Cardiovascular Pathology 12 (2003) 233 – 236
Left atrial myxoma in a child Case report and review of the literature Massimo A. Padalino a, Cristina Basso b,*, Giorgio Svaluto Moreolo c, Gaetano Thiene b, Giovanni Stellin a a
Institute of Cardiovascular Surgery, University of Padua Medical School, Padua, Italy b Institute of Pathology, University of Padua Medical School, Padua, Italy c Department of Paediatrics, University of Padua Medical School, Padua, Italy
Received 27 November 2002; received in revised form 17 February 2003; accepted 19 February 2003
Abstract A left atrial mass in a 2.7-year-old girl who presented with congestive heart failure, which surgical pathology revealed to be a myxoma, is reported. The timely precise diagnosis obtained by means of two-dimensional echocardiography allowed the immediate surgical treatment of this rare, benign but potentially lethal cardiac tumor in infancy. D 2003 Elsevier Inc. All rights reserved. Keywords: Cardiac tumors; Echocardiography; Pathology
1. Introduction Primary cardiac tumors in infants and children are widely known to be extremely rare [1,2]. Atrial myxoma, the most common cardiac primary neoplasm in the adult, is less often encountered in infants [2 – 5]. Despite the benign pathological pattern, its growth in a small infant heart can make it clinically very aggressive, requiring emergency treatment. We report a case of left atrial myxoma in which a prompt clinical diagnosis permitted successful surgical excision.
2. Case report A 2.7-year-old girl presented with dyspnea on effort over a 2-week period. A progressive worsening of symptoms and onset of peripheral edema led to emergent hospital admission. Chest X-ray demonstrated cardiomegaly (cardio-thoracic ratio of 0.6) and severe pulmonary congestion. A transthoracic 2-D echocardiography (TTE) detected a giant left atrial mass. Intravenous diuretics were
* Corresponding author. Institute of Pathology, Via A. Gabelli, 61, 35121 Padua, Italy. Tel.: +39-49-8272286; fax: +39-49-8272284. E-mail address:
[email protected] (C. Basso). 1054-8807/03/$ – see front matter D 2003 Elsevier Inc. All rights reserved. doi:10.1016/S1054-8807(03)00037-1
administered, and the child was transferred to our hospital for further evaluation. On arrival, she was in severe distress with tachypnoea and dyspnoea (respiratory rate of 60/min); her body weight was 9.9 kg, heart rate was 137/min and arterial pressure was 123/96 mm Hg. Physical examination revealed a systolic murmur of 2/6 at the left low sternal border and diffuse bilateral lung rales. Liver was enlarged and palpable at 4 cm from the right costal margin. There was diffuse peripheral edema of the lower limbs. Electrocardiogram showed sinus rhythm, with a heart rate of 137/min, atrial dilatation and biventricular hypertrophy. Repeated TTE confirmed the previous diagnosis of a large (25 –30 mm), round, solid mass in the left atrium, arising from the region of the fossa ovalis and impinging on the mitral valve annulus causing mitral regurgitation (Fig. 1). The right ventricle was dilated and its estimated systolic pressure was at systemic level (75 mm Hg plus the right atrial pressure), with a systemic arterial pressure of 98 mm Hg. She was scheduled for urgent operation. Through a midline sternotomy, with aortic and bicaval cannulation and moderate hypothermia (30jC), cardiopulmonary bypass was instituted and a left atriotomy was performed. The right atrium was opened and the interatrial septum was incised at the level of the fossa ovalis. Through this combined surgical approach, the pedicle of the mass, laying between the mitral valve annulus and the right inferior pulmonary vein orifice, was excised.
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Fig. 2. (a) Gross view of the excised mass: note the villous surface and the gelatinous aspect. (b) Panoramic histologic view confirms the villous structure of the tumor with abundant myxoid matrix (Alcian PAS 3). (c) Close up of a villous: note single ‘‘stellate’’ cells enmeshed within a myxoid matrix. (Haematoxylin – eosin 120.)
Fig. 1. Two-dimensional echocardiography, parasternal long axis view. Preoperative examination shows a round mass occupying the left atrial cavity (a) and prolapsing into the left ventricular cavity during diastole (b). Color Doppler demonstrates moderate mitral valve regurgitation (c).
The friable and gelatinous mass was gently and carefully removed from the left atrial cavity, avoiding any fragmentation. After direct closure of the atrial septum and the right atrium, the child was weaned off cardiopulmonary bypass. Postoperative course in the intensive care unit was uneventful. At discharge on the eighth postoperative day, she was asymptomatic, her body weight was 9.0 kg; electrocardiogram showed sinus rhythm with a heart rate
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of 90/min. 2-D Echocardiography showed good left ventricular function (EF = 72%), no residual intraatrial masses, normal right ventricular pressure (32 mm Hg + right atrial pressure) and trivial mitral valve regurgitation. Macroscopic pathologic examination revealed the mass to be a sessile atrial villous myxoma (Fig. 2a), 35 20 25 mm in the three diameters and 9.4 g in weight. It was jelly-like and multilobulated, oval-shaped and appeared as a white-brownish mass. At microscopic examination, the mass consisted of abundant myxoid matrix with numerous isolated ‘‘stellate’’ cells scattering in the ground substance (Fig. 2b,c). There were areas of hemorrhage and inflammatory infiltrates, while the interatrial septum was not infiltrated by the tumor. At immunohistochemistry, myxoma cells appeared positive for vimentin and CD31 antibodies. At 12 months follow-up, the child is asymptomatic and is on no oral medications. TTE demonstrates good ventricular function and no residual intracardiac masses.
3. Discussion Cardiac myxoma is reported to be rare in infancy and childhood [1,2]. It can be detected in almost all the cardiac chambers, and because of its rapid rate of growth in the small heart of a child, despite histologic benignity, cardiac myxoma may prove to be fatal much earlier in the natural history of the neoplasm than in adults [4]. While in the adult, embolization and constitutional symptoms are described to be the most common clinical features [3], congestive heart failure has been reported to be the leading presenting symptom in 64% of pediatric patients [2], and smaller babies have a higher risk of death [6,7]. Noninvasive diagnostic imaging techniques have recently developed to such an extent that cardiac tumors have become amenable to effective and immediate treat-
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ment [1]. However, computer tomographic scanning and magnetic resonance imaging techniques are expensive and time-consuming, which can slow down diagnosis and delay treatment. On the other hand, TTE often allows a rapid and precise diagnosis of the intracardiac mass, giving the surgeon all the information he needs to perform a safe and complete resection of the neoplasm [8]. In addition, the peculiar echographic features of intracardiac masses can permit a reasonably reliable diagnosis of its nature. Myxoma is usually a single intracardiac mass, attached to the lower atrial septum by a stalk that permits the tumor to move within the atrial cavity. Other types of cardiac tumors (such as rhabdomyomas, hamartomas or teratomas, fibromas) show different echographic characteristics, such as number of masses (rhabdomyomas are usually multiple), dimensions (fibromas are usually huge), intracardiac localization (hamartomas and teratomas are usually atrial) and echogenic pattern (teratomas and hamartomas are usually nonhomogeneous because of calcification, while rhabdomyomas are usually homogeneous masses). These peculiar features allow the cardiologist to make a reliable differential diagnosis based on TTE. However, when TTE detects an intracardiac mass, clinical history should suggest that we are dealing with a tumor, or rather a vegetation or a thrombus. Our positive experience with echocardiographic diagnosis of cardiac myxomas is supported by data reported in the recent English language literature (Table 1) [6,9 – 19]. Despite their rarity, malignant intracardiac tumors have been described [1,2]. Because tissue characterization is not possible from the echocardiographic features alone, an appropriate therapeutic needs histological diagnosis. Even if this could not affect surgical treatment, it is essential for providing optimal therapy. Today, TTE can provide accurate anatomic information about the tumor location, extent and characteristics, helping to provide a clear and timely surgical plan. Histological
Table 1 Review of English language literature concerning in vivo diagnosis of cardiac myxomas in the pediatric age group No.
Reference
Age
Sex
Site
Diagnosis
Outcome
Clinical features
1 2 3 4 5 6 7 8 9 10 11 12 13
[6] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [present report]
2.4 1y 3 mo 4y 5y 1 mo 2.5 y 10 y 5y 7y 6y 8y 2.7 y
M F M M M M F M F F F M F
RA LA RA Aortic valve RA RA LA RA-tv LA LV RA RA-LA LA
Angio Echo Echo Angio Echo Echo Echo Echo Echo Echo Echo Echo Echo
Died Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive Alive
CHF CHF Sepsis LV-Ao pressure gradient of 80 mm Hg Abdominal pain (embolism) Cyanosis and convulsions CHF Syncope CHF Constitutional symptoms Constitutional symptoms CHF and cardiogenic shock CHF
CHF: congestive heart failure; LA: left atrium; LV: left ventricle; RA: right atrium; tv: tricuspid valve; d: days; mo: months; y: years; Echo: two-dimensional echocardiography; Angio: angiography.
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examination is deemed essential for accurate diagnosis and appropriate clinical management.
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