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  Table of Contents    
CASE REPORT  
Year : 2012  |  Volume : 55  |  Issue : 1  |  Page : 83-85
Leiomyosarcoma of inferior vena cava: A case series of four cases


1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Surgical Discipline, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication11-Apr-2012
 

   Abstract 

Vascular leiomyosarcomas are rare tumors, arising most frequently from inferior vena cava (IVC). They are mostly seen in sixth decade, with a female predominance. Their diagnosis is often challenging, as patients may present with non-specific complaints such as dyspnea, malaise, weight loss, abdominal pain, or back pain, preceding the diagnosis by several years. Leiomyosarcoma of the IVC most frequently occurs in the middle segment. The final diagnosis can be made by an ultrasound or computed tomography guided biopsy. Because of limited experience with this disease, optimal management of IVC leiomyosarcoma is unknown. Curative surgical resection remains the current treatment of choice for primary leiomyosarcoma of IVC. Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates. Nonetheless, when complete resection is not possible, debulking combined with radiation therapy still provides good palliation. We, hereby, report four cases of this rare entity with emphasis on management.

Keywords: Inferior vena cava, leiomyosarcoma, neoadjuvant therapy, smooth muscle actin

How to cite this article:
Yadav R, Kataria K, Mathur SR, Seenu V. Leiomyosarcoma of inferior vena cava: A case series of four cases. Indian J Pathol Microbiol 2012;55:83-5

How to cite this URL:
Yadav R, Kataria K, Mathur SR, Seenu V. Leiomyosarcoma of inferior vena cava: A case series of four cases. Indian J Pathol Microbiol [serial online] 2012 [cited 2019 Dec 7];55:83-5. Available from: http://www.ijpmonline.org/text.asp?2012/55/1/83/94865



   Introduction Top


Vascular leiomyosarcomas are rare tumors, arising most frequently from the inferior vena cava (IVC). Since 1871, only about 200 cases have been reported worldwide. [1] They are mostly seen in the sixth decade, with a female predominance. [2] Their diagnosis is often challenging, as patients may present with non-specific complaints such as dyspnea, malaise, weight loss, abdominal pain, or back pain, which may precede the diagnosis by several years. [3] Computed tomography (CT), magnetic resonance imaging (MRI), individually or in combination with cavography, ultrasound (US), and echocardiography, allow an early and accurate preoperative diagnosis. [3] Like leiomyosarcomatous lesion of other origin, these patients also have poor prognosis. Curative surgical resection remains the current treatment of choice for primary leiomyosarcoma of the IVC. Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates. [4] Here, we are describing four cases of a leiomyosarcoma of the IVC.


   Case Report Top


Four patients were diagnosed with leiomyosarcomas of the IVC from August 2010 to June 2011. There were two females and two males with a mean age of 58 years (range: 45-65 years). The demographic details and managements are summarized in [Table 1]. All four patients were symptomatic at presentation, although there was significant variation in symptoms between patients [Table 1]. The diagnosis of a caval tumor was made on ultrasound in one of the four cases, and on CT in three cases. CT scans were performed in all cases to further characterize the origin of the tumors, demonstrate any contiguous invasion, and to exclude extraabdominal metastases. One patient was further evaluated by contrast enhanced MRI cavography to differentiate intraluminal mass from thrombus [Figure 1]a. No preoperative biopsy was performed in any patient. All four patients underwent laparotomy and the tumor was excised en-bloc. In the second patient, the point of origin of the tumor on the IVC was narrow, so that primary repair of IVC with vein patch was possible. While in other three cases, the tumor had a broad base. In these cases, the tumor was excised with a cuff of normal IVC wall, the defect was closed with interposition Polytetrafluroethylene (PTFE) graft. Gross examination revealed nodular lobulated fleshy mass arising from the wall of inferior vena cava [Figure 1]b. Microscopy showed a tumor composed of spindle cells arranged in intersecting fascicles, elongated nuclei with vesicular chromatin, inconspicuous nucleoli, and eosinophilic cytoplasm [Figure 2]a and b. The tumor cells revealed moderate nuclear pleomorphism and frequent mitoses [Figure 2]c and d. The tumor cells were immunopositive for smooth muscle actin (SMA) and desmin [Figure 2]e and f and negative for S100. Final impression was leiomyosarcoma.
Table 1: Clinical parameters, management and outcome of patients

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Figure 1: (a) Contrast enhanced MRI venography showing mass lesion in IVC. (b) Cut surface showing large lobulated solid grey white fleshy tumor

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Figure 2: (a) Tumor arising from wall of IVC (H and E, ×20). (b) Spindle shaped tumor cells arranged in intersecting fascicles (H and E, ×100). (c) Tumor cells showing frequent mitoses (H and E, ×400). (d) Tumor cells showing marked nuclear pleomorphism (H and E, ×400), (e) Tumor cells showing immunopositivity for desmin (IHC, ×100). (f) Tumor cells showing immunopositivity for SMA (IHC, ×100)

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The postoperative course was unremarkable for all patients. There were no complications in relation to the venous blood flow in the inferior vena cava and no renal impairment. Two of the patients (patients' two and four) received postoperative radiotherapy. Patient 1 did not come for follow-up, and patient 3 died in early postoperative period, due to chest infection. Patient 4 developed widespread metastasis and died in six months. Patient 2 is alive and disease free till now.


   Discussion Top


Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor originating from the smooth muscle of the media. [1] 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. Hematogenous metastasis is common, especially to the liver, lung, bone, lymph nodes, and brain. At a later stage, the tumor may spread through lymphatics. [5] Infra renal leiomyosarcomas are often dormant for a protracted period and may cause only venous obstruction at a later stage. 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. [2],[6] 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. CT scan clearly delineates the intravascular tumor, which is usually large lobulated and sometimes heterogeneous owing to hemorrhage and necrosis. The tumor is usually hypovascular, but may show peripheral enhancement following contrast injection. [2] Leiomyosarcoma with extravascular development may be much more difficult to differentiate from retroperitoneal tumors compressing or invading the IVC. [7] The final diagnosis can be made by an ultra sound or CT guided biopsy. Leiomyosarcoma of the IVC does not respond well to chemotherapy or radiotherapy.

Because of limited experience with this disease, optimal management of IVC leiomyosarcoma is unknown. Aggressive surgical treatment is the treatment of choice as these tumors are slow growing and have low metastatic potential. [8],[9] Complete surgical resection with a tumor-free margin of 1 cm is the treatment of choice. [2] 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. [10] Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates. Nonetheless, when complete resection is not possible, debulking combined with radiation therapy still provides good palliation. [2]

Hence, an accurate imaging and histopathological diagnosis is essential for improving the patient survival.

 
   References Top

1.Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cava N. International registry of inferior vena cava leiomyosarcoma: Analysis of a world series on 218 patients. Anticancer Res 1996;16:3201-5.  Back to cited text no. 1
    
2.Hemant D, Krantikumar R, Amita J, Chawla A, Ranjeet N. Primary leiomyosarcoma of inferior vena cava, a rare entity: Imaging features. Australas Radiol 2001;45:448-51.  Back to cited text no. 2
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3.Gowda RM, Gowda MR, Mehta NJ, Osborne R, Bixon R, Vasavada BC, et al. Right atrial extension of primary venous leiomyosarcoma: Pulmonary embolism and Budd-Chiari syndrome at presentation-a case report. Angiology 2004;55:213-6.  Back to cited text no. 3
    
4.Hines OJ, Nelson S, Quinones-Baldrich WJ, Eilber FR. Leiomyosarcoma of the inferior vena cava: Prognosis and comparison with leiomyosarcoma of other anatomic sites. Cancer 1999;85:1077-83.  Back to cited text no. 4
[PUBMED]    
5.Van Zanten TE, Golding RP. CT and MR demonstration of leiomyosarcoma of inferior vena cava. J Comput Assit Tomogr 1987;11:670-4.  Back to cited text no. 5
    
6.Gay SB, Sistronm CL, Pevarski DJ, Feldman PS. Inferior Vena cava mass and Budd Chiari syndrome. Investig Radiol 1993;28:774-6.  Back to cited text no. 6
    
7.Ahluwalia A, Saggar K, Sandhu P, Gupta K. Primary leiomyosarcoma of inferior vena cava: An unusual entity. Vascular 2002;12:515-6.  Back to cited text no. 7
    
8.Griffin AS, Sterchi JM. Primary leiomyosarcoma of the inferior vena cava: A case report and review of the literature. J Surg Oncol 1987;34:53-60.  Back to cited text no. 8
[PUBMED]    
9.Hollenbeck ST, Grobmeyer SR, Kent KC, Brennan MF. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma. J Am Coll Surg 2003;197:575-9.  Back to cited text no. 9
    
10.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;161:231-5.  Back to cited text no. 10
    

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Correspondence Address:
Rajni Yadav
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.94865

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