Sugi Subramaniam RV¹, Elankumaran Krishnan¹, Kulaseharan VH², Jithin Jagan³, Sekar Natarajan³, Shantha Ravisankar⁴

^{1 Liver Disease and Transplantation Centre, Kauvery Hospital, Chennai – 600004
2 Department of Liver Anesthesia& Intensive Care, Kauvery Hospital, Chennai – 600004
3 Department of Vascular & Endovascular Surgery, Kauvery Hospital, Chennai – 600004
4 Department of Pathology, Kauvery Hospital, Chennai – 600004}

ABSTRACT

Retroperitoneal sarcomas are the common form of sarcomas in humans, yet vascular sarcomas arising from vessel walls are extremely rare. These vascular leiomyosarcomas [LMS] most commonly arise from IVC. IVC LMS constitutes only about 0.5 % of the soft tissue sarcoma in adults. Overall, IVC LMS is a very rare neoplasm contributing to less than 1 in 100,000 adult malignancies. The goals of management include achieving R0 resection, restoring the patency of IVC, and reducing local recurrence rate by addition of the most effective adjuvant therapies. Complete surgical resection offers the greatest 5-year survival rate that ranges between 30 and 70%. We report a case of IVC LMS in a 56-year-old female and its management, to highlight the importance of this rare yet aggressive malignancy and review the literature.

Case Summary

A 56-year-old female presented with complaints of indigestion and abdominal pain for 2 weeks. There is no history of abdominal distension, vomiting, altered bowel, or bladder habits. Ultrasound abdomen was performed, which was suggestive of a solid mass with calcification in retroperitoneum. Contrast-enhanced computed tomography of the abdomen presented an ill-defined enhancing hypodense lesion with calcification involving retroperitoneal region extending to caudate and inferior vena cava with features of leiomyosarcoma [Figure 1A-B]. The patient underwent exploratory laparotomy. On mobilization of the liver, mass was found to arise from retro hepatic portion of suprarenal inferior vena cava. The mass was about 10 * 8 cms, and exophytic growth arising from the IVC was about 2 cms above the right renal vein. The tumor was seen infiltrating the caudate lobe. After cross clamping the IVC, the segment of supra renal IVC containing the tumor was excised in toto. Left renal vein was dissected and ligated distal to the left gonadal vein junction. IVC was reconstructed using 22 Fr Gelatin-impregnated knitted polyester straight vascular prosthesis using 5-0 Prolene sutures [Figure 2A -D]. The patient tolerated the procedure well. The Patient was discharged after 1 week of hospital stay and was put on oral dabigatran at the time of discharge. Histopathological examination showed features of a pleomorphic/ dedifferentiated leiomyosarcoma [Figure 3A-B].

Discussion

IVC leiomyosarcoma [LMS] is a very rare neoplasm affecting less than 1 in 100,000 adult malignancies.[1] Most of the patients present with intra or extra-luminal growth, often with invasion of adjacent structures at the time of presentation.[2] IVC LMS is predominant in females mostly in their 6^th decade at the time of diagnosis. Currently the best results in IVC LMS are through complete surgical removal.[3] On rare instances, it occurs even in young individuals as localized diseases. IVC resection followed by reconstruction should be the ideal treatment if long-term survival can be anticipated after complete macroscopic resection.[4]

IVC LMS is classified into 3 groups [Table 1] based on the segment involved, and their presentation varies accordingly.[5] Vascular LMS represents only 2 out of 100 LMSs and 0.5 % of all the soft tissue sarcomas.[6] IVC LMS was first described by Perl in the year 1871.[7] Almost 6 decades later, in the year 1928, Mechoir described the first surgical resection of an IVC LMS.[8] IVC LMS can remain indolent for a prolonged period because of its slow rate of growth. The prognosis is invariably poor because of the advanced stage at presentation. More than one-third of the IVC LMSs occur in the infrarenal portion of IVC.[9]

Table 1. Classification of IVC LMSs

Most of the IVC LMS are diagnosed incidentally or at autopsy. Contrast-enhanced computed tomography or magnetic resonance imaging of the abdomen is done to define the extent of tumor involvement. Middle segment IVC LMSs has a better prognosis compared to segment III. R0 resection has been the only proven treatment option with improved survival. Surgical options include total excision of the tumor and involved portion of the IVC followed by either primary IVC closure, patch angioplasty with autologous vein or prosthetic patch or replacement of the entire involved segment with interposition polytetrafluoroethylene (PTFE) or Dacron or preserved cadaveric venous homograft.[10] Simple clamping is usually sufficient for infrahepatic IVC reconstruction. Cardiopulmonary bypass is rarely necessary in the IVC LMS, except in few tumors with intracardiac extension. The choice of selection of the IVC graft is usually the surgeon’s preference. The ring PTFE reinforcement of IVC resists respiration compression better, thereby preventing graft collapse and graft thrombosis later and hence the most preferred graft for reconstruction. The rationale behind the selection of a smaller size PTFE or Dacron graft (undersize) is that the subsequent increase in blood flow velocity within the graft might prevent thrombus formation.[11] The rationale behind the use of larger diameter graft is that PTFE tends to form a thick pseudo intima which would later result in obstruction or luminal narrowing in an otherwise normal size graft.[12] Recurrence rates following complete resection are as high as 75 % with a documented 50 % 5-year survival rate.[13] Adjuvant chemotherapy or radiation therapy has been tried but with no proven evidence that either of them improves survival. Five-year and ten-year survival following R0 resection are about 50% and 30% respectively. Mortality is higher in high-grade tumors, although grade of the tumor was not predictive of survival nor recurrence rate.

We used an oversized ring 22 Fr Gelatin-impregnated polyester-knitted vascular prosthesis in our patient with simple clamping of the retro hepatic IVC above and below the resected segment. The hepatic veins were dissected and looped for better vascular control as a prophylactic measure. The reconstruction was completed in 20 minutes, and the patient tolerated the procedure well. This was supported by intra-operative anticoagulation with intravenous heparin just before reconstruction followed by subcutaneous low molecular weight heparin in the early post-operative period. At discharge, the patient was put on long-term oral anticoagulation.  Treatment of IVC LMS still remains a challenge. The rarity of the tumor, high local recurrence rate despite complete surgical resection makes IVC LMSs one of the greatest surgical ultimata even today.

REFERENCES:

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FIGURE LEGENDS

1. Figure 1A-B: Contrast-enhanced computed tomography of the abdomen showed an ill-defined enhancing hypodense lesion with calcification involving retroperitoneal region extending to caudate and inferior vena cava with features of leiomyosarcoma.

2. Figure 2A-D:

A & B – Mass arising from retro hepatic portion of suprarenal inferior vena cava measuring about 10 * 8 cms, exophytic growth, infiltrating the caudate lobe.
C – IVC reconstruction using ring 22 Fr Gelatin-impregnated polyester-knitted vascular prosthesis.
D – Resected specimen with the segment of IVC.

3. Figure 3A – B:

A – Pleomorphic spindle to plump oval cells with marked nuclear atypia and frequent mitosis. Giant cells seen with areas of necrosis.
B – Foci shows osteoid with calcification.

Dr. Jithin Jagan A
Associate Consultant – Department of Vascular Surgery
Kauvery Hospital