Inferior vena cava resection for a leiomyosarcoma

Sebai a |

La tunisie chirurgicale - 2019 ; Vol 2019

Resumé

Venous leiomyosarcoma is a rare mesenchymal tumor accounting for 1 in every 100000 malignant tumors. It’s located in the inferior vena cava in more than 50% of cases . As reports are scarce, no clear guidelines are available. Surgical resection is considered as the only potential curative treatment.However, such a surgery is technically demanding and requires a huge expertise in both visceral and vascular surgery.

Mots Clés

Inferior vena cava resection for a leiomyosarcoma

Introduction :

Venous leiomyosarcoma is a rare mesenchymal tumor accounting for 1 in every 100000 malignant tumors. It’s located in the inferior vena cava in more than 50% of cases [1]. As reports are scarce, no clear guidelines are available. Surgical resection is considered as the only potential curative treatment.However, such a surgery is technically demanding and requires a huge expertise in both visceral and vascular surgery.

Article

Aim

Present a case of a leiomyosarcoma of the inferior vena cava and expose the surgical technique to achieve its resection.

 

Case report

Herein, we present a case of a 53 years old woman, with no past medical history, who reported vague and paroxysmal abdominal pain evolving for two years. Abdominal examination found a fixed 4 centimetres mass of the right hypochondrium. Abdominal CT scan revealed an heterogenous lobulated mass developing at the expense of the anterior wall of the inferior vena cava. It measured 50*37*46 mm. On MRI, it had a low signal on T1, T2 and diffusion sequences. It was high lightened after gadolinium injection. All these findings were highly suggestive of a leiomyosarcoma of the inferior vena cava. The mass was in contact with the duodenum, the head of the pancreas and the hepatic pedicula without clear invasion. Radiology didn’t find any metastatic lesion. Thus, we decided the surgery.

A bi-sous-costal laparotomy was performed. Pringle manoeuvre permitted to secure the hepatic pedicula. Cattell-Braasch manoeuvre and Kocher manoeuvre allowed to expose the retroperitoneum, the infrahepatic vena cava and renal veins. We found a 6 centimetres solid mass developing at the expense of the anterior wall of the inferior vena cava and the right renal vein. It also invaded the ostium of the left renal vena. Hepatic pedicula, duodenum and the head of the pancreas weren’t invaded by the mass(Figure 1).We realized anen-bloc resection carrying the tumor with 6 centimetres of the anterior wall of the inferior vena cava (with preservation of the ostium of right renal) and  two centimetre of the left renal vena was  including its ostium. Margins were macroscopically free from tumor(Figure 2). We then performed a primary repair of the inferior vena cava wall and the right renal vein wall. End-to-side anastomosis with a polytetrafluorethylene (PTFE) prothesis was mandatory to reconstruct the renal left vena to the inferior vena cava(Figure 3).

 

Postoperative courses were eventless. The patient didn’t require any blood transfusion nor catecholamines. Diuresis and renal function were normal at day one. The patient was discharged at day five.

 

Discussion:

Leiomyosarcoma of the inferior vena cava (IVC) is an exceedingly rare smoothmuscle sarcoma. Approximately 300 cases have been described in the literature, and furtherresearch is needed to understand the disease and guide its management. Surgery remainsthe only potential curative treatment [2]. Postoperative radiotherapy may decrease the risk of local recurrence, but it seems to not increase the overall survival [3].

Such a surgery is complex and technically demanding, requiring a huge experience in both visceral and vascular surgery. Indeed, enlarged visceral resection may be needed to achieve a free tumor margins as the tumor may invade the duodenum, the head of the pancreas and the segment I of the liver. Moreover, vascular reconstruction is mandatory which might be complex as both venal reins are usually invaded by the tumor.

Prognosis remains poor even with a curative resection. The overall survival is 50% at five years and 30% at ten years. Five-year survival drops to 30% in R1 resections and 0% in R2 resection.[1,4]

 

Conclusion:

Leiomyosarcoma of the inferior vena cava is a rare condition which management is still unclear as randomized controlled trials are lacking. For the moment, surgical resection with R0 margins is the gold-standard. However, this surgery is very complex and technically demanding.

 

Références

  1. Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF. Surgicaltreatment and outcomes of patients with primary inferiorvena cava leiomyosarcoma. J Am Coll Surg2003;197:575e9.
  2. Ghose J, Bhamre R, Mehta N, Desouza A, Patkar S, Dhareshwar J, Goel M, Shrikhande SV. Resection of the Inferior Vena Cava for Retroperitoneal Sarcoma: SixCases and a Review of Literature. Indian J Surg Oncol 2018
  3. Jones JJ, Catton CN, O’Sullivan B, et al. Initial results of a trial of preoperative external-beam radiation therapy and postoperative brachytherapy for retroperitoneal sarcoma.Ann Surg Oncol 2002;9:346e54.
  4. Praseedom RK, Dhar P, Jamieson NV, et al. Leiomyosarcomaof the retrohepatic vena cava treated by excision and reconstructionwith an aortic homograft: a case report and reviewof literature. SurgInnov2007;14:287e91.

Figures

 

Figure 1 : Peroperative view

 

Figure 2: Vascular reconstruction

 

 

 

Figure 3 : Specimen