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PubMed1990

Primary cardiac tumors: experience at the University of Minnesota.

A review of all primary cardiac tumors seen at the University of Minnesota or entered in the Registry of Cardiovascular Disease, affiliated with the University of Minnesota, between 1959 and 1989. Of a total of 124 primary cardiac tumors, 103 (83%) were benign and 21 (17%) were malignant. Atrial myxomas (51 cases) accounted for 42% of all tumors and 50% of benign neoplasias. The remaining 51 benign tumors were, in order of frequency: rhabdomyomas, 14 (13%), papillomas, 12 (11%), fibromas, 9 (9%), hamartomas, (4%), teratomas (2%), lipomas (2%), mesotheliomas (2%), fibroelastomas, hemangiomas, glomangiomas, and a few others. The 21 malignant tumors, all sarcomas, were, in order of frequency: rhabdomyosarcomas, 6 (29%), angiosarcomas, 4 (19%), myxosarcomas, 3 (14%), fibrosarcomas, 2 (10%), and one each leiomyosarcoma, reticulum cell sarcoma, and liposarcoma. There were 3 cases of spindle cell sarcoma (14%) originating in the great vessels: 1 in the pulmonary artery and 2 in the aorta. The prognosis of the resected benign tumors was good, with no complications of recurrences for up to 15 years of follow-up. Tumors that could not be excised included mostly rhabdomyomas, in newborns, and cavernous hemangiomas involving most of the heart, with poor prognosis. Malignant tumors of the heart had a very poor prognosis, even when operated on promptly after diagnosis. The mean survival time of these patients was 5 months after resection, even when followed with chemotherapy and/or radiation; 26% of the tumors had already metastasized at the time of operation. Extensive resections in malignant tumors did not render satisfactory results.

Combined Modality Therapy↗

PubMed1994

Arrhythmias in infants and children with primary cardiac tumours.

Twenty-one patients, aged from 1 day to 16 years (median 8 months), with primary cardiac tumours, were studied with regard to the occurrence of arrhythmias. Standard electrocardiogram (ECG) was available in 20 cases and 24 h ambulatory ECG in 19; nine patients had prospective follow-up investigations (mean follow-up time 3.0 years). Standard ECG revealed preexcitation in two and arrhythmias in six patients. Ambulatory ECG disclosed seven patients with significant rhythm disturbances and in four of these, the arrhythmia was the symptom leading to the diagnosis of a cardiac tumour. Three patients were symptomatic from arrhythmia and two of them were successfully treated with antiarrhythmic agents. The remaining patient with recurrent life-threatening ventricular tachycardia underwent partial resection of a large left ventricular fibroma; no further episodes of ventricular tachycardia were observed postoperatively. Only one of the 15 patients with cardiac rhabdomyomas required antiarrhythmic therapy. None of the nine patients prospectively followed developed arrhythmias requiring therapy. The present data underline the fact that infants and children with cardiac tumours rarely need surgery because of arrhythmia resistant to medical treatment. As far as possible, conservative management is indicated in infants with suspected rhabdomyomas.

Adolescent↗

PubMed2001

Tumors and the heart: molecular genetic advances.

Recent molecular genetic investigations of primary cardiac tumors (myxomas, lipomas, rhabdomyomas, and fibromas) have provided insight into fundamental mechanisms of cardiac cell growth. Myxomas are the most common adult cardiac tumor, and familial cardiac myxomas are now appreciated to be caused by mutations in the PRKAR1alpha gene that encodes a regulatory subunit of protein kinase A. Cytogenetic studies have targeted candidate chromosomal loci that may be perturbed during cardiac lipoma pathogenesis. Rhabdomyomas, the most common pediatric cardiac neoplasm, are frequently associated with tuberous sclerosis, caused by mutations in the TSC-1 and TSC-2 genes. The study of Gorlin syndrome has shed light on the etiology of cardiac fibromas. This disorder is caused by mutation of the PTC gene, which regulates cell growth, commitment and differentiation. In the future, manipulation of PRKAR1alpha-, TSC-, and PTC-dependent pathways may foster new strategies to regenerate myocardium in the ischemic or myopathic heart.

Fibroma↗

PubMedDate not supplied

MR imaging of pediatric cardiac tumors previously diagnosed by echocardiography.

Ten children, ranging in age from 3 weeks to 11 years, with a history of primary cardiac tumors were studied by two-dimensional echocardiography and by ECG triggered magnetic resonance (MR) imaging (six of them had rhabdomyomas). Eighteen of 31 tumors with intracavitary extension diagnosed by echocardiography were detected by MR. In addition, three intracavitary tumors were found by MR but not by echocardiography. Only 2 of 15 intramural tumors detected by echocardiography were identified by MR. Magnetic resonance imaging is inferior to echocardiography in the detection of intramural rhabdomyomas, but superior in delineation of the intra- and extracardiac extension of nonrhabdomyomal tumors.

Child↗

PubMed1996

Electrophysiologic study and radiofrequency ablation in patients with intracardiac tumors and accessory pathways: is the tumor the pathway?

INTRODUCTION: There is a strong association of cardiac rhabdomyomas with the Wolff-Parkinson-White syndrome. This report describes the results of investigations in two patients with accessory pathway-mediated AV reciprocating tachycardia coexisting with intracardiac tumors. METHODS AND RESULTS: Two patients with intracardiac tumors had mapping of the accessory pathway. Echocardiograms obtained in the electrophysiology laboratory while the ablation catheter was at the site of successful radiofrequency ablation demonstrated a close correspondence between the site of intracardiac tumor and the location of the accessory pathway. CONCLUSIONS: These results lend support to the hypothesis that accessory pathways in patients with intracardiac tumors, such as rhabdomyomas, are not typical Kent bundles, but instead are either part of the intracardiac tumor or are closely related to the tumor. Ablation is possible in at least some patients with accessory pathways associated with intracardiac tumors.

Adolescent↗

PubMed1999

Dystrophic calcification of the fetal myocardium.

Intramural cardiac masses were detected antenatally in three fetuses by echocardiography. The masses were initially thought to be rhabdomyomas. All three pregnancies were terminated and histology showed dystrophic calcification in all, with no evidence of tumour. Therefore, dystrophic calcification of the fetal myocardium may have a similar appearance to single or multiple rhabdomyomas. This should be considered when counselling parents after detection of masses in the fetal heart, particularly when considering the risk of associated tuberous sclerosis.

Abortion, Therapeutic↗

PubMed1989

Diagnostic and counselling difficulties using a fully comprehensive screening protocol for families at risk for tuberous sclerosis.

Tuberous sclerosis (TS) results from an autosomal dominant gene which exhibits variable expression and reduced penetrance. Although there are well established diagnostic criteria for TS, examination of first degree relatives can cause diagnostic criteria for TS, examination of first degree relatives can cause diagnostic problems with consequent difficulties in genetic counselling. Using an extensive, non-invasive protocol consisting of skin examination with Wood's lamp, cranial CT scan, specialist ophthalmological and dental examination, skeletal survey, and echocardiography, we have examined 56 first degree relatives of persons with TS. These consisted of 40 parents and seven sibs from 25 sporadically affected families and nine persons from seven multigeneration families. In seven of the apparently sporadically affected families, three mothers had echocardiographical findings consistent with one or more rhabdomyoma. In another, the mother's renal ultrasound showed evidence of single cysts in both kidneys. In a fifth family, the father had suggestive but not diagnostic features of TS on the cranial CT scan and skeletal survey. In the sixth family, the mother was found to have atypical calcification on CT scan. In a seventh instance a sib from a two generation family had echocardiographical evidence of a rhabdomyoma. Even though the proband in three of the sporadically affected families presented with fits, developmental delay, and depigmented patches, and therefore did not strictly fulfil the diagnostic criteria for TS, two mothers were found to have evidence of rhabdomyomata on echocardiography and the brother of the third had typical depigmented patches. Although the presently accepted diagnostic criteria for TS may not allow one to make a definitive diagnosis of TS in these relatives, we recommend that an extensive screening protocol be used to examine first degree relatives and that caution be used in counselling apparently unaffected members of families at risk for TS.

Bone and Bones↗

PubMed1989

Nucleolar organizer regions in normal skeletal muscle and benign and malignant rhabdomyoblastic tumors.

The silver staining of interphase nucleolar organizer regions (NORs) has been shown to have an important application in diagnostic histopathology for distinguishing some benign from malignant conditions. In this study, normal fetal and adult skeletal muscles and tissue from fetal and adult rhabdomyomas as well as rhabdomyosarcomas were stained with the silver method for NORs. The morphologic distribution of NORs in rhabdomyosarcomas was found to be very different from that in normal skeletal muscles. In addition, cases of rhabdomyoma were easily differentiated from rhabdomyosarcomas. Statistical analysis of data, from all cases, regarding the diameter of NORs and number per nucleus confirmed these observations.

Adolescent↗

PubMed1992

Survey of congenital tumors in perinatal necropsies.

In an audit of 17,417 necropsies from 1939 to 1989 at the Royal Women's Hospital, Melbourne, Victoria, 46 congenital tumors comprising 24 teratomas, 8 vascular tumors, 6 neuroblastomas, three rhabdomyomas, two mesoblastic nephromas, one thyroid adenoma, one hepatic adenoma and one cerebellar medulloblastoma were found. The teratomas were generally large tumors and, because of their size and location, incompatible with extrauterine life. Ten teratomas were associated with polyhydramnios, three with obstructed labour, five of the fetuses were hydropic and four had malformations at sites distant to the tumor. Twenty teratomas occurred in stillbirths, half of whom were macerated. The vascular tumors were associated with polyhydramnios in three cases and hydrops fetalis in two. The neuroblastomas were all incidental findings. Four were intra-adrenal, one was an adrenal tumor which had metastasized to the liver and the remaining case was a small paravertebral lesion. Two of the three rhabdomyomas were multiple and one of these occurred in a case of tuberous sclerosis. One of the mesoblastic nephromas occurred in a hydropic fetus who had the Arnold-Chiari malformation. The thyroid and hepatic adenomas were small incidental lesions. The cerebellar medulloblastoma had led to marked hydrocephalus. Congenital tumors have different incidence, presentation and behaviour than those in childhood or adult life. The association of congenital tumors with congenital malformations and hydrops fetalis should always be remembered.

Female↗

PubMed2006

[Cardiac tumors in children and adults: A Multicentric retrospective study].

BACKGROUND: Cardiac tumors are very uncommon at all ages. There are important clinical differences between children and adults in the behavior of these tumors. AIM: To compare the behavior of primary and secondary cardiac tumors, from fetal age to adults. PATIENTS AND METHOD: Multicentric retrospective analysis of 38 children and adults with cardiac tumors, evaluated with echocardiography between January 1995 and August 2001. Medical records, echocardiographic and radiological examinations, surgical protocols and pathologic examinations were reviewed. Follow-up was obtained through data on medical records or calling patients by telephone. RESULTS: Tumors were diagnosed in 38 patients (13 children and 25 adults), from a total of 31.800 echocardiograms. In children the diagnosis was made by fetal, transthoracic or transesophageal echocardiography in 23.6% and 8% of cases, respectively. Eighty five percent were primary (10 benign and 1 malignant) and 15%, secondary tumors. Fifty four percent were rhabdomyomas and 75% regressed spontaneously. Seventy seven percent were symptomatic and 31% were treated with surgery. During a follow up of 44+/-35 months, 31% of patients died. In adults, 76% of tumors were diagnosed by transthoracic and 20% by transesophageal echocardiography. Seventy six percent were primary (18 benign and 1 malignant) and 24% secondary tumors. Fifty six percent were myxomas. Ninety two percent were symptomatic and 84% were treated surgically. Twenty percent of patients died in the early postoperative period. No adult patients had a follow-up. CONCLUSIONS: Rhabdomyomas were solely found in children. In adults, myxomas are the predominant cardiac tumors. Primary and metastasic malignant tumors are observed both in children and in adults.

Adult↗

PubMed1999

[Case report of tuberous sclerosis and congenital heart disease].

The cardiac manifestation usually associated with Tuberous Sclerosis is rhabdomyoma. The authors present a clinical case of Tuberous Sclerosis with the particular coexistence of congenital heart disease (mitral anomaly and pulmonary stenosis) and a single intracardiac rhabdomyoma that appeared at the age of four years.

Child, Preschool↗

PubMedDate not supplied

Tuberous sclerosis with visceral organ involvement.

This study is to determine the incidence of visceral organ involvement in tuberous sclerosis (TS). We reviewed 30 cases of TS diagnosed between 1987 to 1997. There were 17 males and 13 females, ages ranged from one day old to 17 years old. Among the 30 cases, 25 patients had seizures and skin manifestations; 24 had cerebral tubercles; 10 had heart involvement (9 rhabdomyoma, 1 dilated cardiomyopathy); 4 had kidney involvement (3 polycystic kidney disease, 1 renal hamartoma); and 3 had retinal astrocytic hamartoma. Based on our study, the most common visceral organs involved were the heart and kidney. Among the ten patients with cardiac rhabdomyoma, six were less than 1 year old (mean age 1.6 +/- 2.2 years old). One newborn presented with a cardiac mass diagnosed by prenatal sonography and another newborn, noted to have tachycardia after birth, underwent surgical intervention due to impending heart failure. Four patients had kidney abnormalities; three were less than 5 years old (mean age 5.2 +/- 5.2 years). During this 10 year period, there was no mortality seen among patients with visceral organ involvement. We suggest that clinicians treating patients with TS should not overlook the visceral organs, especially heart and kidney, which, if involved can have significant morbidity.

Adolescent↗

PubMedDate not supplied

A case of tuberous sclerosis presenting with dysrhythmia in the first day of life.

Cardiac rhabdomyoma (CR) is the most common primary cardiac tumor in childhood. Although CRs are asymptomatic in many cases, they may cause arrhythmia, heart failure and fetal hydrops. Babies with arrhythmia in the neonatal period must be investigated for structural heart disease including CR. Cardiac rhabdomyoma may either present as an isolated tumor or may be related with tuberous sclerosis. Arrhythmia due to CR may be the initial sign of tuberous sclerosis. We report a case of tuberous sclerosis presenting with ventricular premature beats and second-degree atrioventricular block in the first day of life who was found to have multiple CR during echocardiographic examination.

Arrhythmias, Cardiac↗

PubMed1992

[Cardiac rhabdomyomatosis and Bourneville's tuberous sclerosis in the fetus. Apropos of 2 cases].

Two cases of cardiac rhabdomyoma discovered fortuitously at foetal ultrasonography gave rise to no obstructive cardiac signs or arrhythmias either in the antenatal or postnatal periods. These multiple tumours often observed in Bourneville's tuberous sclerosis orientated the clinical investigations to the diagnosis of this disease from the outset. MRI demonstrated cortical tubers and subependymal nodules in both cases. A retinal hamartoma was present in one case. No renal involvement could be detected by ultrasonic examination. No neurological or cardiovascular symptoms appeared during follow-up (20 and 4 months after birth). Investigations in the parents were negative, these two cases being sporadic forms of Bourneville's tuberous sclerosis. The functional prognosis is related to the neurological outcome. Doppler echocardiography would appear to be the best method of following up cardiac rhabdomyomas, and enabled the demonstration of partial regression of the largest tumour in one of these two cases. Cardiac MRI is also an excellent diagnostic tool. As it is usually performed at the same time as cerebral MRI, essential in the follow-up of Bourneville's tuberous sclerosis, it does not represent additional discomfort to the patient.

Adult↗

PubMed2004

Primary cardiac tumours: eighteen years of surgical experience on 21 patients.

BACKGROUND: The literature on primary cardiac tumours is relatively limited because of their rare occurrence. OBJECTIVE: To report the authors' experience with primary cardiac tumours at McGill University teaching hospitals, Montreal, Quebec. In addition, four exceedingly rare primary cardiac tumours are presented--three valve myxomas and the seventh reported case of a primary synovial sarcoma. METHODS: Twenty-one patients resected for primary cardiac tumours were retrospectively reviewed in terms of their clinical presentation, surgical treatment, histopathological findings and outcome. Postoperative success was defined by disease-free survival after resection. A survival analysis was performed using the Kaplan-Meier life-table method. RESULTS: The mean age at the time of resection was 45.5 years (range birth to 74 years). The most common clinical presentation in adults was dyspnea (38%) or central nervous system/embolic phenomena (24%), and in the pediatric group, it was hypoxia (50%). All but one of the tumours were visualized using transthoracic echocardiography. Coronary angiography was performed in 11 patients, with a diagnosis of coronary artery disease in five patients (45%), all of whom underwent coronary artery bypass grafting. The surgical approach was uniatrial in 10 patients (48%)--seven (33%) via the left atrium and three (14%) via the right atrium. Eight patients (38%) required a biatrial approach; the remaining three patients (14%) with ventricular tumours required a ventricular approach. Of the 21 cases, 17 were adults and four were pediatric cases. There were 16 myxomas (76%), two rhabdomyomas (10%), one fibroma (5%), one angiosarcoma (5%) and one synovial sarcoma (5%). In adults, myxoma was the most common primary cardiac tumour (88%), whereas rhabdomyoma occurred most frequently in the pediatric population (50%). Three valve myxomas, two involving the mitral valve and one involving the tricuspid valve, were encountered; all underwent conservative leaflet resection and valve repair. The probability of disease-free survival for all patients was 80.7% (95% CI 63.6 to 97.7) at one year, and 75.6% (95% CI 57.0 to 94.3) at both five and 10 years after resection. For adult benign tumours, the probability of disease-free survival was 93% (95% CI 79.4 to 100.0) at one, five and 10 years. There was a total of five deaths. CONCLUSIONS: The results suggest that complete surgical resection of primary cardiac tumours prolongs symptom-free survival and is associated with a low recurrence rate. Benign primary cardiac tumours have an excellent prognosis in the adult population compared with in the pediatric population (93% versus 50% survival, respectively, in the present cohort). Furthermore, conservative leaflet resection with valve repair of valve myxomas, rather than radical excision and valve replacement, should be strongly considered. Early identification and diagnosis of primary cardiac tumours are necessary for expeditious surgical resection if a cure is desired, especially when encountering malignant primary cardiac sarcomas.

Adolescent↗

PubMed1992

Tumorigenic effects mediated in the avian embryo by one or more oncogenes associated with v-myc.

We have previously shown that introduction of the v-myc oncogene in chick or quail embryos at E3 induces rapidly growing heart rhabdomyomas. We now report that a retrovirus containing one or two other oncogenes induces additional pathologies specified by the v-myc-associated oncogene. The v-mil/myc combination introduced at E3 induces, in addition to heart rhabdomyomas, tumors of proliferating cells aggregated onto the luminal aspect of vessels in both chick and quail embryos. In the quail these cells react positively with the quail-specific mAb QH1, which recognizes endothelial and most hemopoietic cells, while chick intravascular cells do not react with the chick-specific mAb VIA2 that recognizes hemopoietic cells. Thus the v-mil/myc tumors appear to be of endothelial origin. The v-myb-ets/myc combination injected at E3 induces cardiorhabdomyomas and aggressive VIA2-positive hemopoietic tumors in chick embryos, but only the v-myc-induced cardiorhabdomyomas in quail embryos. When injected into hatched animals, v-myc alone transforms hemopoietic and perhaps endothelial cells, but not cardiac cells. Thus the developmental stage at which a cell type can be transformed by v-myc and another associated oncogene depends on as yet undefined species-specific factors. More importantly, several examples of oncogene cooperation in vivo are adduced by these experiments. The type of cell transformed is specified by the viral oncogene combination.

Animals↗

PubMed1989

Difficulty of fetal monitoring in a fetus with intracardiac tumors.

Fetal intracardiac tumors are rare but, when present, are a common cause of poor or uninterpretable fetal heart tracings due to fetal arrhythmias. The most frequently encountered tumor, the rhabdomyoma, is associated with tuberous sclerosis. We present a case demonstrating this difficulty of fetal monitoring in a fetus with an arrhythmia later found to be due to multicentric intracardiac rhabdomyomas. The appropriate evaluation and management of such fetal heart tracings are discussed.

Adult↗

PubMed1985

[Echocardiography detection of intracavitary ventricular tumors].

Intracavitary ventricular tumors were found in 3 male patients (newborn, and 18 and 23 years old) by 2-D echocardiography (metastasis of an osteogenic sarcoma in the right ventricle, teratoma embryonale growing from the retroperitoneum into the right ventricle, rhabdomyoma of the left ventricle). They all showed a uniform echo pattern and two of them were pedunculated. Wall motility, valves and cardiac dimensions were normal. In each case diagnosis was confirmed intraoperatively or at autopsy. Clinically the metastasis of the osteogenic sarcoma presented as obstruction of the superior vena cava, whereas the teratoma embryonale imitated tricuspid valve disease. The rhabdomyoma caused a systolic murmur and severe postpartal sinus bradycardia. It was successfully removed on the basis of echocardiography alone. 2-D echocardiography is a reliable, noninvasive diagnostic tool for evaluation of intracavitary, ventricular tumors.

Adolescent↗