Inferior vena cava leiomyosarcoma is a rare tumor with a variety of symptoms. A 41-year-old woman was admitted with nonspecific epigastric pain. Computed tomography revealed a dense mass between the inferior vena cava and the liver. The patient underwent successful resection of the mass. The pathologic study confirmed leiomyosarcoma. Adjuvant radiation therapy was completed, and after 12 months of follow-up, the patient had no problems. © 2014 SAGE Publications

Basravi, M.

Moazeni-Bistgani, M.

shahrekord university of medical scinces

Case StudyLeiomyosarcoma of the inferior vena cavaMohammad Moazeni-Bistgani1 and Monem Basravi2AbstractInferior vena cava leiomyosarcoma is a rare tumor with a variety of symptoms. A 41-year-old woman was admitted withnonspecific epigastric pain. Computed tomography revealed a dense mass between the inferior vena cava and the liver.The patient underwent successful resection of the mass. The pathologic study confirmed leiomyosarcoma. Adjuvantradiation therapy was completed, and after 12 months of follow-up, the patient had no problems.KeywordsLeiomyosarcoma, Vascular neoplasms, Vena cava, inferiorIntroductionLeiomyosarcomas of vascular origin are the mostcommon primary tumors of the inferior vena cava(IVC).1 Slightly more than 400 cases have beenrepoted.2 This low number emphasizes the importanceof the need to report individual cases. They may presentwith various symptoms. Several techniques are used fordiagnosis, including computed tomography, magneticresonance imaging, ultrasound, and echocardiog-raphy.3 According to their relationship to the hepaticand renal vessels, IVC leiomyosarcomas are classiﬁedanatomically: segment I (below the renal vessels); seg-ment II (renal vessels to retrohepatic IVC), and seg-ment III (suprahepatic IVC). The most eﬀectivetreatment for cases of IVC leiomyosarcoma is radicalen-bloc surgical excision.4Case reportOur patient was a 41-year-old woman with a 2-yearhistory of nonspeciﬁc epigastric pain. Physical examin-ation was normal. Computed tomography showed adense mass between the liver and the IVC withoutmetastases to other organs (Figure 1). A computedtomography-guided core biopsy was not performedbecause of the high risk of severe post-biopsy hemor-rhage. We decided to carry out a laparotomy and resec-tion of the mass. Intraoperatively, we mobilized theright hepatic lobe and the hepatic ﬂexure of thecolon. The tumor was extraluminal and originatedfrom the anterior aspect of the IVC 1 cm above theright renal veins, measuring approximately 5 2.5 cm.We obtained proximal control of the renal vein, aboveand below the mass on the IVC. The tumor was mobi-lized oﬀ the IVC using sharp dissection. We used aSatinsky clamp on the IVC tangentially to separatethe mass. The tumor was easily teased oﬀ the IVCwith well-demarcated gross total resection.Intraoperative assessment of the surgical margins byfrozen section conﬁrmed that a complete R0 resectionhad been achieved. The IVC was reconstructed primar-ily with a running suture under Satinsky clamping, using4/0 polypropylene, and the Satinsky clamp wasremoved. The pathologic study conﬁrmed leiomyosar-coma. The patient’s postoperative convalescence wasuneventful without any complications (Figure 2). Shedid not require anticoagulant treatment becauseshe had undergone primary repair. Postoperatively,she received a course of targeted radiotherapy withtotal dose of 60Gy to the tumor bed. Subsequent clinicalexaminations every 6 months and imaging with com-puted tomography conﬁrmed that she remaineddisease-free one year after treatment.Asian Cardiovascular & Thoracic Annals2016, Vol. 24(1) 72–74 The Author(s) 2014Reprints and permissions:sagepub.co.uk/journalsPermissions.navDOI: 10.1177/0218492314543930aan.sagepub.com1Department of Surgery, Shahrekord University of Medical Sciences,Shahrekord, Iran2Department of Surgery, Shahrekord University of Medical Sciences,Shahrekord, IranCorresponding author:Mohammad Moazeni-Bistgani, Department of Surgery, ShahrekordUniversity of Medical Sciences, Shahrekord, Iran.Email: dr_m_moazeni@yahoo.comDiscussionWhile there is no strong evidence base for the manage-ment of IVC leiomyosarcoma, the treatment of choiceaccording to the available literature is radical en-blocsurgical excision with a view to obtaining negativeresection margins. In a study by Kim and colleagues,5patients with negative margins had a median survival of86 months. The aims of surgical management of thesetumors include maintenance of caval ﬂow, achievementof local tumor control, and prevention of recurrence.A number of techniques have been reported for deal-ing with the IVC following excision, including ligation,primary repair, patching (cavoplasty), and prostheticgraft replacement.6 Renal or hepatic vein involvementby the tumor determines the strategy for vascularreconstruction. Simple ligation is possible after com-plete or subtotal resection of segment I and/or II.Gradual occlusion of the IVC allows the developmentof venous collaterals, so reconstruction of the IVC isnot always required; but after radical curative resectionwith disruption of collaterals, or if only few collateralshave developed before surgery, ligation of the IVC maylead to lower limb edema with signiﬁcant functionalimpairment. In segment II tumors, ligation of theIVC is precarious because impairment of renal venousoutﬂow, arterial hypotension, or proximal venoushypertension might occur. Partial resection of theFigure 2. Computed tomography before the operation.Figure 1. Computed tomography one week postoperatively.Moazeni-Bistgani and Basravi 73IVC followed by primary repair or prosthetic patchangioplasty is rarely suﬃcient to be curable. In thiscase, we experienced a new procedure: resection ofthe tumor was achieved with primary repair afterapplying a Satinsky clamp tangentially on the IVC.Cavoplasty with a synthetic patch in low-ﬂow venoussegments predisposes to thrombosis and embolization.Several cases of postoperative graft obstruction havebeen reported with cavoplasty.7 Making an arterioven-ous ﬁstula is strongly recommended to ensure patencyand eliminate the need for long-term anticoagulationtherapy. Prosthetic graft replacement gives the bestresults, given the length of the missing segment andthe need for strength due to intraabdominal pressure.However, this procedure may increase the risk of pul-monary embolism as well as further graft-related majorcomplications such as graft occlusion, graft-enteric ﬁs-tulas, and sepsis.7AcknowledgmentsThe authors would like to thank the Research andTechnology Department of Shahrekord University ofMedical Sciences for cooperating in reporting this case.FundingThis research received no speciﬁc grant from any fundingagency in the public, commercial, or not-for-proﬁt sectors.Conflict of interest statementNone declared.References1. Laskin WB, Fanburg-Smith JC, Burke AP, Kraszewska E,Fetsch JF and Miettinen M. Leiomyosarcoma of the infer-ior vena cava: clinicopathologic study of 40 cases. Am JSurg Pathol 2010; 34: 873–881.2. Mingoli A, Sapienza P, Brachini G, Tarantino B andCirillo B. Surgical treatment of inferior vena cava leiomyo-sarcoma. J Am Coll Surg 2010; 211: 145–146.3. Ceyhan M, Danaci M, Elmali M and Ozmen Z.Leiomyosarcoma of the inferior vena cava. Diagn IntervRadiol 2007; 13: 140–143.4. Xu TB, Liu WY, Chen G, Wang HZ and Bie P. Primaryleiomyosarcoma of the inferior vena cava: a case report.Ann Biol Clin (Paris) 2013; 71: 338–340.5. Kim JT, Kwon T, Cho Y, Shin S, Lee S and Moon D.Multidisciplinary treatment and long-term outcomes in sixpatients with leiomyosarcoma of the inferior vena cava.J Korean Surg Soc 2012; 82: 101–109.6. Alexander A, Rehders A, Raffel A, Poremba C, KnoefelWT and Eisenberger CF. Leiomyosarcoma of the inferiorvena cava: radical surgery and vascular reconstruction.World J Surg Oncol 2009; 7: 56.7. Bower TC, Nagorney DM, Cherry KJ Jr, et al.Replacement of the inferior vena cava for malignancy:an update. J Vasc Surg 2000; 31: 270–281.74 Asian Cardiovascular & Thoracic Annals 24(1)

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