Ann Thorac Surg 1998;66:2095-2096
© 1998 The Society of Thoracic Surgeons
Case Reports
Pelvic leiomyoma in the right atrium
Takahiro Katsumata, MDa,
Amihay Shinfeld, MDa,
Rémi Houel, MDa,
Stephen Westaby, FRCSa
a Department of Cardiac Surgery, Oxford Heart Centre, John Radcliffe Hospital, Oxford, United Kingdom
Accepted for publication May 23, 1998.
Address reprint requests to Mr Westaby, Oxford Heart Centre, The John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, United Kingdom
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Abstract
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Intravenous leiomyomatosis is a condition characterized by intravenous growth of histologically benign smooth muscle tumor, originating in the uterus. A case of intravenous leiomyomatosis with right atrial extension in a 64-year-old woman is described. The atrial tumor was successfully removed with a single-stage approach via sternolaparotomy and total circulatory arrest using cardiopulmonary bypass.
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Introduction
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The gynecologist and cardiac surgeon seldom address the same pathology. In this case we describe the combined treatment of a pelvic leiomyoma that entered the right iliac vein, filled the inferior vena cava, and presented in the right atrium.
A 64-year-old white woman was under surveillance after two previous operations for uterine fibroids. Hysterectomy had been performed during the second operation. Computed tomographic scan during follow up showed a 12-cm diameter tumor behind the bladder on the right pelvic wall. In addition, the inferior vena cava was found to be greatly enlarged and distended with tumor. Nuclear magnetic resonance imaging of the chest and abdomen showed the intravenous lesion to extend from the right iliac vein throughout the whole inferior vena cava and to fill the body of the right atrium. The right ureter was dilated through compression by the pelvic tumor. Transesophageal echocardiogram showed prolapse of the lesion into the tricuspid annulus.
A combined operation was planned to remove the pelvic and intravascular lesions. The heart, inferior vena cava, and pelvis were approached through a full length sternolaparotomy carried down to the previous suprapubic incision. The adhesions from previous operations were cleared and the pelvic lesion mobilized toward the midline to expose the infrarenal inferior vena cava, which was expanded to 10 cm in diameter by a rubbery tumor mass within the lumen. Solid but mobile tumor could also be felt filling the body of the right atrium. Cardiopulmonary bypass was then established with ascending aortic arterial return and venous drainage through the right atrial appendage. The systemic temperature was reduced to 22°C, after which the circulation was interrupted and the venous blood drained into the oxygenator. The venous cannula was removed and the right atrium opened down to the inferior vena caval orifice. The intracardiac tumor was secured to prevent embolization. The inferior vena cava was then opened below the renal veins, and the intracaval and iliac vein tumor mobilized. Firm traction delivered the tail of the lesion from the right iliac vein, after which the whole of the intravascular pedicle was delivered through the right atriotomy. The intravascular lesion measured 48 cm long and 10 cm wide at the broadest point (Fig 1). After full rewarming, cardiopulmonary bypass was discontinued uneventfully. The postoperative course was uncomplicated, and the patient was discharged from hospital on the sixth day. The pathologic examination of the specimen showed leiomyoma with no mitotic figures, consistent with benign histology. Clinical and radiologic assessment over a 1.5-year follow up has shown no evidence of tumor recurrence.
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Comment
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In a previous report of cardiac tumors in adults, we determined that more than 50% of lesions in the right atrium are likely to originate from an extracardiac site [1]. The most common of these is the hypernephroma, which enters the inferior vena cava from the renal veins.
It is unusual for a benign pelvic leiomyoma to reach the heart. Scrutiny of the English language literature identified only 28 surgical cases with extension of an intravascular leiomyoma into the heart [2]. Our previous report included one similar patient who also underwent successful removal [1]. In each patient the pathologic findings of an extensive hard rubbery lesion with a grossly dilated inferior vena cava suggested a protracted clinical course. Both patients underwent resection of the pelvic fibroids to prevent recurrence. Intravenous leiomyomatosis most commonly enters by the lumen of the iliac vein, and grows into the inferior vena cava sometimes reaching the right side of the heart. Occasionally the ovarian vein provides an alternative route to the subphrenic segment of the inferior vena cava [2,3].
Most patients were treated successfully with a two-stage approach consisting of sternotomy followed by laparotomy, but in few cases was a single-stage procedure used [4,5]. Profoundly hypothermic total circulatory arrest allows access to the largely retrohepatic intracaval tumor and prevents blood loss. Although these tumors are considered benign, pulmonary deposits might occur [6,7]. The fact that many patients with benign ectopic leiomyoma have undergone previous operations for pelvic myomas (myomectomy or partial hysterectomy) suggests the possibility of intraoperative vascular seeding. Histologically benign deposits have been known to involve the liver and lymph nodes as well as the lungs. Although our literature review failed to explain the lower incidence of ectopic recurrence with single-stage resection under total circulatory arrest, the risk of intraoperative seeding is likely to be reduced by arrested circulation.
Caution must be taken during removal of the tumor. Simply pulling the tumor from the right atrium is not feasible. The site of attachment is located in the pelvic veins, and attempted removal just from the thoracic approach results in either failure of complete retrieval of the tumor or tearing of the vein at the point of attachment. Intraoperative deaths have occurred from massive retroperitoneal hemorrhage after avulsion of the tumor from the cardiac end.
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Acknowledgments
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We thank Mrs Janet Newman for editorial assistance.
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References
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Cleveland D.C., Westaby S., Karp R.B. Treatment of intra-atrial cardiac tumours. JAMA 1983;249:2799-2802.[Abstract/Free Full Text]
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Stegmann T., Garcia-Gallant R., Doring W. Intravascular leiomyomatosis: report of a case and review of the literature. Thorac Cardiovasc Surg 1987;35:157-160.[Medline]
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Gonzalez-Lavin L., Lee H.R., Falk L., et al. Tricuspid valve obstruction due to intravenous leiomyomatosis. Am Heart J 1984;108:1544-1546.[Medline]
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Suginami H., Kaurar R., Ochi H., Matsura S. Intravenous leiomyomatosis with cardiac extension. Ann Thorac Surg 1992;53:139-141.[Abstract]
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Okamoto H., Itoh T., Morita S., Matsuura A., Yasuura K. Intravenous leiomyomatosis extending into the right ventricle: one-stage radical excision during hypothermic circulatory arrest. Thorac Cardiovasc Surg 1994;42:361-363.[Medline]
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Drevelengas A., Kalaitzoglou I., Sichletidis L. Benign pulmonary leiomyomatosis with cyst formation and breast metastasis: case report and literature review. Eur J Radiol 1995;19:121-123.[Medline]
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Abstract
Introduction
