|Year : 2014 | Volume
| Issue : 2 | Page : 121-123
Primary leiomyosarcoma of the inferior vena cava invading the kidney
Ajaykumar R Gajengi1, Anandkumar G Sharma1, Sunil M Mhaske2, Sujata K Patwardhan1
1 Department of Urology, K.E.M. Hospital, Parel, Mumbai, Maharashtra, India
2 Y.C.M. Hospital, Pimpri, Maharashtra, India
|Date of Web Publication||16-Oct-2014|
Ajaykumar R Gajengi
Department of Urology, M.S. Building, 8th Floor, K.E.M. Hospital, Parel, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
Leiomyosarcomas of the inferior vena cava (IVC) with direct invasion of the kidney without involvement of renal vein is rare. A 33-year-old male presented with right flank pain. Contrast enhanced computed tomogram (CT) of the abdomen showed 8.3 cm × 7.5 cm × 5.7 cm vascular mass arising from the suprarenal and hepatic segment of IVC. The IVC was partly occluded by the mass, which was invading right adrenal gland and kidney with normal renal vein. CT guided biopsy revealed low grade leiomyosarcoma. Smooth muscle actin and desmin were positive while S-100, CD34 and C-KIT negative. The patient underwent excision of IVC leiomyosarcoma and right nephrectomy with reconstruction of IVC using saphenous vein and right renal vein graft. Histopathological examination revealed high grade spindle cell sarcoma. Postoperatively, patient received radiation therapy. Patient remained disease free for 2 years after treatment. Surgery remains the primary modality of treatment, whereas the role of chemotherapy and radiotherapy, either as a primary modality or as an adjuvant, remains to be proven. Some recent reports claim promising results, but randomized trials do not exists considering the rarity of this disease.
Keywords: Inferior vena cava leiomyosarcomas, smooth muscle actin and desmin, nephrectomy, inferior vena caval reconstruction
|How to cite this article:|
Gajengi AR, Sharma AG, Mhaske SM, Patwardhan SK. Primary leiomyosarcoma of the inferior vena cava invading the kidney
. Arch Int Surg 2014;4:121-3
| Introduction|| |
Leiomyosarcomas of the inferior vena cava (IVC) are extremely rare, documented in the surgical literature mostly as case reports and only few as others as organized series (PubMed). Involvement of kidney occurs through renal vein through intraluminal spread. Direct invasion without involvement of the renal vein is rare. To the best of our knowledge, there has been no mention in the urologic literature of a leiomyosarcoma of the IVC presenting with direct involvement of kidney without involvement of renal vein. We document this unusual presentation of an extremely rare tumor entity.
| Case Report|| |
A 33-year-old male patient presented with a single episode of stabbing pain in the right flank. Patient was completely asymptomatic for a period of 6 months until he developed a similar episode. He did not have any urinary complaints. General physical and systemic examination was unremarkable. Blood investigations were normal. Ultrasound examination revealed 6.3 cm × 4.6 cm right renal mass arising from the upper pole with increased vascularity and involving IVC. Contrast enhanced computed tomogram (CT) of the abdomen showed 8.3 cm × 7.5 cm × 5.7 cm vascular mass arising from the suprarenal and hepatic segment of IVC, with an intraluminal component causing dilatation and partial occlusion of the IVC and large exophytic component projecting anterolaterally into the subhepatic space [Figure 1]. The IVC was partly occluded by the mass. The upper limit was seen extending 2 cm short of the confluence of hepatic veins. The mass was invading the right adrenal gland and kidney [Figure 2]. Renal vein was normal and no hepatic metastases or significant abdominal adenopathy was detected. Patient underwent CT guided biopsy of the lesion. The histopathological and immunohistochemistry revealed a low grade leiomyosarcoma. Smooth muscle actin and desmin positive, while S-100, CD34 and C-KIT negative differentiating from other smooth muscle tumor. High resolution CT of thorax revealed a suspicious nodule in left lower zone. The positron emission tomography CT showed no evidence of metabolic activity in lung nodule, biopsy was normal. Chevron incision was used and the patient underwent excision of IVC leiomyosarcoma and total right nephrectomy as the tumor was invading into upper pole and hilar region with reconstruction of IVC using saphenous vein and right renal vein graft.
The renal vein which was not involved was opened along its long axis and one side was anastomosed to similar length of opened saphenous vein and turned upwards to cover the defect in the IVC. Postoperative course was uneventful. The patient was discharged on postoperative day 10. Final histopathological examination revealed high grade spindle cell sarcoma. Postoperatively, patient received radiation therapy with a total dose of 50 Gy. The patient tolerated the course of radiation well and reported no side-effects. Subsequent clinical examinations and imaging with CT scans have confirmed that the patient has remained disease free 2 years after treatment [Figure 3]. Patient is under regular follow-up for six monthly Doppler studies of the infrahepatic IVC for evidence of stenosis or any recurrence and also regular ultrasonography of the abdomen and chest radiography for distant metastasis. We plan to do CT angiography with venous phase on a yearly basis.
| Discussion|| |
Leiomyosarcoma of IVC is most frequently seen in the sixth decade with a female predominance.  Clinical findings are nonspecific.  The common presenting symptoms are abdominal pain (52%), distension (20%), and deep venous thrombosis (12%).  Other findings, include Budd-Chiari syndrome, signs of cerebral embolism, and pain in the abdomen and the back. Metastatic disease can involve the liver, lung, lymph nodes, or bone. However, metastases have been reported in fewer than 50% of cases.  Our patient was a young male and except abdominal pain he was asymptomatic. Lung metastasis was suspected but was negative on biopsy. Most common site is middle segment, followed by lower and less commonly upper segment of IVC.  Despite advances in imaging technology, a biopsy is still indicated for diagnosis. Histopathology usually reveals spindle tumor cells, which are positive for markers of smooth muscle activity including vimentin, muscle actin, alfa-smooth muscle actin, and desmin.  In our patient, middle segment was involved and was unable to diagnose on imaging hence biopsy was done, which was suggestive of low grade leimyosarcoma. There is limited experience in the literature regarding surgical management of these IVC tumors. Complete surgical resection with a tumor-free margin (1 cm) is felt to be the treatment of choice.  Analysis of 25 patients showed that complete resection was feasible and that the IVC could be managed either by primary repair or ligation with a low risk of postoperative edema.  Resection of the IVC with reconstruction using graft allows for complete tumor resection and provides durable relief from symptoms of venous obstruction.  In our patient, saphenous vein was used as graft but was insufficient to maintain blood flow hence renal vein, which was not involved was used as patch graft allowing complete resection of tumour.
Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates when complete resection is not possible, debulking combined with radiation therapy still provides good palliation.  In 14 patients with leiomyosarcoma of the IVC treated with wide resection, radiation therapy diminished local recurrence and improved median survival.  Our patient received radiation postoperatively and he is recurrence free since 2 years. Chemotherapy, using adriamycin-ifosfamide or chemotherapy combined with radiation therapy offer potential adjuvant treatment options. 
Unfortunately, the recurrence rate with these tumors is high. Even following a complete primary resection, >50% of patients develop a late recurrence.  In these patients, overall prognosis remains poor with a mean survival of 2 years, but ranging from a few weeks to 8 years. 
| Conclusion|| |
Biopsy is required for initial diagnosis. The role of chemotherapy and radiotherapy in the treatment of IVC leiomyosarcoma, either as a primary modality or as an adjuvant, remains to be proven. Some recent reports claim promising results, but randomized trials do not exists considering the rarity of this disease. Radical excision of tumor whether alone or with adjuvant or neoadjuvant therapy constitutes the only hope of prolonged survival. Normal renal vein can be used to reconstruct the IVC.
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[Figure 1], [Figure 2], [Figure 3]