Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “RHABDOMYOMA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 73 records · Page 4Linked to original sources
PubMed1991

Cardiac rhabdomyoma. Rare cause of fetal death.

Two cases of nonimmune hydrops fetalis and fetal death associated with cardiac rhabdomyoma are reported. Case 1 presented with fetal supraventricular tachycardia, and cardiac rhabdomyoma was accurately diagnosed by fetal echocardiography. Autopsy revealed multiple rhabdomyomata involving the right atrial free wall, the sinoatrial node, and the left ventricle. The left circumflex coronary artery was extrinsically compressed by adjacent tumor tissue, causing left ventricular myocardial infarction. Case 2 had a unique, pedunculated, ball-like rhabdomyoma that almost totally occluded the mitral orifice. The causes of fetal death in patients with cardiac rhabdomyoma are analyzed and the possibility of fetal surgical management is proposed.

Female↗
PubMed2002

Prenatal diagnosis and postnatal outcome of cardiac rhabdomyomas.

AIMS: To evaluate the sonographic appearance of suspected fetal cardiac rhabdomyomas and their evolution until delivery and in the postnatal period. METHODS: The study group consisted of 6 patients at 23-37 weeks of gestation referred to our Ultrasonic Unit, between March 1992 and December 1998, for suspected fetal cardiac rhabdomyomas. RESULTS: The cardiac tumors were single in three cases and multiple in the other cases. The size ranged from 11 to 47 mm. In two cases the tumors arose from the right ventricle, in one case from the interventricular septum and in three cases from the left ventricle. Five infants are alive and in satisfactory hemodynamic compensation, but three of them developed tuberous sclerosis. In two infants a regression in the maximum diameter of the tumor masses has been observed. One child underwent surgical treatment at the age of six months and the baby died after surgery. CONCLUSIONS: Two-dimensional and Doppler echocardiography are useful non invasive methods to diagnose fetal cardiac rhabdomyomas and to monitor their influence on the fetal cardiac function. However they do not allow us to recognize which fetuses presenting with features compatible with rhabdomyomas will develop tuberous sclerosis.

Adult↗
PubMed1999

Left ventricular rhabdomyoma. A case report.

BACKGROUND: Cardiac tumours in infancy are rare, with a predominant incidence of rhabdomyoma. Diagnosis can best be obtained by pre-cordial and transoesophageal echocardiography. Because of the tendency for spontaneous regression conservative therapy is generally recommended. A rare indication for surgical treatment is described. METHODS EXPERIMENTAL DESIGN: case report. SETTING: Pediatric cardiology and cardiac surgery in a university hospital. PATIENT: a case of a rhabdomyoma of the left ventricle is described which was attached to the left ventricular outflow tract and the left coronary cusp of the aortic valve, causing outflow obstruction of the left ventricle. INTERVENTION: the tumour was removed surgically using cardiopulmonary bypass. MEASURE: achievement of relief of left ventricular outflow obstruction. RESULTS: Immediate and complete relief of the left ventricular outflow tract was achieved with preservation of the aortic valve. The patient recovered well. CONCLUSIONS: Cardiac rhabdomyomas can usually be treated conservatively. Surgical therapy can be successfully applied when rhabdomyoma results in hemodynamic problems or arrhythmias.

Echocardiography, Transesophageal↗
PubMed2004

Fetal rhabdomyoma in a neonate.

Rhabdomyoma is a rare benign tumour, majority arising from the cardiac muscle. Seventy to 90 percent of extra cardiac rhabdomyomas are found in the head and neck region, usually within the upper aero digestive tract. We report a case of rhabdomyoma of anterior neck in a neonate. Although rhabdomyomas of posterior neck have been reported, those reported in anterior triangle are infrequent. The lesion has not recurred one year after complete excision. There are no similar reports in Indian literature.

Head and Neck Neoplasms↗
PubMed2006

A rare case of rhabdomyoma of the larynx causing airway obstruction.

Rhabdomyomas are benign tumors of skeletal muscle derivation. Rhabdomyomas of the head and neck are unusual, and laryngeal rhabdomyomas of the adult type are rare. When they do occur, patients may present with symptoms that have progressed slowly over several years. In extraordinary cases, stridor and airway obstruction manifest. We report a case of adult rhabdomyoma of the larynx that features some of its typical and atypical presenting characteristics. This case also exemplifies the favorable prognosis that results following complete yet conservative surgical excision.

Aged↗
PubMed1989

[Echocardiographic study of heart rhabdomyoma in tuberous sclerosis].

In order to study the prevalence of cardiac rhabdomyoma in tuberous sclerosis using non invasive methods, 11 consecutive patients affected by tuberous sclerosis (age: 3 months-22 years, mean 6 years) were examined between January 1984 and April 1987. In each patient clinical examination, 12 lead electrocardiogram, M-mode and two-dimensional echocardiogram were performed: on 7 of them a 24-hour ambulatory electrocardiogram was also performed. Clinical examination revealed a systolic murmur (grade 3/6) in two cases. In one of them it was associated with cyanosis and cardiac failure. None of other patients showed signs or symptoms related to the heart during clinical examination. ECG was abnormal in 3 cases (ventricular pre-excitation in 2 cases and left ventricular strain in 1). Two-dimensional echocardiogram showed single or multiple intracardiac masses suggestive of cardiac rhabdomyoma in 8 cases; in 3 of them masses were isolated, intramural, and in the interventricular septum, from 5 to 16 mm in diameter; in the other 5 cases they were multiple, intramural and endoluminal, in the interventricular septum, in the right or left ventricle, from 7 to 20 mm in diameter. Ambulatory electrocardiogram revealed only isolated ectopic supraventricular and ventricular beats in 2/7 cases. Each patient was clinically controlled every six months using ECG standard and two-dimensional echocardiogram. The mean follow-up period was 32 months (range 9-53). This study confirms the usefulness of the two-dimensional echocardiography to visualize intramural or intracavitary masses due to cardiac rhabdomyoma in patients with tuberous sclerosis, especially when they appear clinically asymptomatic. Two-dimensional echocardiography is probably the ideal method to use in order to evaluate potential increase in cardiac rhabdomyoma, when prolonged follow-up studies are performed.

Adult↗
PubMed1989

[Resection of a cardiac rhabdomyoma of the right and left ventricular outflow tract].

Rhabdomyomas are benign cardiac tumors often occurring as multiple lesions. We describe an infant with multiple cardiac rhabdomyomas causing severe right-ventricular and left-ventricular outflow tract obstructions. The intracavitary parts of the rhabdomyomas were successfully resected when the patient was 5 weeks old. Echocardiography showed regression in size of the remaining intramural tumors. Since there is only a low growth potential in cardiac rhabdomyomas, surgical management is indicated only in patients with hemodynamic obstruction or significant arrhythmias. Surgery should be primarily directed to relieve hemodynamic obstruction. If complete resection is not possible without damage to vital structures, resection should be restricted to the intracavitary parts of the tumor.

Echocardiography↗
PubMed1987

Myoglobin, desmin and vimentin in ultrastructurally proven rhabdomyomas and rhabdomyosarcomas. An immunohistochemical study utilizing a series of monoclonal and polyclonal antibodies.

The present study is an immunohistochemical analysis utilizing a series of mono- and polyclonal antibodies to myoglobin, desmin and vimentin in smooth and striated control muscle tissues, 7 alveolar and 7 embryonal rhabdomyosarcomas and 1 adult and 1 fetal rhabdomyoma with ultrastructurally proven rhabdomyoblastic differentiation in all the tumors. Formaldehyde-fixed and paraffin-embedded tissue was used for the immunohistochemical analysis of all the tumors, while ethanol fixation was also used for the analysis of the control tissues. The staining for myoglobin with the poly- and monoclonal antibody used was positive in both formaldehyde- and ethanol-fixed skeletal and cardiac control muscle. Trypsin treatment abolished the positive staining when the monoclonal antibody was used. Both the striated and smooth control muscle tissues were positively stained by the antidesmin antibodies. The influence of the fixative that was used and the trypsin treatment depended on the antibody used and the type and origin of the muscle tissue. All the tumors were positively stained with the polyclonal antimyoglobin and 8/14 rhabdomyosarcomas and the 2 rhabdomyomas were positively stained with the monoclonal antimyoglobin. All the tumors were positively stained with the polyclonal and 3 of the 5 monoclonal antidesmin antibodies used. Well-differentiated tumor cells were usually positively stained for both myoglobin and desmin. There were small, poorly differentiated tumor cells in the rhabdomyosarcomas and the fetal rhabdomyoma which were positively stained for desmin, whereas very few or no such cells were positively stained for myoglobin. A varying number of mostly small, poorly differentiated tumor cells were positively stained for vimentin in 12 of 14 rhabdomyosarcomas and in the fetal rhabdomyoma. The study showed that one of the monoclonal antidesmin antibodies produced the most consistent result with a positive staining in all cases. The monoclonal antimyoglobin antibody, which is a specific marker of rhabdomyoblastic differentiation, is also considered to be of value, although it did not produce positivity in all cases. It remains to be shown whether desmin can help in the diagnosis of poorly or undifferentiated rhabdomyosarcomas without light- or electron-microscopic evidence of rhabdomyoblastic differentiation.

Adolescent↗
PubMed1982

[Rhabdomyoma of the left atrium. A rare case of mitral valve congenital obstruction simulating hypoplastic left heart syndrome. Echocardiographic diagnosis].

The AA. describe the clinical, surgical and pathological features of a 13-days old infant with multiple cardiac rhabdomyomas diagnosed by two-dimensional echocardiography (2DE). The clinical picture simulated hypoplastic left heart syndrome (HLHS). 2DE showed a well developed left heart. The apical four chamber view disclosed a mass of echoes between the left atrium and ventricle, across the mitral valve. Another little mass of echoes was attached to the left surface of the ventricular septum. The other cardiac chambers and the great arteries were normal. These multiple intracardiac masses were interpreted as rhabdomyomas. The infant underwent an operation at the age of 21 days because of mitral valve obstruction and CHF. At surgery, a left atrial rhabdomyoma was found, that severely obstructed the mitral valve orifice. Although after resecting the tumour we felt that the obstruction was adequately relieved, the little patient died of pulmonary oedema one hour after surgery. At autopsy all the previously detected myomas were confirmed and a small additional one was discovered within the crista supraventricularis. Unexpectedly, the chordae tendineae of the mitral valve were extremely short. This anomaly, masked preoperatively by the rhabdomyoma, was probably the cause of the fatal postoperative mitral regurgitation.

Diagnosis, Differential↗
PubMed1997

Rapid enlargement of cardiac rhabdomyoma during corticotropin therapy for infantile spasms.

OBJECTIVE: To investigate the influence of corticotropin therapy on cardiac rhabdomyoma. DESIGN: Analysis of data from echocardiography performed on in-patients. PATIENTS: Six patients with rhabdomyoma who were admitted to the authors' medical centre with either convulsion (five cases) or prematurity (one case) between 1985 and 1995. Five had tuberous sclerosis. INTERVENTION: Size of cardiac tumours of each patient was measured by echocardiography, and volume index was calculated as the ratio of the tumour volume to its initial volume. MAIN RESULT: Increase in size of some of the tumours was found during corticotropin therapy on follow-up echocardiography. Maximum volume indexes of tumours in the case of patients (n = 4) who did not receive corticotropin therapy was 1.2 to 3.7, whereas those of patients (n = 2) who received therapy was 9.1 to 12; one of the latter patients died. CONCLUSION: Corticotropin may contribute to the enlargement of cardiac rhabdomyoma. The size of cardiac rhabdomyomas must be carefully followed when patients are treated with corticotropin.

Adrenocorticotropic Hormone↗
PubMed1997

[Extra-cardiac rhabdomyoma of the fetal type].

Rhabdomyomas are benign tumors of striated muscle. They are distinguished by topographic data: cardiac or genital and by histological criteria: foetal or adult type. The foetal type is the most heterogeneous, with either a majority of immature cells or a mixture of maturing elements. This diversity has led to distinguish immature (or standard) foetal Rhabdomyomas from intermediate foetal Rhabdomyomas. One observation of this last type is reported. The authors highlight the essential characteristics of foetal Rhabdomyomas, however the exact meaning remains unclear: an anomaly in the differentiation of the striated muscle? or a true tumoral process through genetic anomaly?

Fetal Diseases↗
PubMed1994

Surgical treatment for cardiac rhabdomyomas in children.

Cardiac rhabdomyomas are the most common cardiac tumor in children. These tumors may cause obstruction to blood flow, valvular insufficiency, and cardiac arrhythmias. We present two cases of cardiac rhabdomyomas in infants that were managed surgically and review the literature. Without surgical intervention, the prognosis for symptomatic cardiac rhabdomyomas is dismal, with eventual death likely due to heart failure or arrhythmias. As our two cases indicate, surgical treatment may improve this prognosis. Pediatric cardiac rhabdomyomas should be resected when the tumors cause hemodynamic compromise or cardiac arrhythmias.

Heart Neoplasms↗
PubMed2000

Adult rhabdomyoma of the extremity: a case report and review of the literature.

The adult rhabdomyoma is a rare, benign skeletal muscle neoplasm that usually occurs in the head and neck. A case report of an adult rhabdomyoma arising in the thigh is presented with a review of the literature. This is the first case of an extremity adult rhabdomyoma to be reported. It is also the largest at 13 centimeters. Distinction from a highly differentiated rhabdomyosarcoma is important. Recent chromosomal studies suggest that the adult rhabdomyoma is a true neoplasm. Total resection is curative but the lesion may recur if incompletely excised.

Aged↗
PubMed1975

Parapharyngeal rhabdomyoma.

Rhabdomyoma is a benign tumor of striated muscle origin that is very rare in young children. Review of the world literature revealed 20 cases of extracardiac adult-type rhabdomyoma in the head and neck area. All reported cases have been encapsulated and easily excised. A 22 1/2-month-old girl with a parapharyngeal rhabdomyoma is unique in that her tumor had atypical histology and lacked a capsule; it could not be "shelled-out".

Age Factors↗
PubMed1976

Rhabdomyoma of the larynx. Report of a case.

Review of the English literature shows six reported cases of "rhabdomyoma of the larynx." As far as we have been able to determine, only three cases of these have been undisputed rhabdomyoma. The case reported herein represents the first rhabdomyoma of the larynx that was removed under microsurgical conditions, and adds a fourth case to the literature.

Humans↗
PubMed1993

Rhabdomyoma and ventricular preexcitation syndrome. A report of two cases and review of literature.

OBJECTIVE: A description of two infants with ventricular preexcitation syndrome and supraventricular tachycardia associated with rhabdomyoma and review of the literature. DESIGN: A review of two patients who were seen between 1981 and 1986 in the cardiology department. SETTING: Pediatric Heart Institute at St Christopher's Hospital for Children, Philadelphia, Pa. PARTICIPANTS: Two newborn female infants with ventricular preexcitation syndrome, supraventricular tachycardia, rhabdomyomatous tumor of the heart, and tuberous sclerosis are described. RESULTS: The first patient had unsuccessful partial resection of the rhabdomyomatous tumors obstructing the tricuspid valve and right ventricular cavity and died immediately after surgery. By histologic examination, no direct accessory connection was noted between the myocardial fibers of atria and ventricles through annulus fibrosus. By gross examination, the tumor extended from the right atrium through the tricuspid valve to the right ventricular cavity, suggestive of macroscopic accessory connection. The second patient presented with unsustained ventricular tachycardia and obstructive subaortic rhabdomyoma, requiring emergency surgery. One week later, reentrant supraventricular tachycardia developed and she required digoxin therapy for 15 months. CONCLUSIONS: Infants with rhabdomyomatous tumor of the heart and ventricular preexcitation syndrome may have microscopic or macroscopic accessory connections. Cardiac tumors like rhabdomyoma and oncocytic tumors should be suspected in infants with ventricular preexcitation syndrome or supraventricular tachycardia.

Female↗
PubMed1999

Tuberous sclerosis with intracardiac rhabdomyoma in a fetus with trisomy 21: case report and review of literature.

A large cardiac rhabdomyoma protruding into the left ventricle was diagnosed in a fetus at 21+2 weeks of gestation by grey-scale echocardiography. Obstruction to left ventricular outflow was ruled out by colour and spectral Doppler echocardiography. No other abnormalities were noted and karyotyping by cordocentesis revealed trisomy 21 (47,XY,+21). Post-mortem examination after termination of pregnancy confirmed the prenatal diagnosis of cardiac rhabdomyoma and in addition revealed fetal tuberous sclerosis. Demonstration of cardiac rhabdomyoma by prenatal ultrasound should raise suspicion of the presence of fetal tuberous sclerosis. Despite the incidental association with aneuploidy, fetal karyotyping is suggested for optimal counselling of parents.

Adult↗
PubMed1976

Benign vaginal rhabdomyoma: a light and electron microscopic study.

A benign vaginal rhabdomyoma from a 49-year-old woman was examined by light and electron microscopy. While many of the ultrastructural features conformed to those noted in previously described rhabdomyomas, certain features are described for the first time. These include attachment plaques between cells enclosed within a common basement membrane, cytoplasmic bodies, and peripheral couplings. Finally, the literature is reviewed and the reported cases of rhabdomyoma are divided into adult and fetal types based upon light microscopic criteria.

Cell Nucleus↗