Laparoscopic excision of leiomyosarcoma of inferior vena cava.

Leiomyosarcoma of inferior vena cava (IVC) is a rare soft tissue tumour. Only 0.5% of all soft tissue sarcomas of adults and only 300 cases are reported till date. We describe our encounter with such a case where we were successful in radical excision of tumour with the use of vascular staplers on the IVC without compromising IVC lumen. Leiomyosarcoma of IVC is a rare entity, and laparoscopic excision of the tumour is possible in exophytic tumour.

INTRODUCTION

Leiomyosarcoma of inferior vena cava (IVC) is a rare tumour accounting for almost 0.5% of all soft tissue tumours in adults.[1] Rarity of the tumour is illustrated by the fact that an international IVC leiomyosarcoma registry established in 1992 collected only 300 cases till date.[23] The first case was reported in 1996.[4] It is a rare tumour of mesenchymal origin arising from the smooth muscles of tunica media predominantly within the IVC. Intraluminal tumour growth is rarely found, and extraluminal growth is a common presentation.[1]

It is commonly seen in females in the fifth or sixth decade of life.[5]

Anatomical classification:[6]

Level 1/segment 1: Area below the renal vein

Level 2/segment 2: Area between the confluence of the renal vein and hepatic vein

Level 3/segment 3: Entry of hepatic vein up to the right atrium.

Due to non-specific clinical presentation as well as the absence of early symptoms, retroperitoneal tumours are often not diagnosed till the disease is at an advanced stage with large tumour growth and involvement of surrounding structures.[26]

Radical en bloc resection of the affected venous segment is the treatment of choice for long-term survival.[1] Ito et al. in their study with leiomyosarcoma of the IVC concluded that radical surgery with adjuvant multimodal therapy had a 5-year cumulative survival rate of 62%.[27]

CASE REPORT

A 45-year-old male patient came with the complaints of vague abdominal pain, nausea and vomiting which was on and off. There was no other significant history.

His abdomen was soft and non-tender with no obvious palpable mass or any other remarkable findings. Other basic laboratory parameters were done which were within normal limits. Urinary vanillylmandelic acid and metanephrine, normetanephrine were done and were within normal limits.

Ultrasonography was s/o right-sided adrenal mass.

Contrast-enhanced computed tomography (CT) abdomen showed a well-defined, homogeneously enhancing, low attenuation mass lesion in precaval location on the right side compressing the pancreatic head and second part of the duodenum posteriorly, possibility of retroperitoneal mesenchymal tumour or exophytic tumour of the duodenum. Figure 1 shows CT images of the tumour.

Figure 1

Contrast-enhanced computed tomography abdomen and pelvis showing tumour at the precaval location

The patient was posted for diagnostic laparoscopy. Under all aseptic precautions and general anaesthesia with the patient in supine position and the right side tilted slightly upwards, pneumoperitoneum was created and a 10 mm port was introduced for the scope at the level of umbilicus and slightly medial to the right midclavicular line. Under direct vision, two additional 5 mm ports were placed, one in the epigastric region and the other in the right subcostal region along the anterior axillary. Figure 2 shows an intraoperative image of the IVC tumour.

Figure 2

(a) Tumour at segment II with a short pedicle; (b) tumour separated from the inferior vena cava; (c) pedicle held with vascular stapler; (d) inferior vena cava with the right renal vein and stapler line

After mobilisation of duodenum and colon, tumour was dissected all around, and to our surprise, it was an exophytic tumour arising from suprarenal IVC (just cephalad to the insertion of the right renal vein to IVC) having a very short and narrow pedicle. Major part of the tumour was retrohepatic, and mobilisation of the retrohepatic superior pole was the most difficult.

Since it was an exophytic tumour without any intraluminal extension, total excision of the tumour without compromising IVC lumen was achieved with the help of vascular stapler. Post-operative period was uneventful, and the patient was discharged on the 4th post-operative day.

Histopathology report was suggestive of tumour tissue consisting of spindle-shaped tumour cells with hyperchromatic pleomorphic nuclei arranged in predominantly fascicular pattern with tumour bundles intersecting each other at an angle. Plenty of mitotic figures per hpf were evident. Giant cells were also seen, stroma was infiltrated with dense chronic inflammatory infiltrates and at places arrogates of lymphocytes were seen. The vessels were congested suggesting it to be spindle cell sarcoma (leiomyosarcoma). Later, immunohistochemistry was done which confirmed it to be leiomyosarcoma of IVC.

The patient was advised chemotherapy. He is being followed up regularly and is disease free till date.

DISCUSSION

All cases reported in the literature were operated by open procedure with vascular graft which ranged from 30% to 54%.[6] We were successful in radical excision of leiomyosarcoma by laparoscopic method. Two-thirds of patients with leiomyosarcoma of the IVC present with localised disease amenable to curative resection. In the register of Mingoli et al., female predilection was more common with mean age in the sixth decade of life; frequency of abdominal pain was the presenting symptom, there was inconsistent association with oedema of the lower extremities and potential for development of Budd–Chiari syndrome.[4] In a study by Kieffer et al.,[6] the incidence of leiomyosarcoma involving segment I (infrarenal IVC) was 15% versus 36.7% in the compiled series of Mingoli et al.[4]

The long-term outcome of surgery for leiomyosarcoma of the IVC has been disappointing. In the international register of Mingoli et al.,[4] actual survival rates at 5 and 10 years were 49.4% and 29.5%, respectively. However, in Hines et al. (14 cases) and Hollenbeck et al. (25 cases) with sufficiently large population and satisfactory follow-up, the cancer-free survival rates at 5 years and after radical resection with curative intent were 53.3% and 23.3%, respectively. It is also found that some surviving patients present with local recurrence or distant metastasis responding to various degrees to adjuvant treatment. Recurrence occurs in more than half of the patients who undergo curative resection of the IVC leiomyosarcoma.

Goals of surgical management of these tumours include: achieving local tumour control, maintenance of caval flow and preventing recurrence.[1] Reconstruction of IVC is not only always required because of development of collaterals and gradual occlusion of IVC but also in case of pararenal leiomyosarcoma of the IVC, reconstruction of IVC and renal vein is necessary to prevent transient or permanent renal dysfunction.[28]

Due to its rarity, however, the optimal strategy for management of recurrence is not well defined, and surgical resection, radiation therapy and systemic chemotherapy have been tried with no clear superior modality. In view of the paucity of data regarding best management of this tumour, we sought to share our experience of laparoscopic surgical resection of the IVC leiomyosarcoma.

CONCLUSION

Leiomyosarcoma of the IVC is rare and presents with no specific symptoms. Long-term survival is possible after curative resection of IVC leiomyosarcoma. Surgical resection can be performed with acceptable morbidity and mortality. At the time of presentation, tumours are usually large, and resection is challenging but probably offers the best opportunity in the middle segment II tumours, with benefits of laparoscopy to the patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.