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PubMed1989

[Clinical study of fetal echocardiography].

92 fetuses from 52 women who were about to undergo midtrimester termination of pregnancy and 40 fetuses at high risk were studied by M-mode, 2D and pulsed Doppler echocardiography. One fetus of 24 weeks gestation was found to have a cardiac tumor. The pathological diagnosis was rhabdomyoma. The right ventricular tumor was located between the tricuspid and septum. It obstructed the tricuspid valve and caused tricuspid regurgitation and bradycardia. The measurement of atrial and ventricular dimensions by fetal echocardiography in 91 normal fetuses showed an average RV/LV ratio of 1.11. Doppler velocities of mitral and tricuspid valves showed a Peak A/peakE ratio of 1.

Blood Flow Velocity↗
PubMed1986

Comparative pathology of cardiac neoplasms in humans and in laboratory rodents: a review.

This review deals with the frequency, heredity, and morphology of 19 different histologic types of cardiac tumors that may affect humans, rat, mouse, guinea pig, and mastomys. Chemicals, durable fibrous materials, viruses, and probably irradiation have induced cardiac tumors in rodents. Apart from the involvement of asbestos in the induction of pericardial mesothelioma, no carcinogens have been identified in induction or promotion of cardiac tumors in humans. A hereditary tendency for the occurrence of myxoma and aortic paraganglioma has been indicated in humans as well as strain-specific heredity for rhabdomyoma in the guinea pig and for atriocaval node tumor in the rat.

Animals↗
PubMed1985

[Cardiac tumors with neonatal disclosure. Apropos of a case with spontaneously favorable development].

The authors report the case of a cardiac tumour documented by 2 D echocardiography, presenting in the newborn with cardiac arrest. The echocardiographic features of multiple nodules disseminated in the ventricular walls suggested a diagnosis of rhabdomyoma. The initial course was complicated by poorly tolerated attacks of tachycardia which were rapidly brought under control with amiodarone. The long term outcome was clinically favourable with a rapid regression of the number and size of the tumours on echocardiography, and a tendency to normalisation of the electrocardiogramme. The possibility of regression, which has already been reported by many investigators, suggests that these cardiac tumours may have a better prognosis than previously thought.

Echocardiography↗
PubMed1977

Diffuse rhabdomyomatosis of the heart.

The myocardium of a 13-year-old boy was infiltrated with cells like those of classical congenital rhabdomyoma. In contrast to the latter condition, in which distinct nodules are present, our patient had an infiltrated myocardium without a distinct tumor formation. The condition is similar to that reported earlier and may be designated as diffuse rhabdomyomatosis.

Adolescent↗
PubMed1984

Cardiac tumors. Clinical spectrum and prognosis of lesions other than classical benign myxoma in 20 patients.

Between 1961 and 1983, 20 patients underwent operation at the Texas Heart Institute for primary tumors other than classical benign myxoma. Total follow-up was 70.7 patient years (mean 3.5 years). There were 10 adults and 10 children under 12 years of age. There were two operative deaths and four late deaths. All of the pediatric patients had benign lesions (five fibromas, five rhabdomyomas), and only one patient in this group died (during the operation). All operative survivors are alive between 0.9 and 18 years postoperatively, although in three cases excision was incomplete. Of the 10 adult patients, five had benign lesions; all were completely excised. In the other five adult patients, unresectable malignant tumors were found, and all four operative survivors died of metastatic disease within 8 months. Benign cardiac tumors in childhood have an excellent prognosis when completely excised and appear to have a good short-term prognosis even when excision is incomplete. Although prognosis for benign tumors in adults is good, malignant tumors are associated with very poor survival.

Adolescent↗
PubMed1984

[Incidence of primary heart tumors].

Report on 9 primary tumours of the heart as well as of pericardium (3 sarcomas, 3 myxomas, 1 rhabdomyoma as well as 1 mesothelioma and 1 lipoma). Discussion of histology, complications, diagnostics and therapy of these neoplasias on the basis of own material as well as of literature.

Aged↗
PubMedDate not supplied

Tumors of the heart: surgical considerations.

A series of 23 cardiac tumors is reported. Six were diagnosed at autopsy; 17 tumors were surgically explored. Eleven of the 17 were myxomas within the left atrium. Eight of the 17 patients presented with congestive heart failure; peripheral and cerebral emboli were also common. The diagnosis was made preoperatively in 10 patients. Two-dimensional echocardiography was the most reliable diagnostic tool. Follow-up averages 55 months; there have been no late deaths or recurrences. The other six surgically treated tumors were: a left ventricular rhabdomyoma, a septal lipoma, a right atrial calcified endocardial mass, a right ventricular fibrosarcoma, a rhabdomyosarcoma, and a sarcoma metastatic to the pericardium and right atrium. From this series and a review of the literature, we concluded that: benign cardiac tumors can usually be excised with a low morbidity and excellent long-term results; malignant cardiac tumors have a dismal prognosis, and operation is primarily diagnostic; tumors metastatic to the heart should be operated upon only if successful palliation seems possible.

Adult↗
PubMed1980

Primary cardiac tumors in children.

Case histories of five children with primary cardiac tumors are presented. The clinical and pathological features of the most frequent cardiac neoplasms in childhood are also reviewed. Cardiac tumors should be suspected in any child with unexplained heart murmurs, congestive heart failure, or cardiac arrhythmias. Echocardiography, cardiac catheterization, and biplane cineangiograms provide diagnostic confirmation. Prompt surgical resection is indicated in all symptomatic patients and in those with significant inflow and outflow tumor-related obstruction. Complete resection is not possible in many patients with cardiac rhabdomyoma or lipoma but removal of the obstructive portion of the tumor frequently provides long-term symptomatic relief.

Child↗
PubMed1995

Multiple cardiac masses diagnosed with prenatal ultrasonography in the fetus of a woman with tuberous sclerosis.

Cardiac rhabdomyomas are associated with tuberous sclerosis, but their identification in utero is uncommon. The authors report a case of multiple cardiac masses discovered in utero by prenatal ultrasonography at about 30 weeks gestational age. Follow-up included neonatal echocardiography, ultrasonography and computed tomography of the head. The differential diagnosis of echogenic intracardiac masses, as well as their management, is discussed.

Adult↗
PubMed1995

[Differential diagnosis of non-epithelial rectal neoplasms].

Experience in the treatment of 118 patients with nonepithelial new growths of the rectum has been accumulated at the Scientific Research Institute of Proctology in the period from 1970 to 1991. They accounted for 1.5% of all benign and malignant tumors of this organ. Leiomyoma was diagnosed in 44, leiomyosarcoma in 18, lipoma in 30, fibroma in 17, neurinoma in 7 and rhabdomyoma in 2 of these patients. A differential diagnosis was made between benign and malignant tumors, as well as with other nonepithelial new growth of the rectum. On the basis of the clinical picture and the results of digital and instrumental (radiological, endoscopic, and ultrasonic) examination of the rectum the correct preoperative diagnosis may be established with certain probability. This influences directly the choice of the volume of the operative intervention in various types of nonepithelial rectal tumors. Morphological verification of the tumor is the principal method of examination in establishing the differential diagnosis in the preoperative and postoperative periods.

Adult↗
PubMed1994

[Primary heart neoplasms].

The work analyses the anatomomorphological characteristics of primary tumors of the heart in 106 patients; in 101 patients the tumor was benign (myxoma, neurinoma, rhabdomyoma) and in 5 malignant (angiosarcoma, liposarcoma). Surgical treatment was conducted in 99 patients; hospital lethality was 7.1%. Six patients who refused an operation died at other therapeutic institutions, one patient died on admission to the clinic from occlusion of the left atrioventricular orifice by the tumor. It is pointed out that the diversity and the various degree of the clinical manifestations of the disease are predetermined by the localization and morphological characteristics of the new growths, their size, and mobility. The authors emphasize that knowledge of the anatomomorphological features of primary tumors of the heart and their early diagnosis are conducive to a differential approach in each concrete situation and to the choice of the optimal volume of an urgent surgical intervention.

Adolescent↗
PubMed1993

Surgical treatment of infantile cardiac tumor: report of a case.

We herein report on a male infant who was diagnosed as having a cardiac tumor at the age of four days. This baby was quite well after birth, with the exception of a heat murmur heard by the pediatrician in a routine physical examination. Echocardiograms disclosed a tumor located in the muscular portion of the interventricular septum, almost occupying the whole right ventricular chamber. The patient underwent near total excision of the tumor to relieve the right ventricular outflow obstruction when he was 54 days old. The pathologic report revealed a rhabdomyoma.

Heart Neoplasms↗
PubMed1975

[Heart tumors in children. Report of 3 cases with favorable spontaneous courses].

During a 14-year period, 8 cases of primary heart tumours were observed at Sainte-Justine Hospital. Three of these patients had a favourable course without any surgical treatment. The age of these patients was respectively 3 days, 7 weeks and 6 1/2 years. In what concerns the first two patients, the clinical and paraclinical pictures were suggestive of heart disease from the onset. Catheterization and angiocardiography confirmed the presence of a tumour deforming both ventricular cavities. In these two cases, an attempt at surgical resection proved to be impossible in view of the extent of the lesion. Biopsy demonstrated a rhabdomyoma in one of the patients and a diffuse fibroma in the other. Six and four years later, the patients were still alive, and an improvement of both the electrocardiogram and of the cardio-pulmonary X-ray pictures were noted. A second cardiac catheterization showed an almost complete disappearance of the pathological pictures. In what concerns the third patient, he was a 6-year old child with a classical Bourneville's tuberous sclerosis with a localized tumour at the junction of the superior vena cava and the right atrium. Three years later a control catheterization showed the tumour to have remained unchanged. Two conclusions might be drawn from these cases: 1 a surgical operation, although always indicated, should never entail a desperate attempt at tumour removal; 2 the prognosis should never be considered as lethal from the start.

Child, Preschool↗
PubMed1996

Primary cardiac tumors in infants and children: immediate and long-term operative results.

BACKGROUND: The literature contains few large or long-term series involving infants and children with primary cardiac tumors. This article summarizes our 35-year experience with such lesions. METHODS: Between January 1961 and January 1996, 40 infants and children (mean age, 3.3 years; range, 2 days to 17 years; 65% female) were diagnosed at our institution with primary cardiac tumors. Of these tumors, 37 (92%) were benign and 3 (8%) were malignant. Tumors were resected in 38 patients (95%). In 2 patients (5%), biopsy confirmed rhabdomyoma; however, presenting symptoms spontaneously resolved, so these patients did not undergo tumor resection. Follow-up echocardiographic studies showed a diminishing tumor mass in each of these patients. RESULTS: Immediate, symptom-free status was achieved in all patients. There were two early deaths, for an operative mortality of 5%. Three late postoperative deaths (7.5%) occurred as follows: 1 patient with a myocardial hamartoma died at 3 months of congestive heart failure. Another patient with a recurrent rhabdomyosarcoma died at 6 months, and a third patient with a recurrent fibrosarcoma died at 28 months. Long-term follow-up was available for 34 survivors (97% complete) and totaled 240.2 patient-years (mean, 7.1 years/patient). All remaining survivors were without evidence of presenting symptoms and tumor recurrence or progression. CONCLUSIONS: The data suggest that an aggressive operative approach is warranted for benign symptomatic and malignant tumors. This aggressive approach has resulted in extended symptom-free status in patients with benign lesions, and significant palliation and longer survival in patients with malignant lesions, with acceptably low operative risk.

Adolescent↗
PubMed1996

Allelic loss is frequent in tuberous sclerosis kidney lesions but rare in brain lesions.

Tuberous sclerosis (TSC) is an autosomal dominant disorder characterized by seizures, mental retardation, and hamartomatous lesions. Although hamartomas can occur in almost any organ, they are most common in the brain, kidney, heart, and skin. Allelic loss or loss of heterozygosity (LOH) in TSC lesions has previously been reported on chromosomes 16p13 and 9q34, the locations of the TSC2 and TSC1 genes, respectively, suggesting that the TSC genes act as tumor-suppressor genes. In our study, 87 lesions from 47 TSC patients were analyzed for LOH in the TSC1 and TSC2 chromosomal regions. Three findings resulted from this analysis. First, we confirmed that the TSC1 critical region is distal to D9S149. Second, we found LOH more frequently on chromosome 16p13 than on 9q34. Of the 28 patients with angiomyolipomas or rhabdomyomas, 16p13 LOH was detected in lesions from 12 (57%) of 21 informative patients, while 9q34 LOH was detected in lesions from only 1 patient (4%). This could indicate that TSC2 tumors are more likely than TSC1 tumors to require surgical resection or that TSC2 is more common than TSC1 in our patient population. It is also possible that small regions of 9q34 LOH were missed. Lastly, LOH was found in 56% of renal angiomyolipomas and cardiac rhabdomyormas but in only 4% of TSC brain lesions. This suggests that brain lesions can result from different pathogenic mechanisms than kidney and heart lesions.

Alleles↗
PubMed1996

[Colonic conduit: an alternative to the treatment of urinary incontinence].

We report on 9 patients where on a colon conduit has been constructed. Seven of them suffered from a bladder etrophy, one had a sacral teratoma and the remaining one a vesical rhabdomyoma. The latter two had undergone total cystectomy. This technique involves creation of a conduit with isolation of 10-15 cm of a loop of sigmoid colon. One of its ends is sutured and both ureters become reimplanted with an antireflux procedure. The other end is exteriorized through the skin. Thereafter, once any reflux or uretero-colonic stenosis has been ruled out, the cutaneous stoma is detached and anastomose to the sigmoid colon. Complete technique has been used on six patients. Three of them were operated on two stages with no complications. One patient received one uretero-ureteral anastomosis. Another one had an antireflux technique together with anastomosis between the colon conduit and the sigmoid colon in a one-stage procedure. This same patient had a continent reservoir created with a portion of stomach and the colon conduit. In three cases only the first stage of this operation has been performed and the skin stoma has not been closed because of persisting faecal incontinence. Currently 6 patients are continent (2 of them remain with occasional nocturnal incontinence). The remainder have still an open stoma. Renal function and acid-base balance are normal in all of these patients. Three of them are on bicarbonate support therapy.

Atrophy↗
PubMedDate not supplied

[Primary cardiac tumors in childhood: Three case reports in early infancy (author's transl)].

Three cases of primary cardiac tumors in early infancy are reported: 1) An intrapericardial teratoma which manifested as neonatal respiratory distress, cardiac enlargement and normal electrocardiogram. Diagnosis was not made during life. 2) A fibroma was diagnosed because of the presence of a greatly enlarged heart associated to ventricular tachycardia. Angiocardiography showed the tumor localized in left ventricle. Extirpation was attempted but resulted impossible. 3) A rhabdomyoma simulating severe pulmonic stenosis. Multiple tumors were detected by angiocardiography; only some of which could be extirpated. Histopathological examination was carried out on all the patients and a complete post-mortem examination on two of them. An extensive revision of the three types of tumors is described.

Female↗
PubMed1980

Fibromuscular hamartoma of the esophagus in an infant.

An 11-month-old infant had a history of dysphagia and continuous drooling without evidence for airway obstruction. A biopsy specimen of a neck mass on the left side adherent to the larynx and esophagus demonstrated pathologic features characteristic of a fibromuscular hamartoma. Upper airway obstruction subsequently ensued and the patient was managed for 16 months with a tracheostomy and feeding gastrostomy. Although posing an extremely difficult management problem, this benign tumor was surgically removed without damage to the larynx when the patient was 28 months old. The differential diagnosis of this unusual lesion must consider congenital fibromatosis and fetal rhabdomyoma.

Biopsy↗