A rolling ball like mass in right atrium of a 6 year old child

Srinivas Kola; Tammiraju Iragavarapu; Sushma Vutukuru

doi:10.4103/heartindia.heartindia_38_18

CASE REPORT

Year : 2018 | Volume : 6 | Issue : 4 | Page : 148-152

A rolling ball like mass in right atrium of a 6 year old child

Srinivas Kola¹, Tammiraju Iragavarapu², Sushma Vutukuru³
¹ Department of Ct surgery, Pinnamaneni Siddhartha Medical College, Gannavaram, Andhra pradesh, India
² Department of Cardiology, Alluri Sita Ramaraju Academy of Medical College, Eluru, Andhra pradesh, India
³ Department of Pathology, Alluri Sita Ramaraju Academy of Medical College, Eluru, Andhra pradesh, India

Date of Web Publication

17-Dec-2018

Correspondence Address:
Dr. Tammiraju Iragavarapu
Department of Cardiology, Asram Medical College, Eluru, Andhra Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/heartindia.heartindia_38_18

Abstract

Cardiac myxoma is a common tumor occurring more commonly in the left atrium in adult females. Its occurrence in pediatric age group is rare; the more common tumors are rhabdomyoma and fibroma. Presentation of myxoma on the right side is still rarer in this age group. We report a case of a 6-year-old male child who presented with prolonged fever. On examination, there is an extra diastolic sound with mid-diastolic murmur. Echocardiogram is suggestive of a large ball-like polypoidal mass rolling in the right atrium traversing into the right ventricle. The case was immediately operated, and histopathology confirmed the tumor as myxoma. High index of suspicion is required to diagnose the condition to prevent disastrous complications as the presentation is often enigmatic because of vague constitutional findings in most of the cases.

Keywords: Child, myxoma, right atrium

How to cite this article:
Kola S, Iragavarapu T, Vutukuru S. A rolling ball like mass in right atrium of a 6 year old child. Heart India 2018;6:148-52

How to cite this URL:
Kola S, Iragavarapu T, Vutukuru S. A rolling ball like mass in right atrium of a 6 year old child. Heart India [serial online] 2018 [cited 2023 Jun 2];6:148-52. Available from: https://www.heartindia.net/text.asp?2018/6/4/148/247575

Introduction

Cardiac myxoma is a rare disease with an overall incidence of about 0.5/million/year and accounts for approximately 70% of all cardiac tumors occurring more commonly on the left side. By far, the most common type of tumors reported in children and adolescents is rhabdomyoma followed by fibroma, myxoma, and teratoma. We report a rare case report of myxoma of the right atrium occurring in a 6-year-old child.

Case Report

A 6-year-old boy presented with easy fatigability and low-grade fever for 6 months. He was initially evaluated by a pediatrician. The case was referred to our department in view of abnormal cardiac examination (extra heart sound which is varying with position). Other systems were normal. The chest X-ray showed clear lung fields with right atrial (RA) enlargement. An electrocardiogram showed sinus rhythm with right bundle branch block (RBBB). Two-dimensional echo, to our surprise, revealed a large mobile mass, 6 cm × 5 cm which is solitary, pedunculated with lobulated surface, and is intermittently blocking tricuspid valve. The case was immediately operated, and histopathology confirmed the tumor as myxoma.

Discussion

Cardiac tumors were first described in the 18^th century by Boneti; however, many believe the description by Albers in 1835 is the first authentic report. Primary cardiac neoplasms are rare and occur with an estimated incidence of 0.0017%–0.19%, representing <5% of all heart tumors.^[1] Benign neoplasms occur 3 times more often than malignant tumors. Cardiac myxoma is a rare disease with an overall incidence of about 0.5/million/year and accounts for approximately 70% of all cardiac tumors.^[2] Incidence of cardiac tumors in pediatric age groups as described by Boston's children hospital are Rhabdomyomas-45%, Myxomas -23%, fibromas – 8%, others – 9%, nonspecified – 7%, and metastatic dissemination – 7%. The age of those patients diagnosed with metastatic tumor involvement ranged from 6 to 15 years of age.

Etiopathogenesis

Cardiac myxoma is a globular or polypoid mass that usually arises from the region of the fossa ovalis and is attached to the underlying endocardium by a pedicle. The etiology of cardiac myxomas is not fully understood, but they are thought to originate in undifferentiated and totipotent mesenchymal stem cells that are capable of neural and endothelial differentiation.^[3],[4] Myxomas produce vascular endothelial growth factor, which probably contributes to the induction of angiogenesis and the early stages of tumor growth.^[5],[6] Mutations of the protein kinase A regulatory subunit gene PRKAR1A cause inherited myxomas in the setting of the Carney complex tumor syndrome, providing a possible window for understanding their pathogenesis.^[7] Recent investigations have revealed that interleukin-6 in plasma is the cause of constitutional manifestations.^[8]

Approximately 70% of affected patients are women predominantly between the third and sixth decades of life^[9],[10] with a mean age of 53.9 (19–79) years. They are rarely seen in children, accounting for only 9%–15% of all cardiac tumors from birth to adolescence, and the usual site of location is left atrium.^[11] Right-sided tumors are uncommon, and myxomas are the most common tumors of the right atrium. However, sarcomas and, in particular, angiosarcomas have been reported to arise in the right atrium. The RA myxoma originates in the fossa ovalis or base of the interatrial septum;^[12] our patient is very young (6 years) which is a rare presentation.

Clinical features

The clinical presentation of a patient with a cardiac tumor is determined more by the tumor's location than by its histologic type. RA myxomas may remain asymptomatic.^[13] Approximately 7% of all cardiac myxomas occur in association with Carney's complex. Carney's complex, as described by J. A. Carney, is characterized by the association of cutaneous pigmentation, fibromyxoid tumors of the skin, myxomas of the heart, endocrine overactivity, and autosomal dominant inheritance.^[14] Cardiac tumors may cause symptoms through a variety of mechanisms.^[15] Myxomas present with a classic triad of symptoms, including cardiac obstruction (80%), emboli (70%), and systemic illness (65%). These patients can have an increased risk for acute cardiogenic shock or sudden cardiac death.^[16] Typical cardiovascular signs and symptoms are those of right heart failure (i.e. fatigue, peripheral edema, hepatomegaly, ascites, and prominent “a waves” in the jugular veins) in case of RA myxoma. On physical examination, a diastolic murmur has been described, which is similar to the “tumor plop” heard with left atrial myxomas.

In addition to obstructing circulation through the right side of the heart, tumor fragments may be released into the pulmonary circulation, causing symptoms consistent with pulmonary emboli.^[17] RA hypertension can result in shunting of venous blood into the systemic circulation if a patent foramen ovale (or atrial septal defect) is present, resulting in hypoxemia or systemic emboli.^[18],[19]

Diagnosis

Electrocardiography may show specific chamber enlargements, features of pulmonary artery hypertension, and RBBB if any due to tumor. Echocardiography remains the best diagnostic method for locating and assessing the extent of myxomas and for detecting their recurrence, with a sensitivity of up to 100%. In our case, two-dimensional (2D) echo confirmed RA myxomas originating near fossa ovalis traversing the tricuspid valve [Figure 1]. However, transthoracic echocardiogram may not identify tumors smaller than 5 mm in diameter, and a transesophageal echocardiogram (TEE) is required when there is suspicion of a very small tumor.^[20] The superior diagnostic utility of TEE is due to the proximity of the esophagus to the heart, the lack of intervening lung and bone, and the ability to use high-frequency imaging transducers that afford superior spatial resolution.^[21] Magnetic resonance imaging is a powerful tool in diagnosing tumors, evaluating their extent, and seeking metastases.^[22] It may delineate the area of the mass better than echocardiography because it has a larger field of observation. Contrast enhancement and multislice imaging can provide a 3D view of tissue. It also can help differentiate tumors from thrombi.

Figure 1: (a) Two-dimensional echo four-chamber view showing large ball-like polypoidal mass traversing the tricuspid valve with calcifications (arrow). (b) Modified short-axis view showing large mass measuring 6 cm × 4 cm

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Treatment and prognosis

Once a presumptive diagnosis of myxoma has been made on imaging studies, prompt resection is required because of the risk of embolization or cardiovascular complications, including sudden death.^{[23],[24],[25]} Surgical excision of cardiac myxomas is the mode of treatment and carries a low operative risk and gives excellent short- and long-term results. The operative mortality ranges from 0% to 3% in multiple series. The survival after surgery is elevated.^[26] Right-sided cardiac myxomas present surgeons with a technical challenge because placement of the cannula for cardiopulmonary bypass can be difficult. Tumor fragmentation, followed by embolization of myxomatous debris, can be a serious intraoperative complication of this procedure. The risk of this can be greatly reduced, though, by manipulating the heart as little as possible during cannulation and tumor excision.^[3] Resection should be complete, including all of pedicle and area attachments, plus a margin safety.

Grossly typical myxomas are pedunculated and gelatinous in consistency; the surface may be smooth, villous, or friable [Figure 2]. Tumors vary widely in size. Histologically, these tumors are composed of scattered cells termed lipidic cells within a mucopolysaccharide stroma [Figure 3]. These cells are multinucleated with pink cytoplasm. Typically, they present in clusters surrounding vascular structures. Calcium is present in approximately 10% of patients with a higher incidence of calcification in right-sided tumors.^[27] Usually, the embolized material has a different pattern from the material at the center of the lesion.

Figure 2: (a) Intraoperative image of excision of the right atrial myxoma. (b) Excised right atrial myxoma, postoperative image

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Figure 3: Histopathologic section studied shows lobules of mature adipose tissue with clear vacuolated cytoplasm and eccentrically placed nucleus with intervening thin fibrous septa and scant blood vessels. Background shows myxoid areas suggestive of myxoma

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The recurrence rate of sporadic tumors is very low between 1% and 3%^[28] and may occur within a few months to several years after the initial surgical excision and most are found during the first 4 years.^[18] Incomplete resection, intraoperative displacement, embolization, and multifocal genesis have been proposed as possible explanations. Cardiac autotransplantation (with atrial reconstruction) or transplantation are potential options for the treatment of recurrent atrial myxoma.^[29],[30]

Features of cardiac myxoma are mentioned in [Table 1] with special emphasis on right-sided myxomas.

Table 1: Features of right atrial myxoma

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Conclusion

RA myxoma is rare and its presentation in a 6-year-old child is still rarer. Their presentation is often enigmatic because of vague constitutional findings. Early consideration, expeditious diagnosis, and prompt surgical intervention can avoid profound illness and death in these patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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