Surgical Resection of a Leiomyosarcoma Involving Atrial Reconstruction, Cardiopulmonary Bypass, and Ex-Vivo Liver Resection and Reimplantation

Bunting, Bryant; Marsh, J. Wallis; Wei, Lawrence M.; Hughes, Christopher; Gelzinis, Theresa A.

doi:10.1053/j.jvca.2016.06.019

Cited by 10 publications

(8 citation statements)

References 9 publications

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“…Oppression by giant tumor and / or returning obstacles of venous flow are the main causes of symptoms including back pain, abdominal lump, leg edema and sometimes hepatomegaly and ascites. When the leiomyosarcoma affects heaptocaval region, Budd-Chiari syndrome could develop as clinical symptoms [9,10] . Due to the lack of specific tumor marker, some patients with early leiomyosarcoma could be presented with varicose vein of lower limb, deep venous thrombosis or ascites, therefore these conditions should be cautiously taken with further investigation with CT, MRI scan and ultrasonography required to rule out the possibility of leiomyosarcoma [11] …”

Section: Discussionmentioning

confidence: 99%

“…When the leiomyosarcoma affects heaptocaval region, Budd-Chiari syndrome could develop as clinical symptoms. [9,10] Due to the lack of specific tumor marker, some patients with early leiomyosarcoma could be presented with varicose vein of lower limb, deep venous thrombosis or ascites, therefore these conditions should be cautiously taken with further investigation with CT, MRI scan and ultrasonography required to rule out the possibility of leiomyosarcoma. [11] For patients who are tolerable to general anesthesia and with no remote organ metastasis, R0 surgical resection is vital important and provide long-term survival.…”

Section: Discussionmentioning

confidence: 99%

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Surgical management strategy for leiomyosarcoma of Zone I-II inferior vena cava

Tulahong¹,

Tuxun²,

Yao³

et al. 2022

Medicine

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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.Abbreviations: CT = computed tomography, DFS = disease free survival, IVC = inferior vena cava, OS = overall survival.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Surgical management strategy for leiomyosarcoma of Zone I-II inferior vena cava

Tulahong¹,

Tuxun²,

Yao³

et al. 2022

Medicine

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“…On review of the literature, seven relevant case reports could be identified (Table 1). Leiomyosarcoma with cephalad extension to hepato-caval region, hepatic veins, and right atrium are the main reasons for ELRA (19). Veno-venous bypass was commonly reported and CPB considered as long as atrial reconstruction was needed and/or IVC calming was impossible due to extreme extension to pericardial IVC (17,19).…”

Section: Discussionmentioning

confidence: 99%

“…Leiomyosarcoma with cephalad extension to hepato-caval region, hepatic veins, and right atrium are the main reasons for ELRA (19). Veno-venous bypass was commonly reported and CPB considered as long as atrial reconstruction was needed and/or IVC calming was impossible due to extreme extension to pericardial IVC (17,19). Multi-organ resection and combined bilateral kidney autotransplantation were reported (18,20) as an extreme radical procedure with acceptable survival.…”

Section: Discussionmentioning

confidence: 99%

Ex Vivo Liver Resection and Autotransplantation as Surgical Option for Zone II–III Leiomyosarcoma of IVC: A Case Report and Literature Review

Tuxun

Apaer

et al. 2021

Front. Oncol.

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We report the first documented case of leiomyosarcoma at zone II-III of inferior vena cava with thrombi in three hepatic veins undergoing ex vivo liver resection and autotransplantation (ELRA) and hepatic veins thrombectomy. A 33-year-old female patient presented with abdominal distention and lower extremities edema. Abdominal wall varicosis and shifting dullness were positive on physical examination. Her liver function was classified as Child-Pugh B and a solid tumor at retro-hepatic vena cava extending to right atrium with thrombi in three hepatic veins were confirmed. The diagnosis of leiomyosarcoma with Budd-Chiari syndrome was highly suspected with preoperative ultrasound, echocardiogram, CT scan, and three-dimensional reconstruction. A zone II-III leiomyosarcoma of IVC origin was confirmed at surgery and ex vivo liver resection and autotransplantation, and hepatic vein thrombectomy with atrial reconstruction were performed under cardiopulmonary bypass (CPB). Operative time, anhepatic time, and CPB time were 12 h, 128 min, and 84 min, respectively. The patients experienced post-operative liver dysfunction and was cured with conservative therapy. Hepatic recurrence two years after surgery was managed with radiofrequency. The patient was alive with liver metastasis three years after surgery. Despite being regarded as an extremely aggressive procedure, ELRA could be considered in the treatment of advanced leiomyosarcoma with Budd-Chiari syndrome and hepatic vein thrombi.

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“…Retro-hepatic inferior vena cava (IVC) leiomyosarcoma is a rare condition with special anatomic considerations that make resection and reconstruction difficult. Case reports detailing successful tumor removal are few (in the hundreds), speaking to the condition’s rarity and the expertise required to perform a successful resection ( 1 , 2 ). The techniques of resection described in the literature include: En-bloc liver and IVC resection.…”

Section: Introductionmentioning

confidence: 99%

Case report: Surgical resection of a retro-hepatic leiomyosarcoma involving atrial reconstruction, cardiopulmonary bypass, ex vivo tumor resection, and liver re-implantation

Sharma

Okakpu²,

Murthy³

et al. 2022

Front. Surg.

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IntroductionLeiomyosarcomas (LMS) involving the inferior vena cava (IVC) is a clinically rare entity, accounting for approximately 0.5% of all adult sarcomas.Case presentationA 67-year-old male presented to the emergency department with mild back and lower abdominal pain. During the workup, a computed tomography scan without contrast showed an area of decreased attenuation within the liver adjacent to the intrahepatic IVC. Magnetic resonance imaging confirmed the involvement of the retro-hepatic IVC; biopsy confirmed the diagnosis of LMS. Given the location of the involvement of the retro-hepatic IVC, liver explantation was deemed necessary for adequate tumor resection. The superior extension of the tumor toward the heart necessitated Cardio-Pulmonary (CPB). The patient successfully underwent a complex surgical procedure involving liver explantation with ex vivo back-table resection of the retro-hepatic LMS, replacement of the retro-hepatic vena cava with a ringed Gore-Tex graft, liver re-implantation, and hepatic vein-atrial reconstruction under cardiopulmonary bypass. There were no intraoperative or post-op complications.DiscussionThe role of vascular reconstruction of the IVC varies depending on the level and extent of the tumor, with options ranging from primary repair, ligation, or reconstruction dictated. Surgical resection with negative margins remains the treatment of choice due to the lack of efficacy of adjuvant therapies. Importantly, liver explantation offers a chance for complete surgical resection and reconstruction. Similarly, the complex nature of the tumor necessitated a pioneering approach involving direct hepato-atrial venous anastomosis.ConclusionTo the best of our knowledge, this is the first reported case in which the hepatic veins were anastomosed directly to the right atrium while also replacing the native vena cava with a separate graft.

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Surgical Resection of a Leiomyosarcoma Involving Atrial Reconstruction, Cardiopulmonary Bypass, and Ex-Vivo Liver Resection and Reimplantation

Cited by 10 publications

References 9 publications

Surgical management strategy for leiomyosarcoma of Zone I-II inferior vena cava

Surgical management strategy for leiomyosarcoma of Zone I-II inferior vena cava

Ex Vivo Liver Resection and Autotransplantation as Surgical Option for Zone II–III Leiomyosarcoma of IVC: A Case Report and Literature Review

Case report: Surgical resection of a retro-hepatic leiomyosarcoma involving atrial reconstruction, cardiopulmonary bypass, ex vivo tumor resection, and liver re-implantation

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