)
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

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