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PubMed1980

Rhabdomyoma of the heart: a diagnostic and therapeutic challenge.

The sector scan was decisive in establishing the diagnosis of multiple rhabdomyomas of the heart in a newborn infant. Successful resection of the tumors was performed with profound hypothermia and limited cardiopulmonary bypass. Nine months later, the infant died of the complications of tuberous sclerosis. This case report documents the diagnostic accuracy of the sector scan and the potential benefit of aggressive surgical management of this condition.

Diagnosis, Differential↗
PubMed1986

Pedunculated left ventricular rhabdomyoma.

A patient with an unusual left ventricular outflow tract obstruction caused by a solitary pedunculated left ventricular rhabdomyoma is described. Diagnosis was based on two-dimensional echocardiographic findings alone. The obstructive portion of the tumor was successfully removed from the interventricular septum by an aortic root approach.

Female↗
PubMed1998

Obstructive rhabdomyoma and univentricular physiology: a rare combination.

We report the successful excision of a large left atrial rhabdomyoma producing complete obstruction of both inflow and outflow to the left ventricle. Systemic perfusion was dependent on anterograde ductual flow. The resultant univentricular physiology was initially managed medically, with spontaneous tumor regression contemplated as a means of possible long-term "cure." Failure to achieve hemodynamic stability compelled urgent surgical excision. This neonate was successfully discharged home with an in-series biventricular circulation.

Heart Neoplasms↗
PubMed1990

Rhabdomyoma of the ear pinna in four cats.

Four cases of rhabdomyoma of the ear pinna in white eared adult cats are reported. The lesions were raised, non-ulcerated, red-purple, discoid nodules on the convex surface of the pinna. Histologically, the nodules consisted of interlacing bundles of spindle-shaped cells that occasionally had cross striations. Lesions have not recurred 2-3 years following surgical excision. Although a specific cause for tumour development was not found, the similarity among these cases suggests that common factors played a role in the development of the lesions.

Animals↗
PubMed1979

Lingual rhabdomyoma (adult variant) in a child.

A case of rhabdomyoma of the adult variant arising in the tongue of an 11-yr-old male is presented. Fifteen previously reported cases were reviewed and compared to our case. This patient is believed to be the only child with this lesion.

Child↗
PubMed1992

Diffuse bilateral glomerulocystic disease of the kidneys and multiple cardiac rhabdomyomas in a newborn. Relationship with tuberous sclerosis and review of the literature.

A case of glomerulocystic disease of the kidneys in a newborn is described. The kidneys were very large and diffusely cystic. Histologically, all portions of the nephron were involved and some of the cysts were lined by a prominent hyperplastic eosinophilic epithelium. This, together with the presence of numerous cardiac rhabdomyomas, a nodular haemangiomatous harmartoma and histiocytoid cell nests in the spleen were strongly suggestive of tuberous sclerosis. The relation between glomerulocystic kidney disease and tuberous sclerosis is discussed.

Diagnosis, Differential↗
PubMed1989

Strokes in tuberous sclerosis: are rhabdomyomas a cause?

Abnormalities of the cerebral arteries and the aorta are more common in young patients with tuberous sclerosis than in the rest of the population. Strokes have been reported but there is no confirmation that embolization of cerebral arteries by fragments of cardiac rhabdomyoma plays a role in the higher incidence of strokes in these patients.

Cerebrovascular Disorders↗
PubMed1988

Rhabdomyoma of the tongue. Report of a case with light microscopic, ultrastructural and immunohistochemical observations.

Adult rhabdomyoma is a benign neoplasm of skeletal muscle origin, which mainly occurs in the head and neck region. Because of its rarity the diagnosis may be missed, especially if cross-striations cannot be demonstrated by light microscopy. By applying supplementary diagnostic evaluation with immunohistochemistry and electron microscopy, the diagnosis is established. Positive staining with desmin unequivocally demonstrates that the tumour cells are derived from muscle tissue. The presence of myofibrils, Z-bands and numerous mitochondria by ultrastructural examination are characteristic features of this tumour.

Humans↗
PubMed1995

Unilateral hearing loss due to a rhabdomyoma in a six-year-old child.

A case report of a six-year-old child is presented, who had had a unilateral sensorineural hearing loss for several years. Because of impairment in the ABR as well as in the caloric testing a MRI and CT scan were performed. A 17 mm tumour in the cerebellopontine angle (CPA) was detected, which after suboccipital surgery proved to be a rhabdomyoma. This tumour has not been described before in the CPA. Unilateral sensorineural hearing loss should, at all ages, be an indication for further (radiodiagnostic) investigations.

Cerebellar Neoplasms↗
PubMed2001

Cardiac rhabdomyoma: a report of alternative strategies to surgical resection.

Cardiac rhabdomyoma represents the commonest primary cardiac tumour. Its natural history is generally favourable, with resolution in the majority of cases. Surgical resection has been described for intractable arrhythmia, severe obstruction of the inflow or outflow tracts sufficient to compromise cardiac output, and systemic embolization. We describe an alternative palliative strategy, which was associated with regression of the tumour.

Echocardiography↗
PubMed2001

Rhabdomyoma of a rare type in a child: case report and literature review.

We describe a 9-year-old boy who presented with dyspnea and with a neck mass which was initially described as a thyroid nodule. At the end of the diagnostic-therapeutic research this mass was finally diagnosed as an adult rhabdomyoma originating from the mediastinum. This is an extremely rare tumor which is usually found in the adult population.

Child↗
PubMed2005

A giant rhabdomyoma with left ventricular inflow occlusion and univentricular physiology.

During a routine prenatal ultrasound a huge tumor was diagnosed located in the left ventricle and left atrium with complete obstruction of the left ventricular inflow tract. On the first day of life, urgent surgical intervention was necessary because of progressive heart failure with univentricular physiology. Intraoperatively, the tumor mass was found to originate from the left ventricular wall, reaching into the left atrium. Histological examination revealed a rhabdomyoma.

Cardiac Surgical Procedures↗
PubMed1986

Congenital intracardiac rhabdomyoma: prenatal detection by echocardiography, perinatal management, and surgical treatment.

A case of a newborn infant with an intracardiac tumor, detected in utero by fetal echocardiography at 30 weeks gestation, is presented. The prenatal evaluation by serial fetal echocardiograms and nonstress tests is described. In the perinatal period, ventricular tachycardia occurred and was successfully managed with antiarrhythmic medications. At 20 months of age, a right ventricular rhabdomyoma was removed at open heart surgery. The patient represents the earliest in utero detection of a fetal intracardiac tumor with successful postnatal outcome.

Adult↗
PubMedDate not supplied

[Echocardiographic diagnosis and successful resection of a left ventricular rhabdomyoma in the neonatal period].

Among the rare heart tumours in childhood rhabdomyomas are mostly found in the neonatal period. They cause in 50% left- or right-sided obstruction. By means of 2D-echocardiography extent and relation to neighbouring structures can clearly be disclosed. Because of the severe natural history - mortality in the first year of life between 60 and 78% - successful operation was performed in a 2,5 kg weighing neonate.

Echocardiography↗
PubMed2003

Left ventricular outflow obstruction in rhabdomyoma of infancy: meta-analysis of the literature.

Primary heart tumors are exceptional in infants and children. Most common is the rhabdomyoma, often associated with tuberous sclerosis (Bourneville's disease). This tumor is generally believed to have no hemodynamic effects in the majority of cases. Recently, severe obstruction of the left ventricular outflow tract by a solitary tumor was diagnosed during pregnancy and emergency surgery was needed soon after birth.

Female↗
PubMed1987

The usefulness of Doppler echocardiography in the management of rhabdomyoma diagnosed prenatally.

Cardiac rhabdomyoma diagnosed prenatally by cross-sectional echocardiography was confirmed after birth by Doppler echocardiography and cardiac catheterization in a baby with tuberous sclerosis. Doppler echocardiographic follow-up after 2 years revealed regression of tumour masses and concomitant disappearance of pulmonary regurgitation which was caused by the large tumour mass in the right ventricular outflow tract. In spite of a sizeable mass protruding into the left ventricular outflow tract, repeated Doppler examination failed to demonstrate any significant obstruction, thus avoiding repeated catheterizations and unnecessary surgery. Doppler examination of ventricular inflow revealed normal filling characteristics. Doppler and cross-sectional echocardiography was of great value in the management of this patient.

Echocardiography↗
PubMed1998

Adult rhabdomyoma of the lip.

A 75-year-old man presented with a slowly enlarging crust on the left lower lip. Beneath the crust, there was a palpable nodule approximately 1 cm. A superficial punch biopsy demonstrated an actinic keratosis of the lip. A vermillonectomy with a wedge-shaped excision of the nodule was performed because invasive squamous cell carcinoma was suspected. Histologically, the tumor proved to be an adult rhabdomyoma with an overlying actinic keratosis. The tumor cells were positive for desmin and muscle-specific actin and negative for vimentin, alpha-smooth muscle actin (alpha-SMA), and S100 protein. Some areas of cross-striation were present. Only very rarely are dermatologists confronted with these slow-growing tumors with skeletal-muscle differentiation, occurring predominantly in the head and neck area of older adults. The histologic differential diagnosis includes granular cell tumor, hibernoma, oncocytoma and paraganglioma.

Aged↗