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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 650

Leiomyosarcoma of inferior vena cava: Case series of four patients

Department of Radiotherapy, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Rakesh Kapoor
Department of Radiation Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.138029

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 > Abstract 

Primary vascular leiomyosarcoma (LMS) is a rare tumor accounting for 2% of all LMSs, with the most common site being inferior vena cava (IVC). Overall prognosis is poor, with median survival of 2 years only. We present four cases diagnosed with LMS of IVC, treated with surgery, radiation and chemotherapy. The diagnosis was made based on imaging, intra-operative details and histopathology report and after excluding primary LMS arising from other retroperitoneal structures.

Keywords: Inferior vena cava, leiomyosarcoma, management

How to cite this article:
Kapoor R, Bansal A, Sharma SC. Leiomyosarcoma of inferior vena cava: Case series of four patients. J Can Res Ther 2015;11:650

How to cite this URL:
Kapoor R, Bansal A, Sharma SC. Leiomyosarcoma of inferior vena cava: Case series of four patients. J Can Res Ther [serial online] 2015 [cited 2022 Oct 7];11:650. Available from: https://www.cancerjournal.net/text.asp?2015/11/3/650/138029

 > Introduction Top

Leiomyosarcoma (LMS) of inferior vena cava (IVC) are rare tumors accounting for only 0.5% of all soft-tissue sarcomas in adults. Fewer than 400 cases are reported in the literature until date. [1]

 > Case report Top

Four patients were diagnosed with LMS of IVC, from February 2009 to April 2013. There were three females and one male with mean age of 53 years (range = 43-63 years). The demographic details and management are described in [Table 1]. All patients were symptomatic at presentation. Diagnosis was made by contrast enhanced computed tomography (CECT) in two patients and contrast enhanced magnetic resonance imaging (CEMRI) abdomen and Doppler ultrasonography (USG) in another two patients.
Table 1: Clinical parameters, management and outcome of patients

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CEMRI abdomen of case 1 revealed a heterogeneously enhancing centrally necrotic mass (10.2 cm × 7.6 cm × 8.9 cm), hyperintense on T2 and hypointense on T1, in right subhepatic region extending into retroperitoneum [Figure 1]. Post-contrast study showed peripheral enhancement on arterial phase with centripetal filling in venous and delayed phases. The mass was abutting right lobe of liver, gallbladder, main portal vein (displaced anteriorly), pancreatic head, 2 nd and 3 rd part of duodenum (displaced laterally and inferiorly respectively), but no infiltration was seen. Posteriorly, the mass was showing intraluminal extension into infrahepatic IVC.
Figure 1: Magnetic resonance imaging abdomen showing a large retroperitoneal mass arising anterior to inferior vena cava. (a) T2 weighted images, (b) post-contrast images

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CEMRI abdomen of case 2 showed a mass (9.5 cm × 10.0 cm × 10.3 cm) in suprarenal space with infiltration into IVC having cranial intraluminal extension up to intrahepatic IVC and caudal extension up to origin of the right renal vein. Three peripherally enhancing low attenuation lesions were seen in left lobe of the liver suggestive of (s/o) metastasis. In addition, multiple dorsal vertebras also showed heterogeneous enhancement s/o metastasis.

Case 3 underwent CECT abdomen which revealed a mass (16.0 cm × 11.0 cm × 15.4 cm) arising from infrarenal IVC, extending superiorly up to L4 vertebral level. Inferiorly, it lies anterior to urinary bladder. It was seen abutting the right rectus abdominis muscle.

CECT abdomen performed for case 4 showed a heterogeneously enhancing lesion in the lumen of infrahepatic portion of IVC, for a segment of 7 cm. Right proximal ureter was not visualized separately s/o encasement by the tumor and causing hydronephrosis. Similarly enhancing mass (13.0 cm × 12.0 cm × 10.0 cm) was seen in retroperitoneum, infiltrating into lower pole of right kidney and anteriorly extending up to anterior abdominal wall.

Doppler USG of case 1 revealed an intraluminal filling defect in the proximal part of infrahepatic IVC near renal vein junction.

Three patients (case 1, 3 and 4) underwent en bloc excision of tumor, along with IVC reconstruction with dacron graft in two patients and only ligation in third patient (case 3). Case 4 along with the tumor excision, also underwent nephrectomy and removal of third part of duodenum due to adjacent infiltration.

Intra-operative findings of case 1 suggested a 10 cm × 10 cm lobulated tumor arising from juxta-renal IVC, with distal ends of bilateral renal vein draining into IVC, involved grossly [Figure 2]a.
Figure 2: (a) Photomicrograph of resected gross tumor specimen, (b) Photomicrograph of cut section of gross tumor

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Cut section [Figure 2]b showed the tumor arising from anterior wall of juxta-renal IVC, although posterior wall was free from tumor.

In case 3, intra-operatively, 12 cm × 10 cm bosselated tumor was seen in lower abdomen and pelvis adherent to peritoneum and large feeding vessels were seen coming from retroperitoneum.

Intra-operative findings of case 4 showed 14 cm × 10 cm lobulated tumor attached to IVC. The mass was involving lower half of kidney and the serosa of third part of duodenum was infiltrated.

The histopathology report of case 1, 3 and 4 showed spindle shaped cells arranged in fascicles and was suggestive of LMS arising from IVC wall, with immunohistochemistry (IHC) positive for smooth muscle actin (SMA) [Figure 3]. For case 3, right kidney and part of duodenum were found free of tumor.

Biopsy done in case 2 from the mass was also suggestive of LMS.
Figure 3: Photomicrograph showing spindle shaped sarcoma cells (H and E, ×400)

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Post-operative MRI abdomen of case 1 [Figure 4] revealed a well-defined lobulated peripherally enhancing collection (6.4 × 4.5 × 5) around post-operative IVC in right side of retroperitoneum, s/o post-operative collection. MRI of other two patients (case 3, 4) treated with surgery, showed no evidence of any residual disease.
Figure 4: Sagittal reformatted magnetic resonance imaging images showing well defined lobulated peripherally enhancing collection around post-operative inferior vena cava

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Case 2 had metastatic disease in liver and bones at presentation, so surgery was not planned. This patient therefore received neoadjuvant radiotherapy and is on palliative chemotherapy with vincristine, adriamycin, cyclophosphamide (VAC) regimen alternating with ifosphamide, etoposide (IE) at present. Rest of all the three patients received post-operative external radiation with dose of 45-50 Gy delivered in 25 fractions in 5 weeks to the tumor bed by 3 dimensional conformal radiotherapy (3 DCRT), and two out of them (case 1 and 3) completed adjuvant chemotherapy with VAC regimen in October 2013 and are asymptomatic at present. Case 3 who underwent IVC ligation did not develop any limb edema or deep vein thrombosis on follow-up. However, patient who received only post-operative radiation (case 4, patient was not willing for chemotherapy) developed local recurrence and distant metastasis to lung and liver, 6 months after treatment completion.

 > Discussion Top

LMS of IVC is a rare tumor originating from smooth muscle cells of tunica media. [1] The first case was reported by Perl in German literature in 1871. [2] It is seen in 6 th decade, with female preponderance (M:F = 1:4).

Kieffer et al. [3] classified IVC LMS into three groups: Infrarenal (between common iliac veins and renal veins), suprarenal (between renal and hepatic veins) and suprahepatic (between hepatic veins and right atrium).

Symptoms are usually non-specific. Patients with infrarenal, suprarenal and suprahepatic IVC involvement can present with lower extremity edema, budd-chiari syndrome and cardiac arrhythmias respectively.

It is a slow-growing progressive tumor. Systemic metastasis occurs in late stages in <50% cases, mainly involving liver, lung, lymph nodes or bone. [3]

Cross-sectional imaging helps in early diagnosis, origin, extent and evaluating resectability of tumor. [4] Doppler US and MRI can demonstrate tumor vascularity. Cavography can detect a filling defect/or compression and deviation of IVC due to intraluminal or extraluminal growth and also help in performing transluminal biopsy, which can provide accurate pre-operative diagnosis.

These are encapsulated tumors, with spindle-shaped cells, with varying degree of mitotic activity. IHC demonstrates positive staining for desmin, vimentin, HHF35 and SMA. [1]

The optimal treatment has not been established, due to limited experience. The treatment of choice is radical en-bloc surgical excision with a view to obtain negative resection margins. Following resection, ligation of IVC has been reported by some; however preferred approach is reconstruction by primary or patch repair or segmental replacement with tube grafting.

It is evident from the clinical results of surgery alone, {57} that more than 50% patients develop a late recurrence, even after R0 resection. Local recurrences occur in 13-33% of patients, whereas distant metastasis in 30-50% of patients. [6] This indicates a need for both local and systemic adjuvant treatment.

Though sarcomas are radio-resistant, local radiation is indicated to increase local control, in cases with tumor size > 5 cm, mitotic activity > 5 per high power field, poorly differentiated histology, R1/R2 resection and infiltration of adjacent structures. Dose required is 45-50 Gy/25 #/5 weeks @ 1.8-2 Gy per fraction. Target volume to be treated includes tumor bed, regional lymph nodes and adjacent infiltrated structures.

However, there are difficulties in radiotherapy planning of IVC LMS. Sarcomas require doses more than 60 Gy for control. Critical structures in abdomen limit the delivery of such doses. Volume of kidney receiving 30 Gy and 23 Gy should not exceed 20% and 30% respectively. [8] Mean dose of kidney and liver should not exceed 18 Gy and 28 Gy respectively. Dose maximum for spinal cord is 45 Gy. However, conformal techniques like 3 DCRT and intensity modulated radiation therapy planning help overcome these limitations by sparing these critical organs.

Hines et al. [9] treated 14 patients with R0 resection. A total of 12 patients who received post-operative radiation had median survival of 51 months compared with 2 months only in two patients treated with surgery alone. No local recurrences were seen. However, three patients died of distant metastasis.

To control these high rates of distant metastasis (30-50%), chemotherapy is indicated. [6] Regimens most commonly used are VAC, IE, IA, mesna, adriamycin, interleukin-3, dacarbazine and VAC alternating with IE (M = Mesna, D = Dacarbazine). Studies by Kieffer et al. [3] and Ito et al. [10] indicate that with the use of combined modality, the median survival ranged from 45 to 55 months, compared with 5-6 months only with surgery alone.

Follow-up should continue due to slow tumor growth and possibility of early recurrence. Post-operative anticoagulation therapy is recommended by some for 6 months post-operative in order to allow for good re-endothelization of prosthesis.

 > References Top

Alexander A, Rehders A, Raffel A, Poremba C, Knoefel WT, Eisenberger CF. Leiomyosarcoma of the inferior vena cava: Radical surgery and vascular reconstruction. World J Surg Oncol 2009;7:56.  Back to cited text no. 1
Perl L. Leiomyosarcoma of inferior vena cava. Virchows Arch Pathol Anat 1871;53:378-83.  Back to cited text no. 2
Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L. Leiomyosarcoma of the inferior vena cava: Experience in 22 cases. Ann Surg 2006;244:289-95.  Back to cited text no. 3
Ceyhan M, Danaci M, Elmali M, Ozmen Z. Leiomyosarcoma of the inferior vena cava. Diagn Interv Radiol 2007;13:140-3.  Back to cited text no. 4
Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cavallari 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. 5
Hollenbeck ST, Grobmyer 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. 6
Laskin WB, Fanburg-Smith JC, Burke AP, Kraszewska E, Fetsch JF, Miettinen M. Leiomyosarcoma of the inferior vena cava: Clinicopathologic study of 40 cases. Am J Surg Pathol 2010;34:873-81.  Back to cited text no. 7
Marks LB, Yorke ED, Jackson A, Ten Haken RK, Constine LS, Eisbruch A, et al. Use of normal tissue complication probability models in the clinic. Int J Radiat Oncol Biol Phys 2010;76:S10-9.  Back to cited text no. 8
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. 9
Ito H, Hornick JL, Bertagnolli MM, George S, Morgan JA, Baldini EH, et al. Leiomyosarcoma of the inferior vena cava: Survival after aggressive management. Ann Surg Oncol 2007;14:3534-41.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]

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