Leiomyosarcoma of the Infrarenal Inferior Vena Cava: Management in Three Cases and a Review of the Literature
Article Outline
Leiomyosarcoma (LMS) of the inferior vena cava (IVC) represents 90% of inferior vena cava tumours. It has a poor prognosis. We present three LMS of infrarenal IVC. Survival was long in two patients in spite of a non-radical resection. Chemotherapy and repeat surgery was performed for recurrence. The third patient was in complete remission at last follow-up at 29 months. Mainstay treatment for this tumour is surgical resection. Rescue surgery for local and metastastic recurrences together with systemic chemotherapy and radiotherapy may improve survival.
Keywords: Leiomyosarcoma, Inferior vena cava, Radical surgery, Rescue surgery
Background
Only 0.5% of leiomyosarcomas (LMS) are of vascular origin and although rare, such lesions represent 90% of inferior vena cava (IVC) tumours. Fewer than 250 LMS of the IVC have been reported in the literature.1, 2 The clinical features are non-specific, leading to a delay in diagnosis. Radical surgery is the elective treatment for local control. However, the recurrences are frequent and the prognosis remains poor. We report three cases of IVC LMS.
Report
From 1995 to 2005, 56 patients with retroperitoneal sarcomas were treated at our hospital, a referral centre for these tumours. Three (5.3%) of these sarcomas were located in the infrarrenal IVC.
Clinical features and treatments are reported in Table 1. An infrarenal tumour of IVC was observed in all patients by echography, CT scan and MR angiography. Mean diameter was 9 cm. The preoperative histological study showed a high grade LMS (biopsy during a hysterectomy in patient 1 (P1) and FNAC in patient 2 (P2) and patient 3 (P3).
Table 1. The three patients
| Sex | Age years | Clinical symptoms | CT-scan | Treatment | Margin | Adjuvant therapy | Recurrences | Treatment | Survival | |
|---|---|---|---|---|---|---|---|---|---|---|
| P1 | F | 52 | Dull hypogastric pain (1 yr duration) | Mass below celiac trunk | Surgery +suture v.cava | + | Preop CT Postop RT+CT | *24 mths: Local+nodal mediastinic metastasis | CT | 32 mths |
| P2 | F | 33 | Mesogastric pain+alteration in intestinal motility (1 mth duration) | No invasion of neighbouring structures | Surgery+suture v.cava | <1cm | Postop RT+CT | *24 mths: hepatic metastasis | *Repeated | 108 mths |
| *36 mths: hepatic progression metastasis | Surgery of recurrences | |||||||||
| *60 mths: lung, bone and soft tissue dissemination | *CT | |||||||||
| *84 mths: breast (metastasis) | *RT | |||||||||
| *100 mths: disease progression | ||||||||||
| P3 | F | 61 | Right iliac fossa pain (2 yr duration) | No invasion of neighbouring structures | Surgery+resection of IVC and ligation of left renal vein | + | POSTOP RT | No recurrence | – | +29 mths free of recurrence |
All patients were treated by surgery. P1 received chemotherapy (CT) prior to surgery. In all patients we performed marginal excision surgery. (Fig. 1) Partial resection of the IVC was performed in P1 and P2 and excision of the infrarenal IVC without reconstruction was performed in P3. Resection margins were positive in two cases and less than 1cm in the other case. Postoperatively, all patients received radiotherapy (RT). Two patients (P1, P2) received postoperative CT (Doxorubicin and Ifosfamide).
Fig. 1
Multilobulated tumour found in P3.
Surgical complications were encountered in one patient: acute renal failure due to thrombosis of the right renal artery. This patient also presented oedema of the lower limbs due to the resection of vena cava; which resolved with compressive therapy.
Recurrences appeared at 24 months in two patients (P1, P2). All recurrences were treated with chemotherapy. P2 presented a solitary hepatic metastasis at 24 months that was treated with CHT followed by rescue surgery. At 12 months' follow-up, three hepatic metastasis were also treated with CHT and surgery. Sixty months after the initial surgery this patient presented a lung, bone and soft tissue dissemination and she died.
Death in the two patients was due to metastatic spread (Table 1). Although we were unable to perform radical surgery, P1 survived 32 months and P2 108 months. P3 is presently in complete remission 29 months since diagnosis.
Discussion
According to the literature,3 surgery is the only treatment for LMS that significantly affects survival. Radical resection with disease-free margin of at least 1cm is the recommended surgery but it is not always possible due to a delayed diagnosis. However, the developments in diagnostic techniques (CT-scan, MR) and their increased application enable earlier diagnosis and improve overall patient survival. RT and CHT can be used as adjuvant therapies although no survival increased has been demonstrated.
Hollenbeck et al.3 compared survival in patients in two groups: patients who underwent radical resection and those who did not. Higher survival in the radical surgery group was statistically significant (p<0.05). Hines et al.4 presented 0% survival at 5 years in patients with positive margins. However, surgery of hepatic metastases has increased survival and it is possible to resect the mass and the metastases at the same time. Furthermore, debulking of intraabdominal recurrence offers good palliation.5
Although we could not perform radical surgery, survival was long in our three patients. One patient survived for 108 months. This patient underwent repetitive surgery of local recurrence and metastases and the result was satisfactory. We consider that the relatively high survival in our patients was due to the aggressive surgical treatment of the recurrence.
Another controversial point is the reconstruction of the IVC. LMS usually grow slowly, so if there is involvement of the IVC with impairment of flow, collaterals will develop and when present, a resection can be performed safely. If the IVC is fully patent, it may be difficult to resect IVE without reconstruction. Before choosing a surgical technique, it is important to evaluate the previous patency of IVC, determine the segment to be resected, and assess the patient's general status.6 We only encountered oedema in one patient and this was resolved with compressive therapy. Hollenbeck et al.1, 3 compared the various grades of oedemas using several techniques and concluded that simple ligature, primary reconstruction and autograft patch caused less oedema than prosthetic grafts. We consider that simple ligature or primary reconstruction is the first option with few complications and limited oedema due to marked collateral circulation.
In conclusion, with the increasing availability of non-invasive diagnostic techniques, LMS will be diagnosed earlier and more precisely, leading to a higher rate of radical surgery. Together with more aggressive treatment of recurrences and metastases, overall patient survival could improve. We consider that a multidisciplinary team composed of a medical oncologist, a radiotherapy oncologist, a general surgeon and a vascular surgeon can provide optimal treatment for this pathology.
References
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- . International registry of inferior vein cava leiomyosarcoma: analysis of a world series on 218 patients. Anticancer Res. 1996;16:3201–3206
- . Surgical treatment and outcomes of patients with primary inferior vein cava leiomyosarcoma. J Am Coll Surg. 2003;197:575–579
- . Leiomyosarcoma of the inferior vein cava. Prognosis and comparison with Leiomyosarcoma of other anatomic sites. Cancer. 1999;85(5):1077–1083
- . Palliative surgery for leiomyosarcoma of the inferior vein cava. Ann Surg. 1973;177:237–239
- . Leiomyosarcoma of the retrohepatic portion of inferior vein cava: clinical presentation and surgical management in five patients. J Am Coll Surg. 2005;200:57–63
PII: S1533-3167(07)00020-9
doi:10.1016/j.ejvsextra.2007.04.002
© 2007 European Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.
Refers to article:
- Leiomyosarcoma of the Infrarenal Inferior Vena Cava: Management in Three Cases and a Review of the Literature , 09 July 2007
