|Year : 2016 | Volume
| Issue : 2 | Page : 70-72
Leiomyosarcoma of Inferior Vena Cava: Surgical Excision and Reconstruction of Inferior Vena Cava with Bifurcated Dacron Graft
Chithra Barvadheesh1, P Dhanasekar2, R Sivaprakash2, M Maran3
1 Department of Vascular Surgery, Southern Railway Head Quarters Hospital, Chennai, Tamil Nadu, India
2 Consultant Surgical Oncologist, Bharathi Raja Hospital, Chennai, Tamil Nadu, India
3 Consultant Surgeon, Bharathi Raja Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||8-Jun-2016|
Department of Vascular Surgery, Southern Railway Head Quarters Hospital, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Leiomyosarcomas of the inferior vena cava (IVC) are rare tumors that occur commonly in women of middle age group and usually present with nonspecific symptoms. Treatment consists of surgical excision with or without IVC reconstruction, combined with chemotherapy and radiotherapy. We present a case of leiomyosarcoma of infrarenal IVC extending into both iliac veins, which was resected with reconstruction of IVC and both iliacs with bifurcated Dacron graft. Although IVC reconstruction is mentioned in literature, reconstruction with bifurcated graft is rare.
Keywords: Bifurcated graft, inferior vena cava, leiomyosarcoma, reconstruction of inferior vena cava
|How to cite this article:|
Barvadheesh C, Dhanasekar P, Sivaprakash R, Maran M. Leiomyosarcoma of Inferior Vena Cava: Surgical Excision and Reconstruction of Inferior Vena Cava with Bifurcated Dacron Graft. Indian J Vasc Endovasc Surg 2016;3:70-2
|How to cite this URL:|
Barvadheesh C, Dhanasekar P, Sivaprakash R, Maran M. Leiomyosarcoma of Inferior Vena Cava: Surgical Excision and Reconstruction of Inferior Vena Cava with Bifurcated Dacron Graft. Indian J Vasc Endovasc Surg [serial online] 2016 [cited 2020 Jul 2];3:70-2. Available from: http://www.indjvascsurg.org/text.asp?2016/3/2/70/183647
| Introduction|| |
Vascular leiomyosarcoma is a rare entity, but it is the most common malignant primary tumor of inferior vena cava (IVC). The first report of IVC leiomyosarcoma was done by Peri and Virchow  in 1871. A majority of patients are women in their fifth or sixth decade. The most common clinical presentation is chronic vague right-sided abdominal pain. In the management, pre- or post-operative adjuvant chemotherapy and radiotherapy are considered, but surgery remains the mainstay of the treatment. En masse resection of the tumor with involved IVC is the accepted treatment with or without establishment of continuity for venous return. Various materials are used for restoring continuity such as polytetrafluoroethylene (PTFE) graft and autogenous vein grafts. We present a case of IVC leiomyosarcoma, who was treated with complete resection of the tumor with IVC and both iliac veins and replaced with bifurcated Dacron graft. To our knowledge, in literature, there has been no mention of usage of bifurcated graft for IVC reconstruction.
| Case Report|| |
A 62-year-old female diabetic patient diagnosed to have retroperitoneal mass while being evaluated for mild leg edema in the lower extremities. She did not have any pain in abdomen, bowel or urinary symptoms, except for a positive history for breathing difficulty. Her laboratory investigations revealed nothing abnormal. Computed tomographic (CT) examination revealed a large mass in the retroperitoneum [Figure 1]. She underwent exploratory laparotomy and found to have a huge mass enclosing IVC and compressing right renal vein. Distal extension was up to external iliac vein in the right and common iliac vein in the left. The tumor was resected en bloc [Figure 2]a and [Figure 2]b with IVC and both iliac veins. Venous reconstruction was done with Dacron bifurcated graft (18 × 9 × 9) [Figure 3].
|Figure 3: Operative photography showing replacement of inferior vena cava with bifurcated Dacron graft|
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Microscopic examination of the tumor revealed short, spindled out cells with open hyperchromatic nuclei showing increased mitosis and arranged in closely packed whorls/sheets and reticular pattern. The diagnosis was confirmed with tumor markers (positive for vimentin, desmin, and smooth muscle actin) [Figure 4]. Postoperatively, patient developed right limb of graft thrombosis, which was treated medically and patient recovered with no morbidity. She was advised adjuvant chemo- and radio-therapy. With 1 year follow-up, the patient is disease-free and functioning graft.
| Discussion|| |
Vascular leiomyosarcoma represents just 2% of all leiomyosarcomas with venous involvement more common than arterial sarcoma. Even in primary venous leiomyosarcoma, 60% originate from IVC. After the first documented case in 1871, only 197 cases had been reported well after a century later in 1992 and 218 cases in 1996. With regard to sex (female), mean age group, and site of tumor (lower IVC), our case confirms the findings of Mingoli et al. It often presents very late with symptoms and metastasis, but at present, increase in the usage of radiological investigations results in early diagnosis. Rather than growth by infiltration, leiomyosarcoma tends to grow by intraluminal or extraluminal extension. This may explain the rarity of lower limb edema as a presenting symptom, even in large tumors as in our case. In general, radiological investigations will pick up the mass and its origin can be easily identified, with careful and through examination of the CT films. We missed the obvious diagnosis, but retrospective analysis of CT films showed nonvisualization of IVC which is diagnostic of primary IVC tumors.
The recommended treatment based on the available literature is surgical and we agree with Hollenbeck et al. in wide tumor resection with negative resected margin as the primary treatment. Some authors report that it is not necessary to establish continuity of venous return by IVC reconstruction, because of the presence of collaterals, but others consider preserving venous continuity is mandatory to prevent morbidity. In few cases of complete IVC obstruction preoperatively with development of adequate collaterals, resection of IVC without reconstruction may be considered. During reconstruction of infrarenal segment of IVC, simple clamping of vein is sufficient and does not cause proximal venous hypertension or arterial hypotension. Even if they occur, it can be managed by infrarenal aortic clamping. Various materials are used for the reconstructive material, such as PTFE, autogenous vein, but we used Dacron because of availability and cost factor.
Considering the development of recent advances in adjuvant chemotherapy and radiotherapy, adjuvant therapies may be considered in selected patients with some survival benefit, but surgical resection with microscopically negative margins is still the treatment of choice.
We recommend aggressive surgical team work by oncologist and vascular surgeon for better survival of the patient.
We would like to thank Dr. Meera Govindarajan MD, CEO, R and D Pathology Lab, Chennai, for her contribution in providing HPE photos and HIC support.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Peri L, Virchow R. Ein fall von sarkon der vena cava inferior. Virchows Arch Pathol Anat 1871;53:378-83.
Bower TC, Stanson A. Diagnosis and management of tumours of inferior vena cava. In: Rutherford RB, Editor. Vascular Surgery. Philadelphia: WB Saunders Company; 2000. p. 2077-92.
Dzsinich C, Gloviczki P, van Heerden JA, Nagorney DM, Pairolero PC, Johnson CM, et al.
Primary venous leiomyosarcoma: A rare but lethal disease. J Vasc Surg 1992;15:595-603.
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.
Mingoli A, Feldhaus RJ, Cavallaro A, Stipa S. Leiomyosarcoma of the inferior vena cava: Analysis and search of world literature on 141 patients and report of three new cases. J Vasc Surg 1991;14:688-99.
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.
Zheng W, Song S, Jiang Y, Liang F, Li R. Leiomyosarcoma of inferior vena cava: Report of 7 cases and literature review. Chinese-German J Clin Oncol 2004;3:60-1.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]