Leiomyosarcoma Of Inferior Vena Cava Research Articles

ASO Author Reflections: Inferior Vena Cava (IVC) Resection Without Reconstruction for IVC Leiomyosarcomas: Heading Toward a New Reality.

ASO Author Reflections: Inferior Vena Cava (IVC) Resection Without Reconstruction for IVC Leiomyosarcomas: Heading Toward a New Reality.

En Bloc Inferior Vena Cava (IVC) Resection Without Reconstruction With Right Hepatectomy and Right Nephrectomy for a Large IVC Leiomyosarcoma.

Radical resection remains the only potential cure in the management of inferior vena cava (IVC) leiomyosarcomas with multivisceral resections often needed (Borghi et al. in J Cardiovasc Surg (Torino) 63:649-663, 2022). This video describes the technical nuances of surgical resection of a large retrohepatic IVC leiomyosarcoma. Computed tomography of a 60-year-old woman revealed a 12 × 12 × 9.5 cm mass in the right suprarenal region infiltrating the IVC with intraluminal extension up to the hepatic venous confluence. The mass involved the right hepatic vein with infiltration of segment 7 of the liver and splaying of the right portal vein. Robust lumbar venous drainage from the infratumoral IVC was seen. En bloc IVC resection without reconstruction along with a right hepatectomy and right nephrectomy was performed via a right thoracoabdominal approach. After a Catell-Braasch maneuver, the surgery can be broadly divided into four major steps: (1) Right retroperitoneal mobilization of the tumor and right kidney with infratumoral IVC control, (2) mobilization of the right liver with suprahepatic IVC control, (3) division of the right portal structures with right hepatectomy, and (4) en bloc resection of the IVC tumor. Reconstruction of the IVC was not performed owing to the presence of venous collaterals (Langenbecks et al. in Arch Surg 407:1209-1216, 2022). Final histopathology showed a high-grade leiomyosarcoma with histologic organ invasion in the liver and right kidney with resected margins free of the tumor (R0). Meticulous preoperative planning and expertise in liver resection and retroperitoneal surgeries facilitates such radical yet safe multivisceral resection for a large retrohepatic IVC leiomyosarcoma without the need for a cardiopulmonary bypass.

Surgical outcomes of patients with inferior vena cava leiomyosarcoma

IntroductionPrimary vascular leiomyosarcomas are incredibly rare and have a poor prognosis. The purpose of this study was to analyze the surgical outcomes of patients with primary inferior vena cava (IVC) leiomyosarcoma. MethodsWe performed a retrospective review of IVC leiomyosarcoma resections performed at a single tertiary care hospital from 2014 to 2023. A total of 13 cases were analyzed, including 10 women and 3 men. The presenting symptoms, tumor characteristics, operative management, postoperative complications, and survival rates were assessed for each patient. ResultsThe median patient age was 59 years (quartile [Q]1, 52 years; Q3, 68 years). The median tumor size was 7.0 cm (Q1, 6 cm; Q3, 12 cm). The median mitotic rate was 6 per 10 high-power fields (Q1, 2.5; Q3, 15.5). All 13 patients underwent grossly negative tumor resection, with 9 (69%) having microscopically negative margins (R0). No patient had lymph node involvement. The IVCs were managed with ligation in four patients for tumors already occluding the IVC and bovine pericardial patch angioplasty in seven patients or primary repair in two patients for patent IVCs. Concomitant right nephrectomy was performed in seven patients. Left renal vein ligation was performed in three patients, but no left nephrectomies were performed. Significant postoperative complications included one patient with lower extremity compartment syndrome, two patients with severe leg swelling, and one patient with arm swelling. The 30-day mortality rate was zero. Using the Kaplan-Meier product limit method, disease-specific survival was estimated to be 93%. ConclusionsSurgical resection is a feasible and effective oncologic treatment option for patients with IVC leiomyosarcoma. The IVC can be safely managed by ligation, primary repair, or patch angioplasty, depending on the prior patency of the IVC.

When Vessels and Sarcomas Combine: A Review of the Inferior Vena Cava Leiomyosarcoma

Leiomyosarcomas (LMSs) are malignant neoplasms of soft muscle differentiation that can be classified into five distinct groups according to site-related origin: intra-abdominal, subcutaneous or deep soft tissue of the limbs, cutaneous, external genitalia, and vascular. This distinction reflects different biological behaviors as well as molecular changes, thus reflecting different prognoses and therapeutic options. Vascular LMSs are the least frequent, arising from the walls of the blood vessels, most commonly from the inferior vena cava. Due to its deep location, symptoms are non-specific, and the disease presents at an advanced stage, sometimes with metastases. Surgery is the treatment of choice, associated with chemo- and radiotherapy. Due to its rarity, most departments have minimal experience handling this disease. This article reviews the current knowledge on vascular leiomyosarcomas, particularly the inferior vena cava leiomyosarcoma.

HPB P02 Leiomyosarcoma of IVC

Abstract Background Leiomyosarcoma of the inferior vena cava (IVC) is a very rare entity with poor 5-year prognosis. Less than 400 cases have been reported in literature. The extent of mass forming lesions is quite progressed upon discovery and multiorgan resection is often required. Here we present our 5year experience and surgical management of this malignancy. Methods From 2000 - 2023, 7 cases with IVC Leiomyosarcoma were treated in our institution. The median patient age was 59 years old with female gender predominance involvement. All patients presented with vague abdominal symptoms prompted imaging studies discovering the tumour, or the latter was an incidental finding of a retroperitoneal mass involving the IVC and adjacent organs (liver, kidney, duodenum). Only 4 patients (56%) had preoperative CT guided biopsy confirming the diagnosis and 3 cases were confirmed from post operative histology. Results All cases were reviewed in our multidisciplinary meeting and 4 patients (56 %) underwent surgical resection with caval replacement using a graft apart from one case which underwent IVC ligation. Liver resection was required after portal flow manipulation in two cases. Postoperative complications were ascites and chyle leak which were managed conservatively. Resection margins were negative (R0) to all cases. One case recurred and underwent further liver resections. Chemotherapy was offered only to unresectable cases (42%). From resected patients three of them (42%) are still alive with longest follow up of 4.5years post-resection and one died after multiple resections. Conclusions IVC Leiomyosarcoma is an aggressive malignancy and requires major surgery upon diagnosis. Surgery is the gold standard treatment since unrespectability upon diagnosis has poor prognosis. Due to rarity of the disease, large studies are difficult to be conducted.

Inferior vena cava leiomyosarcoma in a renal transplant recipient

Inferior vena cava leiomyosarcoma in a renal transplant recipient

Comparison of surgical outcomes between leiomyosarcoma of inferior vena cava and other locations

Comparison of surgical outcomes between leiomyosarcoma of inferior vena cava and other locations

Inferior vena cava leiomyosarcoma – A challenge for management of a rare tumour – A case report

Inferior vena cava leiomyosarcoma – A challenge for management of a rare tumour – A case report

Ressecção cirúrgica de leiomiossarcoma de veia cava inferior retro-hepática sem reconstrução vascular: relato de caso

Abstract Inferior vena cava leiomyosarcoma (IVCL) is a rare malignant mesenchymal tumor. Surgical treatment is a challenge because it must combine free surgical margins with vascular reconstruction, using prosthetic or autologous grafts, primary suture, or simple ligation without vein reconstruction. The ligation option is possible thanks to the slow growth of the tumor, allowing collateral venous circulation to develop. We present a case of an IVCL treated with radical resection without vascular reconstruction. The patient was a 48-year-old female with abdominal pain in the right upper quadrant, asthenia, and postprandial dyspeptic symptoms. Abdominal tomography revealed a mass with an expansive formation located in the infrahepatic segment of the inferior vena cava and reduced vessel lumen. During surgery, vein clamping did not provoke hemodynamic repercussions, suggesting sufficient collateral circulation formation. It was decided to perform a radical resection of the entire portion of the retrohepatic vena cava and ligate the vena cava without vascular reconstruction. The patient recovered without complications.

Resection of inferior vena cava leiomyosarcoma and reconstruction using ProxiCor patch

Introduction. Leiomyosarcoma of the inferior vena cava (IVC) is a rare mesenchymal tumor originating from the endothelial smooth muscle of the intima and account for about 1?2% of all the sarcomas of the soft tissue. The objective of this article is to show a case of IVC leiomyosarcoma, it?s resection and reconstruction using a ProxiCor patch. Case outlines. We showed a case of a 65-year-old woman presented with abdominal pain and mass in subhepatic space, who underwent surgery and resection of a leiomyosarcoma of IVC. IVC was reconstructed with ProxiCor patch, and histopathologically confirmed that it was leiomyosarcoma. Conclusion. Our experience has shown that the application of extracellular matrix is safe and has given a satisfactory treatment result. A comparison with a larger patient sample should give a true representation of the advantages and disadvantages of this type of material in vascular reconstructive procedures.

PET/MR: primary inferior vena cava leiomyosarcoma

Positron emission tomography (PET) combined with a magnetic resonance (MR) scanner (PET/MR) with 18F-fluorodeoxyglucose (FDG) tracer is being used in quite a few nuclear medicine centers. The aim of this study is to illustrate two uncommon cases of primary inferior vena cava leiomyosarcoma which were formerly evaluated with anatomical images such as computed tomography and ultrasound. These techniques were inferior in the definition of the tumor and its characteristics. F-18 FDG PET/MR was essential and provided all the necessary information: its origin, local extension, anatomo-metabolic behavior, form of presentation, and distant metastasis in one single diagnostic technique. PET/MR accurately contributed to the diagnosis in a shortened period of time and, therefore, in the prognosis of this disease with greater benefits.

Primary Inferior Vena Cava Leiomyosarcoma Presenting with Proximal Occlusion with Deep Venous Thrombosis and Lower Extremity Edema: Reconstruct vs Ligate Post Resection? A Single Retrospective Case Report

Introduction: Primary leiomyosarcomas of the inferior vena cava (IVC) are rare tumors with a poor prognosis. Curative treatment remains surgical resection with clear margins. Reconstruction vs ligation of the IVC is debatable, especially if the proximal IVC is occluded. This report presents the surgical management of a patient presenting with acutely worsening lower extremity edema and biopsy proven IVC leiomyosarcoma. Methods: This is a retrospective chart review, and the patient was followed up at 2 weeks and at 3 months. Patient provided consent for creation of this case report. Results: The patient’s age at presentation was 67 years and she was of female gender. The IVC leiomyosarcoma had a maximum diameter of 4.8cm, involved the infrarenal IVC only, and the proximal segment was occluded due to bilateral iliofemoral deep venous thrombosis (DVT). She underwent an R0 resection, with immediate reconstruction using 14mm ringed PTFE. Postoperatively, she was noted to have worsening lower extremity edema. The plan was to recanalize her DVTs via endovascular thrombectomy and stenting, however the patient’s edema improved with compression alone. At 2 weeks follow up, her edema had completely resolved. At 3 months follow up, postoperative CT scan showed expected occluded infrarenal IVC graft, and no evidence of recurrent disease. Conclusion: Reconstructing the occluded IVC after resection of the primary IVC leiomyosarcoma gives the patient the option for future endovascular recanalization if there is ongoing lower extremity edema. Further follow up and research is warranted to determine if this provides a feasible long-term solution.

Leiomyosarcoma of the inferior vena cava presenting with bilateral lower extremity edema with comorbid sarcoidosis: A case report

We present a case of a 70-year-old female with Leiomyosarcoma (LMS) of the inferior vena cava (IVC). Although this is an extremely rare entity, in contradistinction, it is also the most common primary malignancy of the IVC [5]. The patient has a history of sarcoidosis, hypertension, diabetes mellitus type two, and chronic obstructive pulmonary disease (COPD). She presented with a complaint of bilateral lower extremity edema and was admitted where a computerized tomography (CT) scan of the abdomen and pelvis showed a large mass filling the IVC, a finding confirmed by magnetic resonance imaging. Radical resection of the retroperitoneal tumor was carried out including portions of the inferior vena cava with en bloc radical right nephrectomy and right adrenalectomy. The pathologic diagnosis of inferior venal caval leiomyosarcoma (IVC LMS) was made with positive immunostains for desmin, vimentin and smooth muscle actin. The rarity of this entity, clinical presentation along with concomitant sarcoidosis makes this an interesting case.

Surgical management strategy for leiomyosarcoma of Zone I-II inferior vena cava: A case series.

Objectives:Leiomyosarcoma of inferior vena cava (IVC) is a rare clinical entity with severe vascular involvement. Surgical management of leiomyosarcoma is still challenging.Methods:This a retrospective study of consecutive patients referred to our hospital from January 2017 to June 2019. Depending on the anatomical site of affected IVC, leiomyosarcomas were categorized into zone I-II. The clinical data including baseline information, surgical parameters, peri-operative management, short- and mid-term outcomes were observed.Results:Four patients with leiomyosarcoma of zone I-III underwent radical resection without intraoperative mortality. Prosthetic grafts were interpositioned in all patients to instruct vena cava. Renal vein reconstruction was perfumed in two patients due to involvement to renal veins. Median blood loss was 450 mL (200–600 mL), median operative time was 215 minutes (150–240 minutes). No Clavien-Dindo IIIa or higher complication was observed. No organ dysfunction and recurrence were observed with median follow-up of 25.5 months.Conclusions:Curative resection of zone I-II leiomyosarcoma is associated with longer survival in selected cases, en-bloc resection with complex vascular reconstruction could be considered.

Surgical treatment of vena cava leiomyosarcomas: Series of cases in a referral center and literature review

BackgroundPrimary tumors of the inferior vena cava are rare tumors of mesenchymal origin. They arise from the smooth muscles of the vena cava wall. Due to its low prevalence, there are few definitive data on its treatment and prognosis. Its treatment is based on general oncological principles. MethodsA series of 6 cases operated from 2010 to 2020 were analyzed. Different parameters related to the demographic characteristics, the tumor, the treatment received, and the results obtained in survival and morbidity were analyzed. In addition, a bibliographical review of the currently available evidence was carried out. ResultsOptimal surgical resection was accomplished in all patients with R0 in 4/6 and R1 in 2/6. The greatest morbidity occurred in a patient who died in the intraoperative period. Cavorraphy was performed in one patient and cavoplasty in 5/6 using cryopreserved graft in 3/6 and prothesis in 2/6. The 50% were still alive at the end of the follow-up (with a mean follow-up of 10.7 months). The mean survival was 11.3 ± 9.07 months. 3/6 patients presented hematogenous recurrences with a disease-free interval of 9 ± 2 months. ConclusionThe diagnosis and treatment of inferior vena cava leiomyosarcoma is still a challenge. Due to its low prevalence, it will be difficult to establish a totally standardized treatment and its approach is recommended in specialized centers. On the other hand, a multicentric study should be made to collect the most cases as possible in order to advance in the understanding of the approach to this disease.

A 10-year experience of leiomyosarcoma of the inferior vena cava.

Leiomyosarcoma of the inferior vena cava (IVC) is rare. The study reviewed patients with IVC leiomyosarcoma in our hospital in the past ten years. Twenty patients diagnosed with IVC leiomyosarcoma between October 2010 and October 2020 were enrolled. Their clinical manifestations, treatments, and follow-up results were analyzed. The sarcoma was located in the lower IVC segment in six patients, with 13 in the middle and one in the upper IVC segment. Eighteen patients underwent R0 resection. After resection, 16 patients (80%) had primary repair of the IVC, while four patients underwent ligation. During a mean follow-up of 37.7months, seven patients died due to tumor metastasis, four patients were alive with the tumor recurrence and other nine patients were alive without recurrence. The management of the IVC after tumor resection depended on the tumor location and size. R0 resection provided a chance for long term survival.

Surgical resection and graft replacement for primary inferior vena cava leiomyosarcoma: A multicenter experience: Accepted for presentation at the 2020 Vascular Annual Meeting of the Society for Vascular Surgery, Toronto, Ontario, Canada, June 17-20, 2020 (conference cancelled). Presented at the 2020 Society for Vascular Surgery ONLINE, June 20-July 31, 2020.

Objective Primary leiomyosarcoma of the inferior vena cava (IVC) is best managed with surgical resection when technically feasible. However, consensus is lacking regarding the best choice of conduit and reconstruction technique. The aim of the present multicenter study was to perform a comprehensive assessment through the VLFDC (Vascular Low Frequency Disease Consortium) to determine the most effective method for caval reconstruction after resection of primary leiomyosarcoma of the IVC.

Leiomyosarcoma of inferior vena cava (IVC): do we really need to reconstruct IVC post resection? Single institution experience.

Inferior vena cava (IVC) leiomyosarcomas (LMS) are a rare group of retroperitoneal tumors. R0 surgical resection is the only curative modality of treatment. IVC resection for retroperitoneal sarcoma is a complex surgery with no definitive guidelines for reconstruction. Retrospective review of all patients who underwent surgical resection of primary leiomyosarcoma of the IVC requiring resection from 2010 to 2020 at our tertiary care center was performed. Among 24 patients who required IVC resection for LMS, only 7 (29%) required reconstruction of IVC. According to Clavien-Dindo classification, there was one grade 3 or more morbidity and 1 post-operative mortality. Seventeen patients underwent R0 resection whereas 7 patients had R1 resection on final histopathology. At a median follow-up of 25months (range 8-91months), the median OS was 40months with median DFS of 28months. Two patients presented with local recurrence while 13 patients developed systemic recurrence on follow-up. Careful preoperative multidisciplinary planning can make IVC resection without reconstruction feasible with acceptable perioperative morbidity, mortality, and oncological outcomes for IVC LMS.

SURGICAL TECHNIQUE IN LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA DEPENDING ON ITS LOCATION

The aim: To improve the outcomes of inferior vena cava (IVC) leiomyosarcoma, propose own classification of IVC segments, which correlates with surgical access, methodology, sequence and amount of surgery. Materials and methods: In the period from 1991 to 2021 in the Transcarpathian Regional Clinical Hospital named after A. Novak and in the Transcarpathian Antitumor Center 8 patients with IVC leiomyosarcoma were operated. The prevalence of leiomyosarcoma in IVC was determined according to the division of IVC into 7 segments. Defeat of one segment of IVC was in 50% of cases, two - in 37.5%, three - in 12.5%. In 5 (62.5%) cases circular resection and alloprosthesis of IVC were performed; in 2 (25%) – circular resection, alloprosthesis of IVC and implantation of the right and left renal veins in the prosthesis; in 1 (12.5%) - circular resection, alloprosthesis of IVC and implantation of the left renal vein in the prosthesis. All surgeries were performed with laparotomy access (87.5% by Chevron type). Results: The average operation time was 215 (160-320) minutes, the average blood loss was 305 (250-500) ml. Postoperative complications were recorded in 2 (25%) cases. There were no cases of pulmonary embolism, venous thrombosis, prosthesis thrombosis, perioperative mortality. In 7 (87.5%) cases, surgery was radical. The overall 1-year, 2-year and 3-year survival rates were 87.5%, 71.4% and 57.7%. Conclusions: The division of IVC into 7 segments characterizes the detailed definition of the cranial limit of leiomyosarcoma and segmental involvement of IVC in the tumor process, which allows to choose the right surgical tactics, perform radical resection of IVC and maintain laminar blood flow to IVC and its tributaries.

SURGICAL TECHNIQUE IN LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA DEPENDING ON ITS LOCATION.

The aim: To improve the outcomes of inferior vena cava (IVC) leiomyosarcoma, propose own classification of IVC segments, which correlates with surgical access, methodology, sequence and amount of surgery. Materials and methods: In the period from 1991 to 2021 in the Transcarpathian Regional Clinical Hospital named after A. Novak and in the Transcarpathian Antitumor Center 8 patients with IVC leiomyosarcoma were operated. The prevalence of leiomyosarcoma in IVC was determined according to the division of IVC into 7 segments. Defeat of one segment of IVC was in 50% of cases, two - in 37.5%, three - in 12.5%. In 5 (62.5%) cases circular resection and alloprosthesis of IVC were performed; in 2 (25%) - circular resection, alloprosthesis of IVC and implantation of the right and left renal veins in the prosthesis; in 1 (12.5%) - circular resection, alloprosthesis of IVC and implantation of the left renal vein in the prosthesis. All surgeries were performed with laparotomy access (87.5% by Chevron type). Results: The average operation time was 215 (160-320) minutes, the average blood loss was 305 (250-500) ml. Postoperative complications were recorded in 2 (25%) cases. There were no cases of pulmonary embolism, venous thrombosis, prosthesis thrombosis, perioperative mortality. In 7 (87.5%) cases, surgery was radical. The overall 1-year, 2-year and 3-year survival rates were 87.5%, 71.4% and 57.7%. Conclusions: The division of IVC into 7 segments characterizes the detailed definition of the cranial limit of leiomyosarcoma and segmental involvement of IVC in the tumor process, which allows to choose the right surgical tactics, perform radical resection of IVC and maintain laminar blood flow to IVC and its tributaries.