endoscopic tumor resection of the inferior vena cava

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Endoscopic tumor resection of the inferior vena cava H. Jeanmart, MD, a P. Lecompte, MD, b F. Casselman, MD, PhD, FETCS, a J. Coddens, MD, b G. Van Vaerenberg, a and H. Vanermen, MD, a Aalst, Belgium Preoperative History A 51-year-old woman was referred for evaluation of an asymptomatic systolic murmur. Transthoracic echo- cardiography showed a large right atrial (RA) mass with intermittent prolapse through the tricuspid valve and extending into the pulmonary artery. On the preoperative transesophageal echocardiographic examination, a tumor with multiple strands was seen. One strand intermittently obstructed the outflow of the hepatic vein (Figure 1). There was apparent ob- struction of the RA inflow and right ventricular inflow and outflow tracts by another strand. Operative and Postoperative Period The resection was performed with a minimally invasive endoscopic approach through a small thoracotomy on the right side. After induc- tion of deep hypothermia and circulatory arrest, the RA was opened, and the mass was immediately seen coming out of the inferior vena cava (IVC; Figure 2, A). The mass could easily be removed form the ventricle by means of simple traction. One large zone of insertion was seen inside the IVC and extending until the RA. After resection, a second mass was seen, originating even deeper in the IVC, below the origin of the subhepatic veins (Figure 2, B). The complete specimen was more than 15 cm long and seemed intact on macroscopic examination. Small tears on the tricuspid valve were primarily repaired, but after coming off pump, a major tricuspid insufficiency was seen, and the valve had to be replaced. The final pathology of the tumor was a leiomyoma. The postoperative period was without compli- cations, and the patient left the hospital after 8 days. Discussion Intravenous leiomyoma is a rare benign vascular tumor, most commonly found in the pelvic veins, of unclear cause that develops exclusively in women, many of whom, as in the case of our patient, have undergone a hyster- ectomy in the past for uterine leiomyoma. 1 Some authors believe that the tumor results form the vascular invasion from a uterine leiomyoma, but it has also been proposed that the tumor is vascular in origin and is derived from the medial smooth muscle cells of a vessel wall. 2 The peripheral fibers of the tumor are compressed into a pseudocapsule with a white glistening appearance and a charac- teristic swirling pattern on the surface. A characteristic of benign leiomyomas is that they grow freely within the vessel lumen but do not invade the wall. Histologic analysis of resected specimens reveals benign smooth muscle architecture without hemorrhage or necrosis. 1 When right-heart involvement is present, the patient presents with typical right-heart failure symptoms. Management of an in- travenous leiomyoma requires surgical excision of the tumor to relieve symptoms and prevent embolism and sudden death. Biopsy of the tumor is generally not indicated because resection of the tumor is indicated, regardless of histologic findings. Few data are available regarding the natural history or the risk of malignant transformation. Although benign in nature, local recurrence has been reported after surgical resection and lung metastasis also. 3 From the Departments of Cardiovascular and Thoracic Surgery a and Anesthesiology, b OLV Clinic, Aalst, Belgium. Received for publication March 28, 2006; accepted for publication April 10, 2006. Address for reprints: F. P. Casselman, MD, PhD, FETCS, OLV Clinic, Department of Cardiovascular and Thoracic Surgery, Moorselbaan 164, Aalst, B-9300, Belgium (E-mail: fi[email protected]). J Thorac Cardiovasc Surg 2006;132:687-8 0022-5223/$32.00 Copyright © 2006 by The American Association for Thoracic Surgery doi:10.1016/j.jtcvs.2006.04.029 Figure 1. On the transgastric view, the mass can be seen coming from the right atrium and going across the pulmonary valve, into the pulmonary artery. RA, right atrium; LA, left atrium; RV, right ventricle; PA, pulmonary artery. Drs Casselman and Vanermen Brief Communications The Journal of Thoracic and Cardiovascular Surgery Volume 132, Number 3 687

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Page 1: Endoscopic tumor resection of the inferior vena cava

Endoscopic tumor resection of the inferior venacavaH. Jeanmart, MD,a P. Lecompte, MD,b F. Casselman, MD, PhD, FETCS,a

J. Coddens, MD,b G. Van Vaerenberg,a and H. Vanermen, MD,a Aalst, Belgium

Preoperative History

A51-year-old woman was referred for evaluation of anasymptomatic systolic murmur. Transthoracic echo-cardiography showed a large right atrial (RA) masswith intermittent prolapse through the tricuspid valve

and extending into the pulmonary artery. On the preoperativetransesophageal echocardiographic examination, a tumor withmultiple strands was seen. One strand intermittently obstructed theoutflow of the hepatic vein (Figure 1). There was apparent ob-struction of the RA inflow and right ventricular inflow and outflowtracts by another strand.

Operative and Postoperative PeriodThe resection was performed with a minimally invasive endoscopicapproach through a small thoracotomy on the right side. After induc-tion of deep hypothermia and circulatory arrest, the RA was opened,and the mass was immediately seen coming out of the inferior venacava (IVC; Figure 2, A). The mass could easily be removed formthe ventricle by means of simple traction. One large zone ofinsertion was seen inside the IVC and extending until the RA.After resection, a second mass was seen, originating even deeperin the IVC, below the origin of the subhepatic veins (Figure 2, B).The complete specimen was more than 15 cm long and seemedintact on macroscopic examination.

Small tears on the tricuspid valve were primarily repaired, butafter coming off pump, a major tricuspid insufficiency was seen,and the valve had to be replaced. The final pathology of the tumorwas a leiomyoma. The postoperative period was without compli-cations, and the patient left the hospital after 8 days.

DiscussionIntravenous leiomyoma is a rare benign vascular tumor, mostcommonly found in the pelvic veins, of unclear cause that developsexclusively in women, many of whom, as in the case of our patient,

have undergone a hyster-ectomy in the past foruterine leiomyoma.1 Someauthors believe that the tumor results form the vascular invasionfrom a uterine leiomyoma, but it has also been proposed that thetumor is vascular in origin and is derived from the medial smoothmuscle cells of a vessel wall.2

The peripheral fibers of the tumor are compressed into apseudocapsule with a white glistening appearance and a charac-teristic swirling pattern on the surface. A characteristic of benignleiomyomas is that they grow freely within the vessel lumen but donot invade the wall. Histologic analysis of resected specimensreveals benign smooth muscle architecture without hemorrhage ornecrosis.1

When right-heart involvement is present, the patient presentswith typical right-heart failure symptoms. Management of an in-travenous leiomyoma requires surgical excision of the tumor torelieve symptoms and prevent embolism and sudden death. Biopsyof the tumor is generally not indicated because resection of thetumor is indicated, regardless of histologic findings. Few data areavailable regarding the natural history or the risk of malignanttransformation. Although benign in nature, local recurrence hasbeen reported after surgical resection and lung metastasis also.3

From the Departments of Cardiovascular and Thoracic Surgerya andAnesthesiology,b OLV Clinic, Aalst, Belgium.

Received for publication March 28, 2006; accepted for publication April 10,2006.

Address for reprints: F. P. Casselman, MD, PhD, FETCS, OLV Clinic,Department of Cardiovascular and Thoracic Surgery, Moorselbaan 164,Aalst, B-9300, Belgium (E-mail: [email protected]).

J Thorac Cardiovasc Surg 2006;132:687-8

0022-5223/$32.00

Copyright © 2006 by The American Association for Thoracic Surgery

doi:10.1016/j.jtcvs.2006.04.029

Figure 1. On the transgastric view, the mass can be seen comingfrom the right atrium and going across the pulmonary valve, intothe pulmonary artery. RA, right atrium; LA, left atrium; RV, rightventricle; PA, pulmonary artery.

Drs Casselman and Vanermen

Brief Communications

The Journal of Thoracic and Cardiovascular Surgery ● Volume 132, Number 3 687

Page 2: Endoscopic tumor resection of the inferior vena cava

Mandelbaum and colleagues4 described, in 1974, the firstsuccessful resection of a leiomyoma in the right heart, whichoriginated in the IVC just below the RA. Various surgicalapproaches have been used for the removal of the tumor. If thetumor is too extensive or adheres to the cardiac and vascularstructures, requiring resection of the abdominopelvic and in-trathoracic components, then a separate operation might bemandatory. Otherwise, 1-stage resection during circulatory ar-rest and hypothermia can be used with success.4 The casereported here is the first case of an IVC tumor removal with anendoscopic approach.

References1. Harris LM, Karakousis CP. Intravenous leiomymatosis with cardiac

extension: tumor thrombectomy through an abdominal approach. J VascSurg. 2000;31:1046-51.

2. Norris HJ, Parmley T. Mesenchymal tumors of the uterus v. intravenousleiomyomatosis. Cancer. 1975;36:2164-78.

3. Mulvany NJ, Slavin JL, Ostor AG, Fortune DW. Intravenous leiomyo-matosis of the uterus: a clinicopathologic study of 22 cases. Int JGynecol Pathol. 1994;13:1-9.

4. Mandelbaum I, Pauletto FJ, Nasser WK. Resection of a leiomyoma ofthe inferior vena cava that produced tricuspid valvular obstruction.J Thorac Cardiovasc Surg. 1974;67:561-7.

Figure 2. A, Right atrial masspassing through the tricuspidvalve. B, Point of insertion ofthe second mass, deep insidethe inferior vena cava. (Thefirst zone of resection can beseen on the left side of theopening of the vena cava.)

Brief Communications

688 The Journal of Thoracic and Cardiovascular Surgery ● September 2006