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At least 217 records · Page 12Linked to original sources
PubMed1987

Rhabdomyoma of the soft palate. Fine structural details of a highly differentiated muscle tumor.

An adult rhabdomyoma was light and electron microscopically studied. The lesional cells presented well-known structural details, such as abundance of mitochondria and glycogen, myofilaments and myofibrils, hypertrophied Z-bands and masses of Z-band material. Triads were randomly scattered in the cytoplasm and also related to the individual sarcomeres. In sarcomeres the triads were regularly placed near to the A-I-junctions. This peculiarity of mammalian skeletal muscle fibers may yield a criterion to distinguish between cardiac and extracardiac rhabdomyomas. Circumscribed surface areas of the tumor cells were provided with elaborate infoldings of plasma membrane and basal lamina. These areas were interpreted as imitating myotendinous junctions. Satellite cells were regularly found.

Aged↗
PubMed1987

[Benign tumors of the posterior urethra in children. Apropos of an unusual case of rhabdomyoma of fetal type].

The authors report a very unusual case of urethral tumor in a child: fetal rhabdomyoma, after nephroblastoma treated by enlarged nephrectomy. Excellent result is observed four years after endoscopic treatment of this prostatic urethral tumor. Clinical and histological features of posterior urethral tumors and rhabdomyoma are recalled; the association nephroblastoma-tumor of urethra is discussed.

Humans↗
PubMed1988

[Neonatal cardiac rhabdomyoma and phakomatosis].

A large cardiac rhabdomyoma was discovered before delivery in a neonate, whose outcome was fatal. The baby and her mother had both cutaneous lesions suggestive of tuberous sclerosis, and the mother's sister had died from Von Hippel Lindau disease. The association of a cardiac rhabdomyoma and a tuberous sclerosis in the same patient is not uncommon, but the existence of Von Hippel Lindau disease in a member of the same family seems to be exceptional.

Angiomatosis↗
PubMedDate not supplied

Fine needle aspiration cytology of a rhabdomyoma of the pharynx.

The fine needle aspiration (FNA) cytology of an extracardiac adult rhabdomyoma of the pharynx is documented. The smear of the aspirate contained large cells with peripherally located, uniform, round nuclei and abundant granular eosinophilic cytoplasm. In addition to these features, peripheral vacuoles were seen in sections of a cell block prepared from the aspirate. Special stains of the cell block sections and of the surgical specimen showed cross striations in some cells while electron microscopy showed the typical features of adult rhabdomyoma. The findings in this case suggest that FNA can diagnose this rare, benign neoplasm when it presents as a solitary mass in the head and neck region. Its differential diagnosis from other neoplasms that occur in that area is discussed.

Aged↗
PubMed1986

Oncocytic change in adult rhabdomyoma.

An adult rhabdomyoma was studied with the light and electron microscopes. The source of eosinophilia of the cytoplasm on light microscopy can be attributed to two components: a filamentous and a granular components. Ultrastructural studies displayed numerous banded myofilaments and mitochondria exhibiting varying degrees of abnormalities. These mitochondrial alterations were similar to those described in oncocytes and oncocytomas. The latter morphologic findings suggest that the mitochondrial abnormalities in adult rhabdomyomas represent an oncocytic change and that they are an integral part of the neoplastic process. Though primarily affecting epithelial cells, oncocytic change, may, under unusual circumstances, involve mesenchymal cells.

Aged↗
PubMed1986

[Cardiac rhabdomyoma in familial tuberous sclerosis].

Case report of a previously healthy girl who presented at the age of 9 months a paroxysmal supraventricular tachycardia. Echocardiography revealed multiple cardiac tumors. The detection of a few hardly visible unpigmented patches of skin allowed the diagnosis of tuberous sclerosis with cardiac rhabdomyomas. Computerised tomography of the brain showed typical calcifications and multiple hypodense parenchymal lesions. In the further course of the disease, progressive multifocal epilepsy and severe retardation of psychomotor development occurred, angiofibromas appeared on the face, and a suspected angiomyolipoma on renal sonography. A very mild form of this autosomal dominant phacomatosis could be detected in the patient's mother. As cardiac rhabdomyomas and tuberous sclerosis are frequently associated, investigations for both these findings with modern methods of body imaging are recommended in order to allow early diagnosis and genetic counselling.

Echocardiography↗
PubMed1986

[Diagnosis and treatment of cardiac rhabdomyomas].

A diagnosis of primary cardiac tumors was achieved by two-dimensional echocardiography in 3 patients. Two of them were female neonates, born of the 29th and 40th gestational week who had shown intrauterine arrhythmias. Subsequently, we were able to diagnose tuberous sclerosis in both. The third child, a 121/2 year old boy, with already diagnosed tuberous sclerosis, presented an electrocardiographical preexcitation syndrome. Because of the multiplicity of the tumors and the association with tuberous sclerosis we presumed that the tumors were rhabdomyomas. As it is known that rhabdomyomas show no, or only a minimal postnatal growth, we decided neither to perform a heart catheterization nor to treat them surgically unless the children show hemodynamical deterioration, although the tumors were huge in both neonates. The subsequent 14 months showed a clear regression in the size of the tumors. One of the neonates was successfully treated with amiodarone because of ventricular tachycardia. The second neonate didn't require any treatment. We conclude that: Surgical management is only necessary in patients presenting with cardiac insufficiency and/or arrhythmias that are resistant to conventional medical treatment. Primary cardiac tumors should be looked for in patients presenting with arrhythmias, even when occurring already during intrauterine life. Diagnostic measures in order to diagnose tuberous sclerosis in patients with cardiac tumors are necessary and vice versa.

Adolescent↗
PubMed1986

[Cardiac rhabdomyomas and aortic valve atresia: an unusual association].

Necroptic examination of a male newborn baby revealed multiple anomalies of the heart, including multiple cardiac rhabdomyomas, aortic valvular atresia, left cardiac hypoplasia, hypoplasia of proximal aorta and hypertrophy of the right heart. Tuberous sclerosis was not present. In are instances, the association of cardiac rhabdomyomas and other congenital cardiac malformations have been reported but the association documented here has not been, to our knowledge, previously described.

Aortic Valve↗
PubMed1985

Surgical treatment for rhabdomyoma of the right atrium causing arrhythmias.

Rhabdomyomas of the heart occur as multiple lesions in 90% of patients so affected, and 80% of the patients die in the first year of life. A 21-month-old child presented with a large isolated right atrial rhabdomyoma causing a supraventricular tachyarrhythmia. Surgical excision cured the arrhythmia.

Arrhythmias, Cardiac↗
PubMed1981

Adult rhabdomyoma: ultrastructure and immunocytochemistry.

Adult rhabdomyoma is a benign, highly differentiated tumor of skeletal muscle. We observed transverse tubules and triads beside myofilaments in a rhabdomyoma from the submandibular area of a 66-year-old woman. Several types of muscle cells were present. In one, mitochondria abounded; in another, muscle filaments predominated. Myoglobin was also detected in the tumor.

Aged↗
PubMedDate not supplied

Rhabdomyoma of the heart: surgical treatment.

Rhabdomyoma of the heart may cause severe hemodynamic obstruction specially in infants. Medical treatment carries a 100% mortality, but there are no clear surgical indications for these patients. Accordingly we reviewed the world literature (30 operated patients) adding our own experience (1 successfully operated patient) Based on our data and that of other we conclude: (1) Severely ill patients with rhabdomyoma should be operated upon. Age or associated tuberous sclerosis are not surgical contraindications. (2) Removal of the hemodynamic obstruction should be the main surgical goal. Radical resection of the tumor is not necessary and may be dangerous. (3) Surgical mortality is acceptable and it is becoming lower with time. (4) Long-term outcome for successfully operated patients is unknown.

Angiography↗
PubMed1981

Sudden unexpected death due to asymptomatic cardiac rhabdomyoma.

A case of cardiac rhabdomyoma producing sudden unexpected death in an asymptomatic six-year-old boy is reported. Cardiac rhabdomyoma is a rare tumor, and its ability to cause sudden unexpected death in children beyond infancy is not generally known. The great majority of afflicted patients die in the early infancy; few survive beyond five years of age. The signs and symptoms present in most cases are said to be due to interference of interaction blood flow or to interruption of the cardiac conduction system. Numerous cases have been clinically misdiagnosed because of this tumor's ability to mimic other cardiac disease.

Child↗
PubMed1978

[Rhabdomyoma of the soft palate (author's transl)].

A case of a rhabdomyoma of the soft palate of a 50-year-old woman is reported. The histological characteristics of this rare tumor are demonstrated by light and electron microscopic studies. The findings are compared with the criteria of extracardiac rhabdomyoma cases described in the literature.

Adolescent↗
PubMed1995

Adult rhabdomyoma: presentation as a cervicomediastinal mass (case report and review of the literature).

The adult rhabdomyoma (ARM) is an unusual and extremely uncommon tumor. Only 96 cases have been reported. Adult rhabdomyomas are found most often in the region of the base of the tongue, floor of the mouth, larynx, and pharynx. Uncommon locations include the soft palate, uvula, lip and cheek, orbit, and stomach. One prior case of extension of an ARM from the supraclavicular region into the mediastinum has been described as an incidental finding at autopsy. It is important to be aware of and correctly identify this tumor because total excision may be curative. Follow-up must be long-term, as recurrence can occur after 35 years. The authors present a case of an ARM diagnosed during the evaluation and treatment of a symptomatic mediastinal mass. This ARM was found to involve the esophagus and trachea from the inferior constrictors in the neck to the azygous vein in the mediastinum. This case represents a previously undescribed presentation of an ARM. We present a review of ARM and outline our diagnostic, therapeutic, and follow-up plans.

Head and Neck Neoplasms↗
PubMed1993

[The complete regression of multiple cardiac rhabdomyomas in childhood].

Rhabdomyoma, even if rare, is the most common primary cardiac tumor in infancy and childhood. We present the case of a newborn in whom several intramural and intracavitary cardiac masses were found at birth by two-dimensional echocardiography, and showed complete regression at the follow-up examinations. Except for supraventricular arrhythmias, there were no symptoms or signs of cardiac involvement at birth: the baby was acyanotic and is no apparent distress; no murmur was detected and chest roentgenogram revealed normal heart size; association with Tuberous Sclerosis was excluded. Despite major anatomical cardiac anomaly, neither in-flow nor out-flow tract obstruction was found by Doppler investigation and therefore a noninterventional approach was preferred. At the two-year follow-up control the baby was asymptomatic and the echocardiogram revealed complete regression of cardiac tumors. Our report confirms the possible favourable outcome of Rhabdomyoma and the utility of echocardiography in these patients' follow-up.

Child, Preschool↗
PubMed1993

[Right ventricular rhabdomyoma causing pulmonary stenosis].

The authors report a case of rhabdomyoma of the right ventricular cavity with outlet obstruction in a three-months old infant. The diagnosis was established by echocardiography. Bidimensional echocardiography study showed a polypoid tumor of the right ventricle. During the systole it was projected through the pulmonary valve, obstructing part of it. The Doppler study showed an important gradient between the right ventricle and the pulmonary artery. The resection was performed with success. The diagnosis of cardiac rhabdomyoma was made by microscopic study. It is emphasized the importance of the echocardiographic study in an infant with oligosymptomatic cardiac murmurs.

Echocardiography↗
PubMed1993

[Successful operation for isolated cardiac rhabdomyoma of the right atrium].

A 12-year-old girl has experienced palpitation and dizziness, and presented atrial flutter with maximal heart rate of 220/min. Two-dimensional echocardiogram and chest MRI revealed a mass attached to the inter-atrial septum. Complete excision of the tumor including the atrial septum was performed without damage to tricuspid valve. The interatrial septal defect was closed primarily. Histology of the tumor was compatible with cardiac rhabdomyoma. Sinus rhythm was present and no tachycardia occurred in the postoperative period. On the literature, only one case of isolated cardiac rhabdomyoma developing in the atrium was reported and this is considered as the oldest operative case in Japan.

Child↗
PubMed1993

[Surgical treatment of the primary cardiac rhabdomyoma in the neonate--a case report].

A successful surgical treatment of intracardiac rhabdomyoma in a 12-day-old female is reported. The neonate was first noted to have a cardiac murmur and she was diagnosed cardiac tumor by two dimensional echocardiography. It showed multiple cardiac tumors which occupied the right ventricular outflow tract and interventricular septum. Under the routine cardiopulmonary bypass the tumor of the right ventricular outflow tract was removed. Histological examination revealed a rhabdomyoma. Follow-up evaluation 7 years after operation showed normal growth and development of the patient. A repeated two dimensional echocardiogram revealed no sign of recurrence and the left ventricular cavity was not obstructed by the residual tumor in the interventricular septum. We reviewed 10 cases treated surgically in infancy including three neonate in Japan.

Echocardiography↗