Leiomyosarcoma of the Inferior Vena Cava and its tributaries, a case series and review of the literature

Introduction 

Tumours involving the Inferior Vena Cava (IVC) and its tributaries are rare accounting for only 0.5% of all adult sarcomas. However it is the commonest primary tumour of the IVC (1). Due to its rarity, large series describing such cases are lacking.  And due to its nonspecific nature of the presentation, the diagnosis is often delayed and incorrect diagnoses are made. This report describes a series of three patients who presented with nonspecific abdominal symptoms and diagnosed to have primary leiomyosarcoma of the IVC.

Figure 1 Leiomyosarcoma of the Inferior Vena Cava in the retro hepatic segment (Arrow)

Case 1

A 51 year old female presented with nonspecific epigastric pain. An ultrasound scan of the abdomen revealed a hypo echoic, well defined, lobular mass postero lateral to the suprarenal IVC (Level II) (Figure 1).The lesion was invading the IVC. Further  evaluation with contrast enhanced Computed Tomographic scan (CT scan) was done. The CT scan revealed a well-defined 3.0 cm* 3.5 cm mass arising in relation to the right adrenal gland. The mass was invading the  postero- lateral surface of the IVC. There was no invasion into the adjacent structures.

A surgical exploration and excision of the lump was a planned. During surgery the tumour was found in the suprarenal area posterior to the IVC, it was well defined. It was attached to the posterior wall of the IVC and was invading into it. Tumour was mobilized from the surrounding tissues.  It was excised with the cuff of the IVC. The IVC was a directly repaired with 4/0 polypropylene sutures.

Histology revealed  a leiomyosarcoma (with malignant spindle cells, Mitotic count of 8 per10 High Power Field (HPF).The tumour was positive for  smooth muscle antigen (SMA) and desmin, indicating smooth muscle cell origin but it was negative for S 100 thus excluding a malignant nerve tumour ).The tumour was reaching the resection margin at one area.  However a PET CT Scan done after 2 months did not show any residual tumours. After 308 days of follow up, the patient is well with no clinical and imaging evidence of recurrence.

Case 2

A 52 year old female presented with nonspecific abdominal pain. Imaging revealed a well-defined 6.0 cm* 4.0 cm dumb-bell shaped tumour invading the posterior surface of the Infra renal IVC (level I). Patient underwent tumour resection with a cuff of the IVC. Histology revealed a leiomyosarcoma ( tumour with atypical spindle cells with a mitotic activity of 3 to 4 per 10 HPF. The tumour was positive for SMA and desmin; it was negative for S 100).  Patient was referred to the oncologist for further management. After a 1230 days of follow up, the patient is well with no clinical and imaging evidence of recurrence.

Case 3

A 66 year old female presented with nonspecific abdominal pain.  Imaging revealed left sided retroperitoneal mass near the left kidney. A radical nephrectomy was performed. At surgery it was found that the tumour was arising from the left renal vein. Histology revealed a differentiated (pleomorphic) leiomyosarcoma extending into the renal vein. 3 months later the patient returned with nonspecific abdominal pain. A CT scan of the chest and abdomen was done. It revealed multiple metastases in the lungs and the liver. The patient was referred for palliative oncological management.

Table 1 Summary of the cases

Summary of results

In the above described cases, all were females (100%).  The mean age at presentation was 56.3 years (51 – 66). All presented with nonspecific abdominal pain.  The locations of the tumours were in the suprarenal IVC (n-1), infrarenal IVC (n-1) and the left renal vein (n-1).  The mean tumour size at presentation was 4.75 cm (3.5 – 6.0). All patients underwent surgical resection in our series. All tumour excision was done through a roof top incision with a vertical extension in the upper midline (Benz incision).  Both IVC tumours were removed with a cuff of IVC. A direct repair of the IVC was done.  One patient who had a primary leiomyosarcoma of the left renal vein (Case 3) underwent radical nephrectomy. At a mean follow up of 542.7 days (90 – 1230), one  patient was diagnosed to have metastasis in the lungs and liver 2 months after the surgery and died in 90 days.  The other two patients are well with no clinical or imaging evidence of recurrence.

Discussion and conclusions

Leiomyosarcomas are a type of sarcoma accounting for only 7% of all soft tissue sarcomas (2). Primary leiomyosarcoma of the IVC are even rarer accounting for only 0.5% of all adult sarcomas (3). However it is the commonest primary malignant tumour of the IVC (1).

It originates from the smooth muscle cells of the media of the venous wall (4). As the tumour grows, it grows both into the vessel (intravascular part) and it also extends to the outside of the vein wall (extravascular), this extravascular part of the tumour could be diagnosed in correctly as tumours arising from surrounding tissues e.g.  Adrenal tumour.  The intravascular part of the tumour grows along the vein as tumour thrombus. On microscopy the tumour consist of bundles of spindle cells. The mitotic activity differs depending on the grade of the tumour. On immuno histochemical staining, the leiomyosarcoma is positive for desmin, vimentin and smooth muscle actin but negative for S-100 protein (5).

Leiomyosarcoma of the IVC was first reported by Perl in 1871 (6).  Until now only about 400 cases are reported in the literature (7). It commonly affects females with the female to male ratio of 4:1. It affects individuals in 50 to 60 year age group as the cases reported in this series (3).

Patients commonly present with nonspecific symptoms, sometimes specific symptoms can occur according to the location of the tumour and the completeness of the IVC or occlusion i.e.  Lower limb oedema, hepatic Venous Outflow   Obstruction (HVOO), etc. Due to the nonspecific nature of its presentation, the diagnosis is often missed or delayed (1).

Contrast enhanced computed tomography scan of the abdomen is the imaging of choice. If supra hepatic extension (level III) is detected or suspected, a 2D echocardiogram is performed.

For the purpose of describing, the extent of the leiomyosarcoma of the IVC  was classified into 3 levels lased in the upper extent of the tumour (Figure 2). The level one extends from the beginning of the IVC (the level of the confluence of the common iliac veins) to the lower border of the renal veins. The level II extends from the renal veins level to the lower border of the hepatic veins. The level III is above the level of the hepatic veins (1).

Figure 2 Level of the IVC involvement (according to Kieffer E, et.al.)

Surgical excision is done with curative intent. Options depend on the extent of the tumour, the presence of IVC occlusion, the presence of metastasis and the fitness of the patient.

The challenges of excision of the IVC tumours are the control of the bleeding. For the level I tumours the proximal and distal control of the infrarenal IVC is adequate. For the tumours in the suprarenal and retro hepatic area (level II), control of the Infra renal IVC and renal veins Supra hepatic control of the IVC and control of the hepatic hilar vessels are needed ( to achieve total hepatic vascular exclusion if needed). For the tumours extending above the hepatic venous level (Supra hepatic / Level III), sternotomy and cardio pulmonary bypass is needed.

For the intra luminal tumours which are not an adherent to the wall of the IVC, the tumours can be excised with the cuff of the IVC. And for the tumours which are attached to the wall of the IVC the tumour and the segment of the IVC need to be excised to achieve tumour clearance.

The options for IVC reconstruction include; direct repair of the IVC, repair with a patch, excision of IVC and reconstruction with synthetic graft ( Poly Tetra Fluoro Ethylene – PTFE or Polyester graft). Excision of the IVC and ligation of the ends can be done if the IVC was already occluded for a long time and the collaterals are adequate. However if the IVC is ligated, the distal stump pressure should be assessed.  If the stumm pressure is more than 30mmHg (1) ,  a prosthetic reconstruction of the IVC  is recommended to avoid future development of venous hypertension i.e.  in the lower limbs.

The consensus on adjuvant chemo and radiotherapy is lacking due to the rarity of the IVC leiomyosarcoma (9). In the past adjuvant chemotherapy and the radiotherapy was considered as not effective (10). However recent reports suggest that there are survival benefits for patients with adjuvant chemo or radiotherapy (11).

Overall the leiomyosarcoma of  IVC is associated with poor prognosis with a 5 and 10 year survival of 31.4% and 7.4% (8).

Therefore  leiomyosarcoma of the IVC and its tributaries should be considered as a differential diagnosis,  especially if the tumour  lies near the IVC or it extends into the IVC. Well planned surgical excision  with vascular control provides the best chance of survival.

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