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PubMed2003

[Tuberous sclerosis and cardiac tumor (case report)].

We report the case of a cardiac tumor, which is found in a systematic cardiac examination of an 11 year old girl who had a tuberous sclerosis. The interest of this observation is to show the importance of a general examination, especially cardiac, within this pathology. Tuberous sclerosis is an hereditary disease, associated with rhabdomyomas in 50% of cases or more. Cardiac rhabdomyomas are frequently multiple and detected in utero in some cases. Conversely, 60% of these tumors are seen in a context of tuberous sclerosis. Rhabdomyomas are the most common cardiac tumors of infants and children, the large majority occurring in patients younger than one year and are diagnosed more and more in foetal echocardiography.

Child↗
PubMed2001

Primary cardiac tumours: when is surgery necessary?

OBJECTIVE: Primary cardiac tumours are rare. The literature predominantly contains series on myxomas in adults and only a few long-term series that involve the very different primary cardiac tumours in early childhood. As foetal ultrasonography has continued to improve, cardiac tumours are increasingly detected early before significant symptoms develop. It is a challenge for paediatric cardiologists and surgeons to ascertain which patients need surgery and which will benefit from conservative follow-up. METHODS: A retrospective review of a 10-year period revealed 51 tumours in 26 children (median age: 1 month). Analysis was by presentation, location, associated findings, interventions, histological findings, and clinical course. RESULTS: The most common tumours were rhabdomyomas (29), fibromas (nine), teratomas (two), and haemangiomas (two). The tumour location was the right ventricle in 24 and the left ventricle in 22 patients. The symptoms varied between abnormal heart murmur (20), arrhythmia and conduction abnormalities (ten), obstruction of the outflow tract >30 mmHg (nine), severe cyanosis (three) and congestive heart failure (two). Fourteen children with haemodynamic compromises underwent surgery. There was one post-operative death and one heart transplantation after bridging with an assist device. There was no tumour recurrence even when resection was incomplete. Nine of 13 children with rhabdomyomas had spontaneous tumour regression without intervention. CONCLUSIONS: Most of the cardiac tumours in children are benign. Spontaneous regression is possible not only in rhabdomyoma. Surgical intervention is only required for children who develop relevant clinical symptoms. Total resection of the tumour is not the only therapeutic aim; more important is the restoration of the best possible heart function.

Adolescent↗
PubMed2005

Maternal and fetal tuberous sclerosis complicating pregnancy: a case report and overview of the literature.

Tuberous sclerosis complex (TSC) is an autosomal-dominant phakomatosis that can result in cardiac and central nervous system lesions and may adversely impact fetal and maternal health. We report a case of a 19-year-old primagravida with TSC whose pregnancy was complicated by preeclampsia, preterm labor, and fetal demise. The fetus, also affected with TSC, was diagnosed with a cardiac rhabdomyoma on ultrasound at 24 gestational weeks and intracranial tubers on fetal magnetic resonance imaging at 26 gestational weeks. Hydrops fetalis developed in the 30th gestational week. Fetal demise occurred during induction of labor. A systematic review of the medical literature was conducted. Our objective was to quantify maternal and fetal morbidity and mortality associated with TSC. We identified 36 additional cases of fetal TSC with cardiac rhabdomyoma diagnosed prenatally. Including our case, we also identified 23 pregnancies (17 mothers) complicated by maternal TSC. Rates of complications are calculated. We conclude that pregnancies complicated by maternal or fetal TSC deserve careful vigilance. Although benign histologically, cardiac rhabdomyomas can result in fetal morbidity and mortality.

Adult↗
PubMed1992

Cardiac tumours in intrauterine life.

Since 1980, 11 examples of cardiac tumour have been detected in the fetus out of a total of 794 congenital cardiac malformations. Patients were referred because of fetal hydrops in two, a family history of tuberous sclerosis in two, and because of the detection of a tumour mass during a scan at the local hospital in seven. The gestational age range at presentation was from 20-34 weeks. Of eight fetuses where death occurred, the histological type was rhabdomyoma in seven and teratoma in one. In seven cases, the lesion appeared single and in four there were multiple tumours. In two of the cases of rhabdomyoma, other family members had evidence of tuberous sclerosis. Termination of pregnancy took place in four cases; of seven continuing pregnancies, spontaneous intrauterine death occurred in four, and three children are still alive. Two of the three survivors has the clinical picture of tuberous sclerosis. The last case is as yet only 1 month old. In summary, even where the lesion is single, the most likely diagnosis in fetal cardiac tumour is rhabdomyoma, with associated tuberous sclerosis. However, the characteristic features of this latter condition may not become evident until some months after birth.

Echocardiography↗
PubMed1993

10 year review of cardiac tumours in childhood.

OBJECTIVE: To review the commonest types of cardiac tumours in childhood, their different presentations, and management. DESIGN: A retrospective study of patients with cardiac tumours. SETTING: Cardiac department of a teaching hospital. PATIENTS: Six patients with an age range between one day to three years presented over a period of 10 years. MAIN OUTCOME MEASURES: To determine different presentations, management, and prognosis of cardiac tumours. RESULTS: Three patients presented with an arrhythmia, two with an asymptomatic heart murmur, and the sixth was discovered accidentally. Surgical resection of the tumours was performed in five cases, one patient died during the operation, one developed new tumours after surgery, and the remaining three have had an excellent result. Histology of those operated on showed benign rhabdomyomas in three and fibromas in the other two. The sixth patient was managed conservatively and the multiple tumours he had showed gradual resolution over a period of 10 months follow up. One baby with rhabdomyoma had signs of tuberous sclerosis whereas the others were normal at follow up five months to three years after diagnosis. None of them had a positive family history. CONCLUSION: Cardiac tumours in childhood are extremely rare. The commonest types are rhabdomyomas, then fibromas. Most cases are diagnosed by echocardiography. The prognosis for most patients is excellent.

Child, Preschool↗
PubMed1986

Soft tissue tumor with prominent leptomeric fibrils and complexes.

A recurrent soft tissue tumor from the groin in a 37-year-old male is described. The tumor cells contain many leptomeric structures, both fibrils and complexes, but none of the other characteristic features of striated muscle: alternating parallel thick and thin myofilaments with Z-line material. A review of one rhabdomyoma and six rhabdomyosarcomas demonstrates that leptomeric structures are present in large amounts in the rhabdomyoma but are very infrequent in only three of the six rhabdomyosarcomas. A careful search for leptomeric structures is indicated when the ultrastructural diagnosis of either rhabdomyoma or rhabdomyosarcoma is being considered.

Adult↗
PubMedDate not supplied

Cardiac tumors in infants and children.

Cardiac tumors in infants and children are extremely rare. Their clinical manifestations vary widely from asymptomatic presentations to life-threatening cardiac events. Improvements in diagnostic techniques, such as those offered by echocardiography, have made early detection of cardiac masses possible, with or without the presence of clinical symptoms. Fifteen pediatric cases of cardiac tumor were diagnosed at our institution between July 1989 and July 2002 (male-female ratio, 10:5; age range, one day to nine years). Eleven of the cases involved primary cardiac tumors [rhabdomyoma (n = 10) and fibroma (n = 1)]. Ninety percent of the rhabdomyomas (9/10) were associated with tuberous sclerosis. Four of the fifteen cases were secondary metastatic tumors [hepatoblastoma (n = 2), hepatoma (n = 1) and rhabdomyosarcoma (n = 1)]. Clinical manifestations of the cardiac tumors included shortness of breath (n = 5), seizure (n = 4), cardiac murmur (n = 6), and cyanosis (n = 3). Surgery was performed for three of 11 patients with primary cardiac tumor (27%) due to severe obstruction of flow (n = 2) and other cardiac defects (n = 1). The primary cardiac tumor spontaneously regressed in five of the tuberous sclerosis patients. All four of the patients with secondary cardiac tumors continued to receive chemotherapy, and only one of them subsequently experienced regression. Based on our experiences, we conclude that: 1) rhabdomyoma is the most common primary cardiac tumor in children; 2) most pediatric tumors are associated with tuberous sclerosis; 3) clinical presentation is determined by the tumor size and number of tumors, and whether expansion of the malignancy has resulted in cardiac blood-flow obstruction; 4) there is a strong possibility of regression of the primary cardiac tumor, with surgery recommended only when cardiac symptoms are severe; and, 5) unless there is a significant obstruction of blood flow, chemotherapy is still the treatment of choice for secondary cardiac tumors.

Carcinoma, Hepatocellular↗
PubMed2005

[Diagnostic value of echocardiography for cardiac tumors in infancy and childhood].

OBJECTIVE: The present study was designed to evaluate the clinical manifestations, surgical findings, pathologic types and treatment of cardiac tumor and to analyze the echocardiographic characteristics of the cases. METHODS: Records of 19 patients with cardiac tumors confirmed by operations and pathology at Fuwai Cardiovascular Hospital in Beijing, China between Jan, 1983 and Dec, 2003 were reviewed. Clinical and echocardiographic data of all patients were analyzed. RESULTS: The median age of patients was 7 +/- 5 years, ranging from 5 months to 14 years. There were 8 male and 11 female cases. The surgical findings revealed that 57.9% (11 cases) of cardiac tumors were found in left heart, 36.8% (7 cases) in right heart, 5.3% (1 case) in two ventricles. The pathological study revealed that 17 cases (89.5%) were diagnosed as primary cardiac benign tumors including myxoma in 10 cases (52.6%), rhabdomyoma in 4 cases (21.1%), fibroma in 2 cases (10.5%) and lipoma in 1 case (5.3%). Two cases were diagnosed (10.5%) as cardiac malignant tumors including a primary cardiac rhabdomyosarcoma and a metastatic epithelioid sarcoma. By using echocardiography 11 cases were diagnosed as myxomas and rhabdomyoma (11/19, 57.9%), 8 cases were diagnosed as space occupying lesions of the heart or myxoma (8/19, 42.1%). CONCLUSIONS: Myxomas are the most common heart tumors seen in infancy and childhood, followed in frequency by rhabdomyomas, fibromas and lipomas. Surgery is recommended for patients with refractory and severe clinical symptoms.

Adolescent↗
PubMed1998

Neonatal intracardiac rhabdomyomatosis: a case report.

Intracardiac tumors are rare in neonates. Most of these lesions are rhabdomyomas and they occur almost exclusively during infancy. Rhabdomyomas are commonly associated with tuberous sclerosis and often involve the brain, kidneys and pancreas; they are frequently multiple and originate most commonly from the ventricular septum. Surgical intervention is indicated for rhabdomyoma with either mechanical cardiac obstruction or dysrhythmias resulting in symptoms or sudden death. A newborn with diffuse rhabdomyomatosis over the right atrium, right ventricle and left ventricle of the heart complicated with congestive heart failure and intractable supraventricular tachycardia is reported herein. No tuberous sclerosis or other organ involvement was noted. The tumor was resected.

Heart Neoplasms↗
PubMedDate not supplied

Primary intraventricular cardiac tumors in children: contemporary diagnostic and management options.

We reviewed the intraventricular cardiac tumors presenting at our institution between 1985-1991, studying the presentation, modes of investigation, and evidence of hemodynamic compromise. Thirteen patients presented with intraventricular tumors during the study period. Two of the tumors were rhabdomyosarcomas, one was a myxoma, and 10 were rhabdomyomas. All patients were evaluated with two-dimensional and pulsed Doppler echocardiography and B-color imaging was undertaken in three patients. Four patients presented for elective scans to complement investigations for tuberous sclerosis, seven patients had cardiac symptoms, and two patients presented prenatally. Obstruction to intracardiac flow was present in five patients. Two patients had the tumor excised and one had an open biopsy of the tumor. One patient had an transvascular biopsy at cardiac catheter. Early detection of cardiac tumors is increasing, particularly rhabdomyomas. With fetal echocardiography, more patients should come to attention prenatally. B-color may be useful addition in assessing cardiac tumors, aiding detection and definition of intramural lesions.

Child↗
PubMed1990

Unexpected infant death attributable to cardiac tumor or cardiomyopathy. Immunohistochemical and electron microscopical findings in three cases.

The pathological findings, including immunohistochemical and electron microscopical findings, in three infants who died unexpectedly of cardiac tumor or cardiomyopathy are reported. The first was a 13-month-old boy with tuberous sclerosis and multiple rhabdomyomas of the heart, who presented with a postpartal cardiac murmur and moderate cardiomegaly. The further history was unknown. The rhabdomyoma nodules were composed of spider cells containing small amounts of desmin and myosin as well as isolated myofibrils. Microscopically small glioma nodules contained high amounts of GFAP. The second case, a boy 4 months of age, died of a large benign fibrous histiocytoma of the heart after an uneventful history. Tumor cells contained alpha-1-anti-chymotrypsin and lysozyme. The third case, a girl 2 months of age, died unexpectedly of histiocytoid cardiomyopathy. The affected cells contained fat droplets, glycogen granules, many leptomer myofibrils and small amounts of myosin and desmin.

Astrocytoma↗
PubMedDate not supplied

Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians.

Because primary tumors of the heart in infants and children are extremely rare, most knowledge is based on collections of case reports rather than large cohort studies. The types of heart tumors encountered in the pediatric age group differ from those seen in adults. In the latter, cardiac myxomas are by far the most common tumor; in infants and children the most common primary tumor of the heart is the rhabdomyoma. Spontaneous regression of these tumors has been well established so that surgical intervention is no longer indicated unless there are clinical manifestations from the heart. There is a high incidence of associated tuberous sclerosis. Any intracavitary mass in infants is suggestive for a cardiac rhabdomyoma unless otherwise proven. The second most common tumor in this age group is cardiac fibroma. These tumors probably represent hamartomatous lesions and this has led to a strategy in which radical surgical excision is not indicated if the procedure endangers postoperative heart function. Cardiac myxomas are of interest in this age group because of their familial occurrence as part of the myxoma syndrome. Other types of primary heart tumors occur, including malignant variants, but all are exceedingly rare. It is because of the sporadic nature of these tumors that treatment and insights into prognosis are based largely on case documentations and analogies from similar tumors originating elsewhere.

Child↗
PubMed1997

Pediatric primary benign cardiac tumors: a 15-year review.

An increase in the incidence of primary cardiac tumors has been reported since the development or enhancement of noninvasive imaging modalities. We identified 56 children with primary cardiac tumors. Forty-four (78%) children had rhabdomyomas, 6 (11%) fibromas, 1 (2%) pericardial teratoma, 1 (2%) epicardial lipoma, 1 (2%) multicystic hamartoma, and 3 (5%) unspecified tumors. The mean age at diagnosis was 19 +/- 35 months (median 4.7 months, range 0.03 to 204 months), excluding 12 patients who were given the diagnosis before birth. Among 27,640 patients assessed for cardiac disease, the incidence of tumors was 0.06% (1980 to 1984), 0.22% (1985 to 1989), and 0.32% (1990 to 1995). Diagnosis was made in 55 of 56 patients by echocardiography. Catheterization was performed in five patients and magnetic resonance imaging in nine. No tumor-related deaths occurred. Nine patients had surgery because of hemodynamically significant obstruction or arrhythmias. Partial or complete regression occurred in 24 (54%) of 44 patients with rhabdomyomas. Overall, the prognosis was excellent. Individualized surgery allowed early safe treatment of symptomatic tumors.

Child↗
PubMed1997

[Fetal cardiac tumors].

INTRODUCTION AND OBJECTIVES: Fetal cardiac tumors are rare and have a different histology than in adults: in the fetus, rhabdomyoma is more prevalent, but myxoma has not been described. We report our experience with nine fetuses with prenatally diagnosed primary cardiac tumours. METHODS: This is an observational and descriptive study of fetuses investigated because of the prenatal and echographic diagnosis of heart tumors. RESULTS: There were nine fetuses with thirteen cardiac masses among more than 700 fetal echocardiographic studies performed by pediatric cardiologists. Histology was available in four of them; three rhabdomyomas and once cavernous hemangioma. In one of the cases, the parents elected to interrupt the pregnancy; three patients died in the neonatal period (two as a direct consequence of the tumors and one due to sepsis) and we have no histological information regarding any of the other five fetuses. The subjects who survived beyond the neonatal period are mostly doing well. However, one developed tuberous sclerosis and another developed a hypoplastic left heart syndrome, perhaps due to the massive restriction of flow across the foramen ovale. The tumors in the other cases were well tolerated and hemodynamic or arrhythmic consequences were minimal of absent. No women with risk factors such as tuberous sclerosis were included in our study. CONCLUSIONS: Fetal cardiac tumors are rare and display a different histology. We recommend a conservative approach to treatment.

Echocardiography↗
PubMed1983

Investigation and management of primary cardiac tumors in infants and children.

Seven children with primary cardiac tumors are discussed. The diagnosis was achieved by cardiac catheterization in all seven patients. In four patients, surgical excision was undertaken with one long-term good result. One of the other three surgical patients died of acute heart failure at the discontinuation of cardiopulmonary bypass. One of the other two surgical patients died suddenly late postoperatively, presumably from arrhythmia. The other, affected by fibrosarcoma, also died suddenly while undergoing chemotherapy. The histologic findings on the tumor masses were available in six patients. There were three rhabdomyomas, one fibrosarcoma, one fibroma and one hamartomatous mass. In one patient with tuberous sclerosis, a presumptive diagnosis of rhabdomyoma was made clinically on the basis of multiple masses infiltrating the left ventricle. Two-dimensional echocardiography is the best noninvasive tool to determine the extent and location of the tumor and suitability for surgery. When surgery is indicated, cardiac catheterization for pressure data is also required. Surgery is recommended in symptomatic solitary lesions that most likely are fibromas and have an overall good surgical outcome.

Cardiac Catheterization↗
PubMed1999

Primary pediatric cardiac tumors: a 17 year experience.

We reviewed 22 cases of primary pediatric cardiac tumors followed at our institution from January 1981 through November 1997, analyzing them by subtype, age and manner of presentation, location, associated findings, interventions, and clinical course. Rhabdomyomas were the most common (11), followed by intrapericardial teratomas (2), myxomas (1), fibromas (1), hemangiomas (1), mesotheliomas (1), and rhabdomyosarcomas (1), with 4 undetermined tumors. The majority (77%) of tumors were diagnosed before the age of one year, including six prenatally. The most common presentations were murmurs (5), arrhythmias (5), and abnormal screening fetal ultrasound examinations (4). Tumors were located most frequently in the right ventricle (13) and left ventricle (7), with multiple tumors being present in 10 cases. Eight patients (36%) had associated arrhythmias or conduction abnormalities, and of the 11 patients with rhabdomyomas, tuberous sclerosis was diagnosed in 8. Eight patients underwent cardiac catheterization, including two for electrophysiologic study with radiofrequency ablation, and seven patients had complete or partial tumor resection. The follow-up period ranged from 2 months to 15 years, and there were 3 tumor-related deaths. Therefore, despite the benign histology of most primary pediatric cardiac tumors, there may be significant associated morbidity and occasional mortality. As echocardiographic techniques such as fetal ultrasonography have continued to improve, however, these cardiac tumors have increasingly been detected early before significant symptoms develop.

Age Distribution↗
PubMed2001

Perinatal diagnosis of cardiac tumors.

OBJECTIVE: As fetal cardiac tumors are a rare condition, we report the perinatal diagnosis and ultrasound findings of 12 cases. METHODS: In 10 cases the tumors were identified prenatally by fetal echocardiography; gestational age at detection ranged from 22 to 34 weeks. In two symptomatic infants cardiac tumors were diagnosed on the first day postpartum; prenatal ultrasound evaluation at 26 and 38 weeks of gestation did not reveal a cardiac lesion. RESULTS: Six fetuses had singular tumors, in six fetuses they were multifocal. The left ventricle was most often affected. Termination of pregnancy was chosen in three cases, one in association with trisomy 21 and tuberous sclerosis. One intrauterine and three neonatal deaths due to cardiac failure occurred. Histopathologic examination revealed cardiac rhabdomyoma in six fetuses and fibroma in one case. In the five surviving fetuses the size of the tumors spontaneously decreased postpartum. Rhabdomyomata were associated with tuberous sclerosis in four out of 11 cases. CONCLUSION: Cardiac tumors are detectable as early as 22 weeks of gestation. Presenting symptoms may be arrhythmia, dysfunction of the atrioventricular valves, pericardial effusion and fetal hydrops. The most common perinatal diagnosis is rhabdomyoma, which is often associated with tuberous sclerosis. Sequential examination in high risk patients should be considered as most tumors increase in size during pregnancy and may become evident in late second and third trimester of pregnancy. Postpartum, however, regression of tumor size is common.

Echocardiography↗
PubMed1990

Special considerations on primary cardiac tumors in infancy and childhood.

Primary cardiac tumors are rare at all ages and are even less common in infants and children. In the vast majority of cases, they are benign tumors that show little tendency to growth. Primary malignant tumors of the heart are exceptionally rare. Rhabdomyomas are the most common type of tumors in infants, followed in frequency by the fibromas and the teratomas. Myxomas are very rare in pediatric patients. Symptoms that derive from the presence of a neoplastic mass are usually due to obstruction or compression. In young symptomatic patients, usually affected by rhabdomyomas, there is little chance of survival without surgery. On the other hand several patients with cardiac neoplasms are totally asymptomatic. The indication for surgery is differenciated according to the severity of the clinical picture. Since 1974, 15 infants with a cardiac tumor have been observed in our hospital. Our recent experience with 5 cases, which are described in this report, confirm our opinion, that the most important fact is the restoration of the best possible heart function. Total resection of a tumor should not always be the main therapeutical aim. Surgical intervention is required for those patients, who develop relevant clinical symptoms. Close observation of the clinical course is mandatory in all patients with a doubtful indication for operation.

Child, Preschool↗