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Background Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumour with poor prognosis. Surgical resection is the first line of treatment to achieve the best possible outcome. However, precise preoperative evaluation is essential to guide therapeutic decisions. Here, the preoperative evaluation potential of gadobutrol-enhanced magnetic resonance imaging (MRI) was assessed in the management of a 42-year-old patient with a large IVC mass. Methods The patient first underwent enhanced computed tomography (CT), but the relationship between the left renal vein and the mass in the dilated IVC was ambiguous, and it remained unclear whether the right hepatic vein was invaded by the lesion. To make a precise assessment of the tumour, the patient subsequently underwent high-resolution MRI angiography examination combined with high-concentration contrast medium gadobutrol. Results MRI demonstrated the integrity of the right hepatic vein and the left renal vein. Following a multidisciplinary consultation, a complicated surgery including complete resection of the mass, artificial vessel replacement of IVC, total hepatectomy, and bilateral nephrectomy with liver and kidney auto-transplantation was performed successfully. The surgical plan formulated after reviewing the MRI preoperatively was adhered to the course of the surgery. Postoperative CT re-examination showed that the blood flow of the artificial blood vessel and the right hepatic vein was unobstructed. Histopathological examination confirmed the tumour to be a leiomyosarcoma. Conclusion Preoperative imaging diagnosis and assessment have important implications for the surgical planning of IVC leiomyosarcoma. High-resolution MRI angiography examination with high concentration contrast medium gadobutrol may be of particular benefit in IVC tumours.

Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare malignant tumor, accounting for 2% of all LMSs. Less than 400 cases have been reported in literature. Computed tomography (CT) is the most accurate imaging method in assessing the location of the tumor within the IVC and magnetic resonance imaging (MRI) accurately identifies its extent and the potential for surgical resection. We present the case of a patient with inferior vena cava leiomyosarcoma (IVCL), for whom the pathological diagnosis was different from the initially expected one, the tumor appearance on pre-operative imaging mimicking renal cell carcinoma. The intraoperative difficulty of approaching renal hilum and IVC was a factor suggesting the vascular origin of the tumor, which was confirmed at pathological analysis. The extensive defect in the IVC after tumor excision led to the decision of complete transverse suturing of IVC, as significant collateral venous circulation was already present. Because IVCL is a rare disease, there is scarce data regarding the prognosis and treatment options. Long-term survival depends on the extent of the surgery. The need of vascular reconstruction is not always mandatory. Despite high recurrence rates, no consensus regarding adjuvant treatment exists yet. A multidisciplinary approach including surgical oncologists and vascular surgeons is mandatory to achieve the best patient outcomes. Perioperative planning, coordination and adherence to oncological techniques are critical.

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare and fatal disease. Imaging is the main diagnostic method. CT and MRI images of IVC leiomyosarcoma have been published, but ultrasonographic features have been scarcely described, especially with contrast-enhancement. We report the case of a patient in whom ultrasonography incidentally revealed a mass in the IVC. Contrast-enhanced ultrasonography showed heterogeneous enhancement in the arterial phase with some irregular non-enhanced areas, and mild clearance in the venous phase. The location and nature of the lesion as shown by ultrasonography were confirmed by CT and MRI.

Patient: Male, 42 Final Diagnosis: IVC leiomyosarcoma with multiple liver metastases Symptoms: Abdominal pain Medication: — Clinical Procedure: IVC resection with hepatectomy Specialty: Surgery Objective: Unusual or unexpected effect of treatment Background: Leiomyosarcoma of inferior vena cava (IVC), a rarely encountered malignancy originating from the smooth muscle cells of media of the IVC, frequently metastasize to the liver. The suggested treatment of choice of IVC leiomyosarcoma is radical en-bloc excision aimed to obtain a negative resection margin. There are a few reported cases of surgical management in patients with liver metastasis from IVC leiomyosarcoma. Case Report: This report describes a simultaneous surgical approach for a case of IVC leiomyosarcoma with multiple liver metastases followed by chemotherapy. Conclusions: Tumor volume reduction surgery of metastatic lesions combined with radical resection of the primary tumor may provide better survival benefit in patients with advanced IVC leiomyosarcoma.

Leiomyosarcomas originating from the inferior vena cava are very rare malignant tumors with an extremely poor prognosis. We report FDG PET/CT findings of pathology-proven hepatic metastases from leiomyosarcoma originating from the inferior vena cava in a young woman whose initial presentation was worsening abdominal and chest pain.

International audience; Inferior vena cava leiomyosarcoma (IVCL) is a rare tumor with a poor prognosis, and its surgical resection remains a challenge. To date, surgery is the only potentially curative treatment for IVCL with a 5-year survival rate of 55%. The main challenge is to combine oncological surgery with clear margins and vascular reconstruction of the inferior vena cava (IVC). In this review, we discuss the different approaches to vascular reconstruction after IVCL resection, using a prosthetic or autologous patch, direct suture or simple ligation without IVC reconstruction. The reconstruction of IVC depends of tumor location and its extension. We recommend no reconstruction if venous collaterality is well-established. When vascular reconstruction is required, we prefer prosthetic PTFE graft. These patients should be referred to high-volume centers with a multidisciplinary team of sarcoma surgeons with cardiothoracic, vascular and hepatic specialties.

BackgroundThe en bloc resection of inferior vena cava (IVC) leiomyosarcoma often necessitates IVC reconstruction. The objective of this study is to examine outcomes after IVC reconstruction and determine optimal graft sizing.MethodsA retrospective review was conducted of all IVC reconstructions after IVC leiomyosarcoma resection at a single institution. Cross-sectional dimensions at the IVC resection margins were measured on preoperative imaging. The tumor location was based on the most superiorly involved region of the IVC and was classified as infrarenal, between hepatic and renal veins, or superior to the hepatic veins. Perioperative details and long-term outcomes including graft sizing, graft patency, morbidity, and mortality were recorded.ResultsBetween 2007 and 2017, 12 patients (6 females, mean age: 64.5years, age range: 46-80years) underwent IVC leiomyosarcoma resection and reconstruction. All reconstructions were performed with ringed polytetrafluoroethylene (PTFE); graft sizes ranged from 12mm to 16mm. The tumor location was exclusively infrarenal in seven patients, between the renal and hepatic veins in two patients, and involved multiple segments in three patients. Larger graft sizes were utilized in reconstructing more superior segments of the IVC. Grafts were typically undersized and based on the diameter of the superior resection margin with 12mm grafts approximately correlating to a 20mm diameter, 14mm to 25mm, and 16mm to 30mm. The average undersizing ratio was 0.6. At a mean follow-up time of 43 ± 27months, radiographic graft patency was 92%, overall survival was 83%, and disease-free survival was 25%.ConclusionsAfter en bloc resection of IVC leiomyosarcoma, caval reconstruction with an undersized ringed PTFE has acceptable patency. Grafts sizes should be based on the IVC diameter superior to the tumor and undersizing by approximately 40% appears to be associated with acceptable patency rates. Further multiinstitutional studies should be performed to best determine the optimal treatment of this rarely encountered tumor.

Primary vascular tumors such as vascular leiomyosarcomas are rare, but exhibit markedly different characteristics than tumors that invade the vasculature from a secondary source. Establishing a diagnosis is essential in determining the appropriate treatment plan, but obtaining a histologic specimen may prove challenging and carry significant risks. Minimally invasive endovascular biopsy techniques can be pivotal in the diagnosis-and thus in the management-of vascular tumors. We present a case of a primary inferior vena cava leiomyosarcoma, not able to be adequately assessed by noninvasive imaging and deemed too risky to be approached with traditional percutaneous biopsy techniques. Accurate diagnosis of such tumors is critical, as the success of surgical resection, although high risk, depends greatly upon the type, location, and extent of malignancy.

Primary leiomyosarcomas of the inferior vena cava (IVC) are rare sarcomas, none of which have been described in literature during a third-trimester pregnancy. Here, we describe the complex care of a patient at 30 weeks of gestation who presented to her obstetrician with shortness of breath and lower extremity swelling. She was found to have a 5.0 × 5.0 × 13 cm heterogeneous mass of her IVC, ultimately diagnosed as a leiomyosarcoma. She underwent a cesarean delivery under combined spinal epidural and a subsequent tumor resection and IVC reconstruction requiring multidisciplinary surgical and anesthetic care.

Introduction Leiomyosarcoma of the Inferior Vena Cava (IVC) is a rare smooth muscle neoplasm typically presenting in the fifth to sixth decades of life with both intraluminal and extraluminal growth patterns. Surgical resection remains the gold standard for non-metastatic disease and often requires vascular reconstruction. We present an atypical case of leiomyosarcoma involving both the IVC and infrarenal abdominal aorta necessitating reconstruction with intraoperative veno-venous bypass. Methods A 63-year-old man initially presenting with back pain was found to have a large mass adjacent to the IVC on MRI, subsequently confirmed to be leiomyosarcoma by biopsy. After six months of neo-adjuvant chemotherapy the patient was taken for resection. However, intra-operatively the tumor was found to involve the aorta necessitating combined aorto-caval reconstruction. To facilitate en-bloc resection of the tumor, the aorta was reconstructed first followed by the inferior vena cava using veno-venous bypass. Results Post-operatively, the patient was taken to the intensive care unit for resuscitation and had an uncomplicated hospital course. He was discharged to rehab six days post-operatively and at one year remains free of significant tumor burden with patent aorto-caval bypass grafts. Conclusion Primary leiomyosarcoma of the IVC is estimated to have aortic involvement in

Primarni tumory srdce a dolni dute žily jsou poměrně vzacne. Incidence primarnich tumorů srdce je 0,001-0,030 %, zatimco u metastazujicich tumorů je to 20krat až 40krat vice. Z celkoveho poctu zaznamenaných primarnich tumorů je jen 10 % malignich. U dolni dute žily je situace podobna, i zde je větsina tumorů pouze sekundarnich.Předkladana kasuistika popisuje připad 70leteho muže s cerstvou anamnezou pobytu v rovnikove Africe přijateho pro dusnost a srdecni selhani, jemuž byla diagnostikovana expanze v oblasti prave sině a dolni dute žily. Pro podezřeni na trombus byla zapocata antikoagulacni lecba, nicmeně vzhledem k dalsimu zhorseni pacientova stavu předevsim v důsledku pravostranneho srdecniho selhani byla provedena chirurgicka excize tumorozni hmoty. Navzdory uspěsnemu odstraněni tumoru vsak pacient na nasledky pravostranneho srdecniho selhani zemřel.V diskusi jsou rozebrany na zakladě soudobe literatury cetnosti výskytu, možnosti lecby a prognozy uvedene diagnozy.

Leiomyosarcoma (LMS) of primary vascular origin is a rare entity with only potentially curative option being complete surgical resection; despite which the prognosis remains dismal. Tumour recurrence is very common, and the benefits of adjuvant therapy are undefined. A 39-year-old woman presented with 6 months’ history of abdominal pain, abdominal distension and pedal oedema. On evaluation, she was diagnosed to have chronic Budd-Chiari syndrome (BCS) secondary to a tumour arising from the inferior vena cava (IVC) on evaluation. Her liver decompensation included jaundice, gastrointestinal bleed and ascites. Following a detailed multidisciplinary team discussion, she underwent complete excision of the tumour along with a segment of the IVC with living donor liver transplantation. She remains disease-free 24 months following surgery. This is the first reported case of liver transplantation for IVC LMS causing chronic BCS.

Background Primary leiomyosarcomas of the inferior vena cava (IVC) pose unique surgical challenges. Due to the rarity of the disease, little definitive data exists on prognosis and treatment options. Methods A pooled data analysis was performed on all cases of initial IVC leiomyosarcoma resection identified by literature search ( n = 371) and our institutional database ( n = 6). Kaplan–Meier and Cox regression analyses were performed to identify factors associated with disease-free survival (DFS) and overall survival (OS). Results Patients were predominantly female (76%, n = 286); the median age of presentation was 55 years. Five-year DFS and OS were 6% and 55%, respectively. Preoperative factors independently associated with decreased OS included older age (HR:1.05, 95% CI:1.00–1.09), larger tumor size (HR:1.14, 95% CI:1.04–1.24), resection of adjacent organ(s) (HR:3.62, 95% CI:1.34–9.77), and R2 resection (HR:7.80, 95% CI:1.94–32.05). Isolated involvement of the suprarenal infrahepatic IVC was associated with longer OS (HR:0.22, 95% CI:0.06–0.78). A scoring system incorporating independent predictors of OS stratified outcomes: score 4–5 ( n = 10, median OS 6 months), score 2–3 ( n = 88, median OS 23 months) compared to a score of 0–1 ( n = 44, median OS 29 months). Conclusions Following resection of IVC leiomyosarcomas, recurrence is a near certainty; long-term survival, however is possible. The dominant predictors of survival include margin status, tumor size and radical resection. These can be combined into a risk score that has prognostic value.

Objective This retrospective study aimed to show the practice of preserving renal function during curative resection of inferior vena cava leiomyosarcoma (IVCL) involving the renal veins at a single institution over a 7 year period. Materials and methods From February 2009 to February 2017, 10 patients (6 women; median age 49 years) with IVCL involving the renal veins were treated surgically at Peking Union Medical College Hospital. En bloc resections were performed in all patients, and the renal outflows were preserved in eight patients using a new method: venoplasty of the renal ostia (VRO). Data regarding patient details, pre-operative preparation, surgical procedures, post-operative recovery, and follow-up results were obtained and reviewed retrospectively. Results Computed tomography and intra-operative examinations revealed that renal vein confluences were involved but not invaded in all cases except Patient 4. All patients underwent curative en bloc tumour excision; a right nephrectomy was performed in only one patient (Patient 4) whose tumour invaded the right renal vein. The mean operation time was 358 min and the mean blood loss 1935 mL. At a median follow-up of 54.5 months, the 5 year local recurrence, distant metastasis, overall survival, and disease-free survival rates were 20%, 10%, 68.6%, and 38.1%, respectively. Conclusions Venoplasty of the renal ostia is an effective method of preserving the renal veins and reconstructing renal outflow.

Objectives Primary tumors of the inferior vena cava are rare, with leiomyosarcoma representing the vast majority. Method A 60-year-old man was admitted in emergency for fainting and mild anemia. A whole-body computed tomography revealed a retroperitoneal mass of approximately 8 cm in diameter, invading the lumen of the inferior vena cava, extending to the renal vein confluence. An en bloc resection of the solid mass was performed. Macroscopically the tumor did not seem to insist on the resection margin. Results Histopathological examination confirmed the diagnosis of leiomyosarcoma of the inferior vena cava. Postoperative recovery was uneventful and the patient was discharged after eight days, starting adjuvant chemotherapy. During the follow-up, the patient did not show other fainting episode, and at 24 months he is disease free. Conclusions: Unusually, fainting could even be the isolated sign of a large leiomyosarcoma of the inferior vena cava, also when it affects its middle portion.

Inferior Vena Cava (IVC) leiomyosarcoma (LM) is a rare malignancy of mesenchymal origin with an incidence of 1/100,000. We present an 82-year-old lady with a past history of open cholecystectomy who presented with a large indeterminate mass on abdominal imaging. Open resection of the mass was performed and histology with immunohistochemical staining revealed leiomyosarcoma. She received adjuvant radiotherapy and remained disease free 4 years after.

Introduction. Leiomyosarcomas of the inferior vena cava are rare malignant tumors. A limited number of these cases have been described so far. Only few of them have intracardiac propagation and surgery is rarely undertaken for their treatment. Case Outline. We present a 52-year-old female patient in whom leiomyosarcoma of the inferior vena cava with intracardiac propagation was diagnosed. The patient underwent successful surgical treatment with complete removal of the tumor and direct suture of the inferior vena cava. No additional modalities of therapy were undertaken. Conclusion. Surgery, without radiation therapy can be a successful option for the treatment of inferior vena cava leiomyosarcoma with a good short-term result.

Inferior vena kava (IVK) leiomyosarkomu, nadir gorulen bir hastaliktir. Bu nadir tumorun en iyi tedavisi, tumorun tamamen cerrahi rezeksiyonu ve dolayisiyla venoz rekonstruksiyon ihtiyaci doguran IVK’nin rezeksiyonudur. Rezeksiyon sonrasi IVK’nin baglanmasi veya primer onarim, yama ile onarim veya sentetik ya da biyolojik materyallerle tup greft konulmasi gibi farkli rekonstruksiyon yontemleri mevcuttur. Inferior vena kava leiomyosarkomlarina iliskin mevcut bilgiler olgu sunumlari ve kucuk olgu serilerine ait oldugu icin, olgu sunumlarinin bildirilmesi onemlidir. Bu yazida, IVK leiomyosarkomu olan ve Dacron tup greft ile rekonstruksiyon uygulanan 69 yasinda kadin bir olgu, IVK leiomyosarkomalarinin tanidan tedaviye klinik ozellikleri, literatur verileri esliginde incelenerek sunuldu. Anah tar soz cuk ler: Inferior vena kava; leiomyosarkom; vaskuler tumor. Inferior vena cava (IVC) leiomyosarcoma is a rare clinical entity. The best treatment for this rare tumor is complete surgical resection of the tumor and consequently resection of IVC leading to the need for venous reconstruction. Ligation of IVC after resection or reconstruction including primary repair, patch repair or tube grafting with synthetic or biological materials are available. As the current knowledge of IVC sarcomas is based primarily on case reports and small case series, it is of utmost importance to report individual cases. In this article, we report a 69-yearold female with IVC leiomyosarcoma who underwent reconstruction with a Dacron tube graft in the light of clinical characteristics of the disease from diagnosis to treatment along with a detailed review of the literature data.

Inferior vena cava leiomyosarcoma is a very rare tumor, accounting for only 0.5% of all soft tissue sarcomas. As the other leyomiosarcomas of vascular origin, they have a poor prognosis, and radical resection with surgical margins free of tumor is the only potentially curative treatment. We present a case of a 46 year-old woman with metastatic inferior vena cava leiomyosarcoma who progressed after anthracyclines and ifosfamide and achieved a complete and sustained response with trabectedin. Beyond progression, the patient started third line treatment with pazopanib. A brief review of literature is also given. This case supports the efectiveness of a recent therapeutic agent, with an impressive progression-free survival in a recurrent metastatic inferior vena cava leiomyosarcoma.

Leiomyosarcoma of the vascular origin is a rare malignant tumor. It originates from the smooth muscle cells of the media with intra- or extraluminal growth, and in most cases it arises in the inferior vena cava. The diagnosis is often delayed because the clinical symptoms of this disease are often nonspecific. Accurate diagnosis of inferior vena cava leiomyosarcoma (IVCLMS) needs histologic confirmation. We report a case of IVCLMS histologically confirmed by aspiration biopsy with a catheter during digital subtraction angiography presenting with pulmonary emboli in a 65-year-old man.

Leiomyosarcoma (LMS) of inferior vena cava is a rare and aggressive tumor, arising from the smooth muscle cells in the vessel wall. A large complete surgical resection is the essential treatment. The need of vascular reconstruction is not always mandatory. It’s above all to understand the place of the reconstruction with artificial vascular patch prosthetics of vena cave after a large resection of the tumor. We rapport two cases of LMS of inferior vena cava in two women who underwent successful large resection of tumor and lower segment of inferior vena cava. In first case, reconstruction of the inferior vena cava was not performed because of the development of venous collaterals derivation. In the second case reconstruction was done using Dacron interposition graft. The necessity of a large resection in management of primary leiomyosarcoma of vena cave makes sometimes unavoidable the sacrifice of a portion of the vena. Indeed, a better comprehension of the development of venous derivation may render unnecessary the reconstruction.The Pan African Medical Journal. 2016;24

We report a rare case of leiomyosarcoma of the inferior vena cava (IVC) in which F-18 fluorodeoxyglucose (F-18 FDG) positron emission tomography-computed tomography (PET-CT) scan provided vital evidence, which led to its diagnosis, in a background of procoagulant state of the patient, where previous ultrasound-Doppler and echocardiography studies were nonspecific and revealed bilateral lower limb deep vein thrombosis with thrombus in IVC. The whole body F-18 FDG PET-CT scan was done in view of no significant improvement in clinical status of the patient over few months in spite of appropriate medical management. FDG PET-CT scan revealed high grade uptake in a large mass lesion occupying the right atrium, extending superiorly into terminal superior vena cava, inferiorly into dilated IVC and probably into hepatic veins. CT guided biopsy of this F-18 FDG avid mass was consistent with the diagnosis of leiomyosarcoma, which however was not amenable to surgery at this stage. F-18 FDG PET-CT accurately differentiated tumor mass from bland thrombus and further had a significant impact on the management, since aggressive surgery combined with adjuvant therapy offers the best outcome for patients with leiomyosarcoma of the IVC.

Background and Objectives Inferior vena cava (IVC) leiomyosarcomas are rare and are a relatively small subset of retroperitoneal sarcomas. The current approach is resection and ligation or reconstruction of the IVC. This study was undertaken to analyze the outcomes associated with the use of neoadjuvant radiotherapy and IVC reconstruction in the treatment of IVC leiomyosarcoma. Methods A retrospective clinicopathological review of patients treated during a 10-year period. Results Four patients were treated with neoadjuvant radiotherapy, median 47.5 Gy, all underwent margin negative resection with 75% of the tumors being high grade and all patients requiring resection of adjacent organs. Reconstruction of the IVC was performed with an autologous superficial femoral vein graft. There were no mortalities and the morbidity rate was 50%. At a median follow up of 37 months; two patients had a patent IVC, no patients had a local recurrence, and one patient developed a distant metastases treated successfully with metastectomy. Conclusions Neoadjuvant radiotherapy and resection of the IVC leiomyosarcoma resulted in 100% local control, and all patients are alive at median follow up of 37 months. IVC reconstruction with the superficial femoral vein is safe and associated with acceptable short and long term morbidity. J. Surg. Oncol. 2011; 103:175–178. © 2010 Wiley-Liss, Inc.

e23556Background: Vascular leiomyosarcomas (LMS) are rare tumors that often arise from the inferior vena cava (IVC). Despite several surgical case series in the literature, there remains little dat...

Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is exceedingly rare. The role of adjuvant therapy remains undefined. This study evaluated outcomes after aggressive management. Records on 20 patients undergoing surgery for IVC LMS between January 1990 and April 2006 were retrieved. Histology was confirmed upon re-review. Most patients received perioperative chemotherapy (CT), radiation therapy (RT), or both (CRT). Disease-free and overall survival (DFS, OS) rates were calculated using the Kaplan-Meier method. Twenty patients (60% women, median age 57 years) with primary IVC LMS were treated with curative intent. Median follow-up was 41 months. All patients underwent resection of the primary tumor; one was found to have unresectable liver metastases. The IVC was managed with ligation (3), primary repair (12), or prosthetic graft (5). Additional organs were resected in 14 (70%) patients. Chemotherapy and/or RT were administered to 9 (45%) patients preoperatively (CT 2, RT 6, CRT 1) and 8 (40%) postoperatively (CT 4, RT 1, CRT 3). Median DFS was 21 months. Of 13 (68%) patients who developed recurrence, 4 underwent surgery, and 11 received CT. Median OS for 19 patients who underwent complete resection was 71 months. Tumor size was associated with disease recurrence (P = .004). No variables were prognostic for OS. Patients with IVC LMS treated with curative intent develop early recurrent disease. Nevertheless, long-term OS can be achieved even in the setting of metastatic disease. The independent impact of perioperative CT, RT, or CRT treatments cannot be adequately determined.

Inferior vena cava (IVC) leiomyosarcoma is a rare malignant neoplasm more commonly seen in women in the fifth to sixth decade of life. Complete resection of the tumor with negative margins is the only therapeutical option that has demonstrated a survival benefit. This report presents a case of a 67-year-old woman affected by a lower segment IVC leiomyosarcoma incidentally detected during a chronic abdominal pain study. The patient was treated with tumorectomy, resection and ligation of the infrarenal IVC without signs or recurrence on a 12-month follow-up.

Inferior vena cava (IVC) leiomyosarcoma represents an extremely rare disease that commonly involves the segment between the inflow of the renal veins and the inflow of the hepatic veins (46% of cases). We report the case of a patient affected by an IVC leiomyosarcoma, treated with surgical resection, caval reconstruction with polytetrafluoroethylene (PTFE), and right nephrectomy, followed by external beam radiotherapy. Oncological follow-up was negative for 17 years after this combined treatment, since the patient developed a duodenocaval fistula (DCF).

Background. Leiomyosarcoma of vascular origin is a rare tumor, occurring mainly in the inferior vena cava (IVC). When involving the hepatic vein confluence, it often causes Budd–Chiari syndrome, and IVC removal with a complex hepatectomy is required (Mingoli in J Am Coll Surg 211:145–146, 2010; Griffin in J Surg Oncol 34:53–60, 1987; Heaney in Ann Surg 163:237–241, 1966; Fortner in Ann Surg 180:644–652, 1974). Methods. A 57-year-old male, without previous oncological history, presented with Budd–Chiari syndrome due to a leiomyosarcoma extending to the supra-diaphragmatic IVC and involving the right and middle hepatic veins. The patient did not receive neoadjuvant treatment. Results. A femoral to superior vena cava veno-venous bypass was inserted, and both a median sternotomy and phreno-laparotomy with right subcostal extension were performed. A hemi-portocaval shunt was created between the right portal branch and the IVC, while a catheter was connected to the left portal branch for cold perfusion. Under extracorporeal circulation, the IVC was sectioned after infrahepatic and supra-diaphragmatic cross-clamping. The left liver was flushed with Celsior solution and packed with ice. A right trisectionectomy extended to the caudate lobe with en bloc vena cava removal was performed. The IVC was replaced by a cryopreserved aortic homograft, to which the stump of the left hepatic vein was anastomosed. Bypass duration, warm and cold liver ischemia, and operation time were 280 min, 8 min, 112 min, and 11 h, respectively. Duct-to-duct biliary anastomosis tutored by aT-tube was performed, and the patient was discharged on postoperative day 29, without major complications. After 16 months free of disease, the patient developed bilateral lung metastases. After 4 years the patient is still alive and receiving systemic chemotherapy. Conclusions. Leiomyosarcoma of the IVC involving the hepatic veins can be treated with extended hepatectomy and removal of the IVC through extracorporeal circulation.

Background The inferior vena cava (IVC) is a rare site for primary soft tissue sarcoma. There are limited data in the literature regarding surgical management of the IVC and longterm survival of these patients. Study design From 1982 to 2002, a total of 25 patients with primary IVC leiomyosarcoma was treated as inpatients and followed in a prospective database at Memorial Sloan-Kettering. Presenting symptoms, tumor characteristics, operative management, postoperative morbidity, and disease-specific survival were assessed for each patient. Results The 25 patients with primary IVC leiomyosarcoma accounted for 0.5% of all adult patients with soft tissue sarcoma treated during this time. The median patient age was 56 years (range 41 to 79 years). The three most common presenting symptoms were abdominal pain (52%), distention (20%), and deep venous thrombosis (12%). Of the patients, 21 (84%) underwent complete resection of the tumor. The IVC was managed in one of three ways: ligation (n = 11), primary/patch repair (n = 8), and expanded polytetrafluoroethylene tube grafting (n = 2). Among patients undergoing IVC ligation and primary/patch repair (n = 19), 11% had severe postoperative edema and none had worsening renal function. Local recurrence occurred in 33% of patients and distant recurrence occurred in 48% of patients. Patients undergoing complete resection had 3-year and 5-year disease-specific survival rates of 76% and 33%, respectively. There were no 3-year survivors among patients with incomplete resections. Conclusions Complete resection of primary IVC leiomyosarcomas is feasible and associated with improved survival. The IVC can be managed by primary repair or ligation with a low risk of severe postoperative edema.

El leiomiosarcoma de la vena cava es un tumor vascular maligno de presentacion rara que tiene mal pronostico. Habitualmente se presenta en mujeres entre la quinta y sexta decadas de la vida. Es una lesion de bajo grado de malignidad pero habitualmente fatal. En este articulo se revisan dos casos clinicos de leiomiosarcomas de la vena cava inferior de localizacion pararrenal. Ambos ocurrieron en mujeres y la reseccion radical fue la forma de manejo. En un paciente se utilizo pericardio bovino y en el otro un injerto de politetra-fluoroetileno como reemplazo de la vena cava. Ambos pacientes presentaron recurrencias locales y sistemicas. Una fallecio 5 anos despues del diagnostico con recurrencia local y sistemica, la otra se encuentra viva pero con recurrencia local y hepatica.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor of mesenchymal origin, occurring most frequently in middle-aged women. Insidious complaints delay diagnosis, prognosis is poor, and the only curative modality remains an aggressive surgical resection yielding clear margins of disease. Commonly, radical tumor excision mandates caval repair or reconstruction, with significant related morbidity and mortality. We present a case of a 50-year-old woman with a leiomyosarcoma arising from the lower segment of the IVC, managed by surgical en-bloc resection of the tumor and IVC segment without further caval repair or reconstruction. During 14 months of follow-up the patient is well, had not had any complications, and is disease free.