Leiomyosarcoma (LMS) of inferior vena cava (IVC) is a rare neoplasm affecting approximately 1/100,000 people. The prognosis is poor and potential curative intent occurs through challenging operations, such as vena cava resection, occasionally multivisceral when required, and vascular reconstruction. There are few retrospective series regarding this retroperitoneal neoplasm, and the aim of the present study was to discuss the experience at the São Paulo Cancer Institute and Clinics Hospital of University of São Paulo Medical School, São Paulo, Brazil. The current study is a retrospective review of 7 patients treated in the two tertiary hospitals between 2005 and 2013. Oncological and operative aspects were discussed, primarily regarding surgical aspects highlighting
Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare type of neoplasm, accounting for ~0.5% of adult soft tissue sarcoma, affecting <1/100,000 of all adult malignancies (
LMS of the IVC are usually presented as large tumors at the time of diagnosis. In several studies, tumors were >10 cm (
Surgery is currently the only potentially curative therapy. The paramount aim when approaching IVC LMS include achieving local control, maintaining the patency of major venous flow and identifying the most effective adjuvant therapeutic strategies to reduce the recurrence rate. However, the technical challenges presented by anatomical characteristics of this disease raise important issues, such as the role of multivisceral resection and vascular options of reconstructions. The clinical expertise on radical resection and venous reconstruction remains limited and data regarding multimodal therapies, such as chemotherapy (CT), radiation therapy (RT), or both (CRT) in combination with surgical resection are scarce, with the optimal treatment strategy remaining unclear.
In the present study, a series of seven patients submitted to operative treatment of primary LMS of the IVC was reviewed, and the effect of multivisceral resection on survival rate and the options of venous reconstruction were analyzed.
A retrospective review was performed on the medical records of all the patients treated with upfront resection of primary IVC LMS over a five-year period between June/2007 and October/2013. Variables collected from the medical records included demographic and clinical data, tumor location along the IVC, adjuvant therapies received, surgical technique employed, and the surgical pathology report. Segments of IVC affected by the tumor were classified according to Kulaylat
The primary endpoints of the study were postoperative mortality and morbidity, and OS rate. Other variables, such as status of resection secondary to radical resection (R0, R1 or R2 resections) and the patency of the graft utilized in the vascular reconstruction were reported as secondary endpoints.
The classification by Kulaylat
OS rates were calculated using the time between the date of surgery and the date of mortality or last contact. DFS was defined as the time to local or distant tumor recurrence following initial treatment. Kaplan-Meier estimator survival curves for OS and DFS were determined (
A 78-year-old male with a 4-month history of abdominal pain was admitted to the pancreas and biliary tract surgery service at the Clinics Hospital affiliated to the University of São Paulo (São Paulo, Brazil). A computed tomography (CT) scan (
A 68-year-old female with a six-month history of right upper quadrant pain was found to have a 20 cm sub-hepatic mass at the level of the right renal hilum. The patient was admitted to the Heart Institute at the University of São Paulo and was referred to the general surgical oncology group for further evaluation. A CT scan of the abdomen revealed the mass arising from the vena cava and extending into the lower segment of the IVC, with compression of the right renal vein. Operative resection was similar to the first case. Exploration was performed via a Mercedes incision, and the tumor was identified along the IVC just below the liver and the right renal vein. Proximal and distal controls from the cava were obtained, and the mass and the right kidney were dissected free. The IVC was clamped inferiorly 4 cm above the bifurcation of the common iliac veins and superiorly in the infra-hepatic segment of the IVC. The left renal vein was sectioned near the IVC. A large mass was removed with 20 cm of the IVC
A 34-year-old female with a six-month history of right upper quadrant pain was found to have an 11 cm sub-hepatic mass, which was suspected as a primary IVC tumor. A CT scan of the abdomen revealed a mass arising from the vena cava and extending into the lower segment of the IVC. Intraluminal filling defects were detected, indicating the invasion of the tumor into the IVC and bilateral renal veins (
An 81-year-old female patient, who had atrial fibrillation and was anticoagulated, was present with vague abdominal pain for 3 months. The CT scan demonstrated an ~5 cm mass arising from the IVC and inferior to the right renal vein with no other associated abnormalities. The patient underwent surgical resection of the mass, including complete resection of the IVC below the level of the renal veins. No reconstruction was performed. The patient had an uneventful postoperative recovery. Pathological analysis revealed a high-grade 5.3 cm LMS of the IVC with negative surgical margins. The patient has no evidence of disease at the time of this report, 69 months following the initial surgery. However, at 42 months the patient appeared in the emergency department with vaginal bleeding. The pelvic CT scan revealed an exuberant collateral circulation and multiple pelvic varices. The bleeding stopped spontaneously. At present, the patient is doing well with no more episodes of bleeding.
A 53-year-old female patient presented with right lumbar pain for 2 months. An abdominal ultrasonography revealed a 4.5×4.5 cm mass, which was suspected as a primary tumor of the right kidney. The patient was admitted to the urology service at the Clinics Hospital. The CT scan demonstrated a 6.9×6.3 cm tumor between the pancreas head, right kidney and liver, which was most probably a primary IVC tumor. Preoperative endoscopic eco-guided biopsy revealed fusiform cells with no atypia. The urology and vascular surgery teams planned the operative procedure together. Exploration was performed via a right subcostal incision. Complete resection of the IVC below the level of the renal veins was performed. Surgical margins were negative. IVC reconstruction was performed using a 20-mm Dacron prosthetic graft. Pathological analysis revealed an 8.0 cm grade II (FNCLCC) LMS of the IVC. The postoperative course was unremarkable. Therapeutic anticoagulation with enoxaparin was performed within 6 months. Thrombosis of the graft was identified in an abdominal CT 3 months following resection. A thoracic CT scan 4 months following surgery demonstrated multiple small pulmonary nodules, which was suspected as pulmonary metastasis. The last follow-up was 38 months following diagnosis. The patient succumbed to the disease.
A 49-year-old female patient presented with right lumbar pain for 1 month. The CT scan demonstrated a 9.2 cm retroperitoneal mass suspected to be a right primary adrenal tumor with invasion of the IVC. Biopsy guided by imaging revealed an LMS. Surgical exploration was performed via a bilateral subcostal incision. IVC ligation below the tumor was performed, as well as ligation of the left renal vein. The retrohepatic IVC was sutured with vascular reconstruction. The tumor was removed
A 53-year-old female patient presented with abdominal pain. The CT scan revealed a heterogeneous 10.0×9.0×7.0 cm mass adjacent to the right kidney and invading the posterior wall of the IVC. Following clinical staging, a complete macroscopic resection was performed by the surgical urology team with ligation of the IVC just below the right renal vein. No vascular reconstruction was attempted. Pathological analysis revealed an 11.5×7.2×6.5 grade II (FNCLCC) LMS of the IVC with narrow margins. The CT scan 4 months postoperatively revealed atrophy of the right kidney with thrombosis of the right renal vein. Following 49 months from the resection of the primary tumor, the patient is doing well with no evidence of disease.
Out of the seven patients evaluated in the present study, only one was male (
Reconstruction of the IVC was performed in 4 patients using Dacron grafts. Reconstruction of the left renal vein was performed in 3 patients using PTFE grafts. No patients underwent resection on cardiopulmonary or venovenous bypass. All the patients underwent complete resection of the tumor and microscopic-free surgical margins were accomplished in 6 patients.
The median tumor size was 10 cm [interquartile range (IQR) 25–75%, 8–12 cm]. The tumor grade and follow-up for all the patients was reported. The OS rates were 100, 60 and 25% at 3, 4, and 5 years (
LMS is one of the most frequent types of retroperitoneal soft tissue sarcoma. For all newly diagnosed soft tissue sarcoma, the estimated incidence of LMS ranges between 10 and 20% (
Macroscopic surgical resection is the primary curative treatment for patients with localized disease. This is the only potentially curative therapy since the first resection of the LMS of the IVC at Lexington Memorial Hospital in Chicago, in 1951 (
The majority of patients described in the present study presented with abdominal pain, which is the first indication for upfront resection when feasible. Approximately 60% of the patients presented with non-specific abdominal pain (
In the present study, ~50% of patients underwent
In the present case series, the median survival of 21 months confirmed that radical resections of the LMS of the IVC with the intention of obtaining a complete macroscopic resection with negative margins should be the aim for those with localized disease and acceptable clinical performance. All the patients achieved complete macroscopic resections and only one patient had microscopic positive surgical margins. This patient succumbed to the disease 48 months following resection of the primary tumor. The results of previous studies have revealed that microscopic positive surgical margins have no effect on DFS and OS rates (
One issue following radical resection is the reconstruction of the IVC and occasionally the left renal vein. Several options exist when vascular reconstruction is considered (
In a long-term follow-up, no lower extremity edema was observed in patients who underwent IVC reconstructions in the current series. In a previous study, lower extremity edema was considered significant in 50% of patients when no IVC reconstruction is undertaken (
When the patients presented with a patent IVC, vascular reconstructions were performed, as identified by previous results (
In the present study, two patients with level II LMS underwent an end-to-side anastomosis between the left renal vein and an 18-mm Dacron prosthesis was used to reconstruct the IVC. A 6-mm PTFE between the left renal vein and Dacron were chosen to accomplish the left renal outflow. In another patient with level II LMS of the IVC it was possible to remove the tumor and maintain both kidneys. The distal end of the IVC was ligated and excluded, and the bilateral veins were clamped. Vascular reconstruction was performed creating an anastomosis between the cranial stump of the IVC and an 18-mm Dacron graft was used with bilateral 8-mm arms that were anastomosed to the bilateral renal veins. This type of reconstruction has previously been reported in other studies (
In conclusion, LMS of the IVC is a rare retroperitoneal sarcoma, and radical resection is the only therapeutic option capable of conferring long-term survival. To obtain complete macroscopic resection, removal of adjacent organs is usually necessary. Microscopic-free surgical margins are necessary but its effect on long-term survival remains unclear. Venous reconstruction is selectively indicated. There is no consensus, but in general, when partial obstruction of the IVC occurs the reconstruction of the IVC is encouraged.
The classification of vena cava sarcoma according to Kulaylat
Abdominal computed tomography scan revealing an inferior vena cava sarcoma infiltrating the right renal vessels, indicated by the arrow.
Retroperitoneal tumor dissected in a patient with leiomyosarcoma of the inferior vena cava.
Image captured during inferior vena cava reconstruction of a patient with leiomyosarcoma.
Computed tomography scan revealing an inferior vena cava sarcoma infiltrating both renal vessels. The arrows highlight the vessel's invasion.
Image captured during inferior vena cava reconstruction of a patient with leiomyosarcoma.
Kaplan-Meier survival curve for the overall survival rate at 5 years of patients with leiomyosarcoma of the inferior vena cava.
Kaplan-Meier survival curve for the disease-free survival rate at 5 years of patients with leiomyosarcoma of the inferior vena cava.
Surgical oncological features and vascular reconstructions of patients with leiomyosarcoma of the IVC.
Patient | Gender | IVC segment | R status | Organs resected | IVC/LRV reconstruction | RFS, months | Recurrence site | Follow-up, months | Current status |
---|---|---|---|---|---|---|---|---|---|
1 | M | II | R0 | Right kidney | VC 18 mm Dacron graft + PTFE on LRV | 28 | Lung | 57 | Succumbed |
2 | F | II | R0 | Right kidney | VC 20 mm Dacron graft + 6 mm PTFE on LRV | – | None | 39 | Alive with NED |
3 | F | II | R0 | IVb liver segment | Distal VC ligation + 18 mm Dacron with bilateral 8 mm RVA | 14 | Lung/liver | 46 | Alive with disease |
4 | F | I | R0 | None | Both IVC segments ligated with no vascular reconstruction | – | None | 69 | Alive with NED |
5 | F | I | R0 | None | VC 20 mm Dacron graft | 38 | Lung | 38 | Succumbed |
6 | F | II | R1 | Right kidney and adrenal | Both IVC segments ligated with no reconstruction | 8 | Lung | 48 | Succumbed |
7 | F | I | R0 | None | Both IVC segments ligated with no reconstruction | – | None | 49 | Alive with NED |
IVC segment was classified according to Kulaylat