Inferior vena cava reconstruction for leiomyosarcoma of zone I–III requiring complete hepatectomy and bilateral nephrectomy with autotransplantation

Abstract

The inferior vena cava (IVC) is the most common site of leiomyosarcomas arising from a vascular origin. Leiomyosarcomas of the IVC are categorized by anatomical location. Zone I refers to the infrarenal portion of the IVC, Zone II from the hepatic veins to the renal veins, and Zone III from the right atrium to the hepatic veins. This is a rare presentation of a Zone I-III leiomyosarcoma. Fifty-two-years-old female with a medical history significant only for HTN was admitted to the hospital with bilateral lower extremity edema and dyspnea. Two-dimensional echo demonstrated a right atrial thrombus, extending into the IVC. On subsequent CT and MRI, a 15 cm mass was noted that began in the right atrium and extended into the IVC, with continuation below the renal veins to above the level of the confluence of the common iliac veins. The patient underwent a complete resection of the mass, replacement of the IVC with Dacron graft, total hepatectomy and bilateral nephrectomy, with liver and kidney autotransplantation. Pathology was consistent with a high grade spindle cell sarcoma of vena cava origin. Patient was readmitted approximately 4 weeks postoperatively to begin adjuvant chemotherapy. This case represents a zone I-III IVC leiomyosarcoma treated with surgical R0 resection. This included a hepatectomy, bilateral nephrectomy, and hepatic and left renal autotransplantation. These complex tumors should be treated with surgical resection, and require a multidisciplinary approach.

Primary Leiomyosarcoma in the Inferior Vena Cava Extended to the Right Atrium: A Case Report and Review of the Literature

“…We also performed a PubMed literature search of the past 10 years by entering the search terms ‘leiomyosarcoma’ and ‘inferior vena cava’ [3, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, …”

<p>A Case of a Huge Inferior Vena Cava Leiomyosarcoma: Precise Preoperative Evaluation with Gadobutrol-Enhanced MRI</p>

“…This is the second reported case of IVC tumour resection combined with multiple organ auto-transplantation worldwide since 2015. 20 High concentration contrast medium gadobutrol-enhanced MRI with high-resolution T2-weighted black blood sequence showed great accuracy in preoperative diagnosis and tumour extent identification. The findings helped guide a complex, radical surgical operation involving multiple organs that led to the survival of the patient.…”

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

“…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. Despite the frequent use of veno-venous bypass in these reported cases, it seems not to be necessary.…”