1998;14:635-638 Eur J Cardiothorac SurgA. Kornberg, S.M. Wildhirt, E. Kreuzer and B. Reichart

young female patientAsymptomatic right atrial leiomyosarcoma with tricuspid valve obstruction in a

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ISSN: 1010-7940. European Association for Cardio-Thoracic Surgery. Published by Elsevier. All rights reserved. Printfor Cardio-thoracic Surgery and the European Society of Thoracic Surgeons. Copyright © 1998 by The European Journal of Cardio-thoracic Surgery is the official Journal of the European Association

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Case report

Asymptomatic right atrial leiomyosarcoma with tricuspid valve obstructionin a young female patient

A. Kornberg*, S.M. Wildhirt, E. Kreuzer, B. Reichart

Department of Cardiac Surgery, Ludwig-Maximilian-University, Munich, Klinikum Großhadern, Munich, Germany

Received 7 May 1998; revised version received 9 September 1998; accepted 29 September 1998

Abstract

Primary cardiac leiomyosarcomas of the heart are rare tumors usually diagnosed post-mortem. Like other sarcomas located in the heart, itcauses symptoms by obstruction or occlusion of cardiac cavities, local invasion, embolization or by systemic manifestations. We present anunusual case of a previously healthy young female patient who was accidentally diagnosed with a cardiac tumor of unknown origin duringroutine physical examination in May 1997. Until a few days prior to elective cardiac surgery for diagnostic purposes in June 1997, noclinical symptoms were present. To our surprise, a primary right atrial leiomyosarcoma was found which almost completely occluded theright atrium and destroyed the tricuspid valve. Despite the combination of surgical removal and adjuvant chemotherapy the patient died 3weeks after the operation due to progressive tumor disease and development of congestive heart failure and lung embolism. 1998Elsevier Science B.V. All rights reserved

Keywords:Cardiac leiomyosarcoma; Heart tumor; Palliative resection; Adjuvant therapy

1. Introduction

Approximately 10% of primary cardiac tumors are malig-nant and most of them are sarcomas. They occur in eithersex and may be found in patients of all ages [1,2].

Primary cardiac leiomyosarcomas usually originatewithin the major thoracic vessels [3].

We report an unusual case of a primary cardiac leiomyo-sarcoma evolving within the right atrium with subtotalocclusion of the atrial cavity and complete destruction ofthe tricuspid valve; there was a lack of clinical symptomsand early death due to massive tumor progression despitecombined surgical and adjuvant chemotherapy.

2. Case report

A 21-year-old caucasian female was admitted to our hos-pital because of a rough diastolic murmur in the third left

intercostal space for further medical examination. She didnot complain of any clinical symptoms. Chest X-ray wasnormal, the electrocardiogram showed a regular sinusrhythm and a P-dextrocordiale.

Transthoracic and transesophageal echocardiographyshowed a tumorous mass of approximately 3 cm× 6 cmin size within the right atrium, invading the tricuspidvalve and the interventricular septum.

Abdominal ultrasound demonstrated a thrombus of 1.3cm × 1.6 cm× 4.1 cm extension in the inferior vena cavajust below the diaphragm without extension to the rightatrium.

Computed tomography of the chest and abdomenrevealed a large hypodense tumorous mass of 3.5 cm× 6cm within the right atrium, invading the right ventricle (Fig.1). In addition, several suspicious foci in the right lung wereidentified. Due to the patient’s age and the suspected neo-plasm’s aggressiveness, an elective thoracotomy for openchest cardiac biopsy and curative tumor resection afterintraoperative rapid histological analysis was planned. Dur-ing induction of anesthesia the patient became hemodyna-mically unstable and was put on cardio-pulmonary bypass.

European Journal of Cardio-thoracic Surgery 14 (1998) 635–638

1010-7940/98/$19.00 1998 Elsevier Science B.V. All rights reservedPII S1010-7940(98)00243-7

* Corresponding author. Tel.: +49-89-7095-2433/2633; fax: +49-89-7095-8898.

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A large atrial tumor including the completely tumorousobstructed tricuspid valve were partially removed in orderto open the valve area and thus reduce an existing upperinflow obstruction. Only palliative resection could be per-formed. Postoperatively, the patient was hemodynamicallystable with low dose catecholamines and a reduction of thecentral venous pressure from 30 mmHg to 14 mmHg.

Despite postoperative treatment with Adriamycin (120mg/day) and Ifosfamid (2.5 mg/day) as adjuvant chemother-apy, the patient died from autoptically confirmed pulmon-ary thrombemboly and progressive tumor disease 3 weekslater.

3. Pathological findings

Histologically, the tumor cells had hyperchromatic,blunt-ended nuclei. Mitotic numbers ranged between twoand four per ten high-power fields (HPF). Zonal necroseswere present (Fig. 2).

Immunohistochemically, the majority of the tumor cellsshowed a positive reaction for smooth-muscle actin and apart of the cells also for desmin. Accordingly, the tumor wasclassified as leiomyosarcoma, grade 2.

At autopsy the main tumor mass originated in the rightatrium (7× 5 × 2 cm). Moreover, the leiomyosarcoma

invaded the tricuspid valve, the atrial septum, the mitralvalve and the base of pulmonary artery and aorta.

There was no evidence of peripheral abdominal or cere-bral metastasis.

The cause of death was recurrent lung embolism, partlyof neoplastic origin.

4. Discussion

Approximately 10% of primary cardiac tumors are malig-nant with angiosarcoma being the most common type [4].

Leiomyosarcoma of cardiac origin is uncommon anddiagnosis has often been made post-mortem [4]. In theAFIP cohort 76% of these tumors were located in the leftatrium, 16% in the right atrium, and 8% diffuse in the ven-tricles [4]. The mean age of presentation is within the fourthdecade. Other large surgical series were even negative forthis kind of tumor [5].

Primary leiomyosarcomas are highly aggressive and infil-trate adjacent tissues.

Although most patients die within 1 year of diagnosis,Pesotto et al. could recently document a 7-year survivalafter combined surgical and adjuvant radiochemotherapy[10]. The short survival time generally observed in thesepatients is due to the inability of complete tumor removal

Fig. 1. Computed tomography of the chest revealing a large hypodense tumorous mass within the right atrium and invading the right ventricle.

636 A. Kornberg et al. / European Journal of Cardio-thoracic Surgery 14 (1998) 635–638

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and surgical resection is often palliative in order to relievesymptoms caused by obstruction of cardiac cavities or majorblood vessels. The clinical signs depend on the location ofthe tumor and its mass effect [2].

Echocardiography is a very useful non-invasive tool forimaging suspicious cardiac tumors and for further differen-tiation concerning the tumor’s dignity [7]. For the operativeplanning and surgical staging computed tomography ormagnetic resonance imaging remain essential [6]. Indispen-sable for performing a differentiated therapy is the exacthistological examination of tumor specimen gained bytransvenous or open chest biopsy [1,10]. The therapy ofchoice is complete surgical resection in combination withadjuvant chemotherapy or radiotherapy [8,10].

As a last resort, heart transplantation is a possible therapyif the patient is in good condition and there are no signs ofmetastasis, unlike in our case [9].

Several unusual findings were observed in our case. First,the patient did not have any clinical symptoms at the time ofdiagnosis despite progressive tumor growth.

Second, diagnosis was made before clinical symptomsoccurred but no sufficient therapy could be offered. Thisindicates, that considering the literature available, no ade-quate diagnostic tool exists in order to identify patients atrisk in very early stages.

Third, the tumor was located primarily in the right atrium,leading to a destructive obstruction of the tricuspid valve. In

addition, the tumor was invading the pulmonary vessels. Arecurrent pulmonary thrombemboly was in part responsiblefor the patients poor prognosis. This case remarkablyreflects the difficulty of early diagnosis in this kind oftumor disease and the necessity of immediate radical surgi-cal therapy in combination with adjuvant radiochemother-apy.

References

[1] Basso C, Valente M, Poletti A, Casartto D, Thiene G. Surgicalpathology of primary cardiac and pericardial tumors. Eur J Cardio-thorac Surg 1997;12:730–738.

[2] Kapoor AS. Cancer and the heart. New York: Springer, 1986;62–75.

[3] Gonzalez-Campora R, Rubi-Uria J, Mora-Marin J, Hevia A, Galera-Davidson H. Pulmonary vein myxoid leiomyosarcoma. Pathol ResPract 1989;185:900–904.

[4] Burk A, Virmani R. Tumors of the heart and great vessels. In: Atlasof tumor pathology, Vol. 16, Washington, DC: Armed Forces Insti-tute of Pathology, 1996;127–170.

[5] Kirklin JW, Barrat-Boyes BG. Cardiac surgery. New York: Wiley,1986.

[6] Szucs RA, Rehr RB, Yanovich S, Tatum JL. Magnetic resonanceimaging of cardiac rhabdomyosarcoma. Cancer 1991;67:2066–2070.

[7] Lynch M, Clements SD, Shanewise JS, Chen CC, Martin RP. Right-sided cardiac tumors detected by transesophageal echocardiographyand its usefulness in differentiating the benign from malignant ones.Am J Cardiol 1977;79:781–784.

Fig. 2. Tumor cells with blunt ended nuclei and the presence of a mitosis (H and E stain, magnification 400×).

637A. Kornberg et al. / European Journal of Cardio-thoracic Surgery 14 (1998) 635–638

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[8] Dein JR, Frist WH, Stinson EB, Miller DC, Baldwin JC, Oyer PE,Jamieson S, Mitchel RS, Shumway NE. Primary cardiac neoplasms.Early and late results of surgical treatment in 42 patients. J ThoracCardiovasc Surg 1987;93:502–511.

[9] Siebenmann R, Jenni R, Makek M, Oelz O, Turina M. Primarysynovial sarcoma of the heart treated by heart transplantation. JThorac Cardiovasc Surg 1990;99:567–568.

[10] Pesotto R, Silvestre G, Luciani GB, Anselmi M, Pasini F, Santini F,Mazzuco A. Primary cardiac leiomyosarcoma: seven-year survivalwith combined surgical and adjuvant therapy. Int J Cardiol 1997;60:91–94.

638 A. Kornberg et al. / European Journal of Cardio-thoracic Surgery 14 (1998) 635–638

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1998;14:635-638 Eur J Cardiothorac SurgA. Kornberg, S.M. Wildhirt, E. Kreuzer and B. Reichart

young female patientAsymptomatic right atrial leiomyosarcoma with tricuspid valve obstruction in a

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