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 811 records · Page 45Linked to original sources
PubMed1978

Lipomatous infiltrates of the heart.

Fatty masses of the heart usually arise in the interatrial septum and are uncommon. The autopsy files from LAC-USC Medical Center contain 15 such cases from 1952 through 1972. Although these masses are yellow and attain a large size, most are considered to represent lipomatous infiltrations rather than true lipomas since they usually lack encapsulation and do contain cardiac muscle in varying amounts. The lesion is most often associated with obesity. Surprisingly, even the large masses produce few clinical symptoms and none was suspected clinically, each being an incidental finding at necropsy. However, these masses do infiltrate the conduction system and can cause arrhythmias and sudden death. Echocardiography or other noninvasive techniques may bring attention to a lipomatous mass that may or may not be asymptomatic. This can result in a clinical dilemma in differential diagnosis and treatment since myxomas, rhabdomyomas, and sarcomas also arise in the interatrial septum.

Adult↗
PubMedDate not supplied

Pseudotumor of the heart: subepicardial hematoma of the right atrium.

A 28-year-old man with a huge subepicardial hematoma of the right atrium, which is so rare that a review of the literature has failed to disclose similar case, is reported. Suspecting of rhabdomyoma, we excised free wall of the right atrium and a part of lateral wall of the superior vena cava. The excised part was reconstructed with expanded polytetrafluoroethylene (Gore tex). This synthetic material has a few merit and application of this one to the reconstruction in the venous system seems more promising. He was discharged with a regular sinus rhythm, normal heart size three months after the operation. The histologic examination of the excised material revealed amorphous blood clots and chronic granulation tissue containing hemosiderin-cells. The cause of the hematoma was not clarified even by microscopic examination.

Adult↗
PubMed1982

[Granular cell tumors of the larynx].

Based on four observations of our own and a survey of the 151 cases described to date, the clinical and morphologic results of granular cell tumours of the larynx are presented. These tumours, which were considered as being of myogenic origin ("myoblastic myoma", immature rhabdomyoma", "granular cell myoblastoma" etc.) are probably of neurogenic origin. They develop primarily inthe posterior section of the vocal cords in middle aged males and females. The granular cell tumours remain benign and can usually be extirpated endoscopically without recurrence.

Adolescent↗
PubMed1984

Immunoperoxidase demonstration of a new muscle protein (Z-protein) in myogenic tumors as a diagnostic aid.

Z-protein, a new muscle protein with a molecular weight of 55,000 daltons recently discovered in Japan, is an excellent marker for muscle differentiation in paraffin sections. Positive staining by a peroxidase-antiperoxidase technique for detection of intracellular Z-protein was observed in 2 of 2 cardiac rhabdomyomas, 5 of 5 leiomyomas, 9 of 10 leiomyosarcomas, and in 15 of 18 rhabdomyosarcomas including 5 of 5 of the pleomorphic type, 7 of 9 of the embryonal type, and 3 of 4 of the alveolar type. Other types of tumors (77 cases) that sometimes mimic myogenic tumors on histologic examination gave negative results. The immunoperoxidase method for detection of intracellular Z-protein is of use in distinguishing myogenic tumors from other types of tumors.

Adolescent↗
PubMed1984

[Tuberous sclerosis. Clinical and computer tomography findings in infancy and childhood].

Tuberous sclerosis was diagnosed in 21 patients (13 female, 8 male) at an age of 3 months to 17 years, 8 of them being younger than 1 year at the time of diagnosis. 14 cases occurred sporadic, 7 patients had a positive familial history of the disease. The diagnosis was based on typical cutaneous lesions (white spots in 19, adenoma sebaceum in 12), cerebral seizures (20 patients) and intracranial calcifications which were found in all 17 patients who were investigated by cranial computerized tomography, especially in all 5 patients who were investigated in the first year of life. In addition two patients showed tumors arising from the region of the frontal horns. Histological examination which was performed in one of them showed the typical picture of a subependymal giant cell astrocytoma. Seizures occurred within the first year of life in 15 of 20 patients. The most common seizure type were infantile spasms (11 patients), grand mal (10 patients) and partial seizures (7 patients). In two children Todd's palsies were observed. Mental retardation of variable degree was observed in 11 patients. Cystic renal lesions were found in one patient with severe haematuria and they were suspected in 3 others on ultrasonographic examination. Retinal phakoma was found in only one patient, cardial rhabdomyoma was suspected in another one who suffered from congenital multifocal atrial tachycardia. The diagnosis in the first year of life was based upon the typical trias of white spots, seizures and intracranial calcifications on CT examination.

Adolescent↗
PubMed1980

[Tuberous sclerosis: first manifestation as cardiac arrhythmia in a newborn].

A newborn baby is found to have multifocal atrial tachycardia. Myoclonic seizures and depigmented nevi appear at the age of five months. The clinical diagnosis of tuberous sclerosis is confirmed by the result of cranial computerized tomography, which shows typical lesions. Though cardiac angiography fails to show an intracavitary tumor, multiple intramural rhabdomyomas of the myocardium are probably the cause of the persistent arrhythmia.

Arrhythmias, Cardiac↗
PubMed1993

[Supraventricular extrasystolic arrhythmia as the first sign of tuberous sclerosis: description of a case].

Tuberous sclerosis (TS) is a rare congenital disease characterized by proteiform symptoms that effect the skin and the central nervous system. Occasionally morphofunctional anomalies of other organs such as the kidneys, lungs and heart are present. The clinical picture of the illness varies among patients, and diagnosis is based primarily on cutaneous or nervous system manifestations. During the last ten years, however, some cases of prenatal diagnosis have been reported. The literature reports very few cases of tuberous sclerosis with arrhythmias as the initial symptom. The case described refers to a newborn who presented with supraventricular arrhythmias, refractory to the common antiarrhythmic drugs; a subsequent M-mode and two-dimensional echocardiogram showed a small roundish mass in connection with the chordae tendinae of the mitral valve (rhabdomyoma). We initially diagnosed TS based on the appearance of achromic smooth spots of the skin. The onset of convulsions and a CAT scan that identified numerous calcified endocerebral nodules confirmed the diagnosis of tuberous sclerosis.

Cardiac Complexes, Premature↗
PubMed1994

Tuberous sclerosis associated with neurofibromatosis: report of a case.

Neurofibromatosis and tuberous sclerosis are phakomatous syndrome diseases. They are both inherited as autosomal dominant diseases. Neurofibromatosis type 1 and tuberous sclerosis very seldom occur together. We report a 16-year-old male who had characteristics of these two diseases. This patient had all the criteria for a definitive diagnosis of tuberous sclerosis such as: classical shagreen patches, periungual fibroma, retinal hamartomata, facial angiofibroma, renal angiomyolipomata, and subependymal glial nodules on computed tomography. He also had the three presumptive diagnostic criteria: cardiac rhabdomyoma, seizure history and first degree relatives with tuberous sclerosis. The patient had more than six café-au-lait spots, the greatest diameter of which exceeded 15 mm in diameter. Multiple neurofibromas, one plexiform neurofibroma, axillary freckling and optic gliomas were also found on his body. These criteria are sufficient for a diagnosis of neurofibromatosis type 1 to be made. The skin biopsies were also consistent with the disease. Apart from the patient's mother and younger sister, who had tuberous sclerosis, there was no other person with neurofibromatosis type 1 in his immediate family. We believe that tuberous sclerosis was inherited from his mother and that neurofibromatosis type 1 may have resulted from mutation.

Adolescent↗
PubMed1993

Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies.

Cardiac involvement by primary and secondary tumors is one of the least investigated subjects in oncology. Seven cases of primary and 154 cases of secondary cardiac tumors from autopsies performed over a 20-year period (1972 through 1991) at Queen Mary Hospital, Hong Kong, were reviewed. During this period, 12,485 autopsies were performed, and the autopsy incidence for primary and secondary heart tumors is thus 0.056% and 1.23%, respectively. Only seven primary cardiac tumors were found, including two myxomas, two rhabdomyomas, two hemangiomas, and one lipoma. For secondary tumors involving the heart (including both metastasis and local extension), important primary tumors in male subjects were carcinoma of the lung (31.7%), esophageal carcinoma (28.7%), lymphoma (11.9%), carcinoma of the liver (6.9%), leukemia (4.0%), and gastric carcinoma (4.0%), while in female subjects, carcinoma of the lung (35.9%), lymphoma (17.0%), carcinoma of the breast (7.5%), and pancreatic carcinoma (7.5%) predominated. Overall, the three most common malignant neoplasms encountered were carcinoma of the lung, esophageal carcinoma, and lymphoma. Pericardium, including epicardium, was the most common location of cardiac involvement by secondary tumors, followed by myocardium and endocardium. The present study showed a higher percentage of esophageal carcinoma and carcinoma of the liver (reflecting the higher incidence of these tumors in Hong Kong Chinese), but a lower incidence of carcinoma of the breast when compared with other series. The metastatic lung tumors showed an unusual predominance of adenocarcinoma.

Adolescent↗
PubMed1993

[Botryoid rhabdomyosarcoma of the cervix. Clinico-pathologic study of a case].

The botryoid rhabdomyosarcoma of the cervix is a rare tumour occurring in young woman or during genital activity. It mainly causes vaginal bleeding or appears as a polypoid grape-like mass with a gelatinous cut-surface. The diagnosis is based on the presence of a submucosal cambium layer and a rhabdomyoblastic differentiation, corresponding to an intracytoplasmic double cross-striation. Metaplastic cartilaginous islands are sometimes observed. The immunostaining ensures the muscular origin of the tumour, characterized by the expression of actin and desmin. Apart from mullerian adenosarcomas, the main differential diagnosis is represented by the benign polypoid formations of the cervix: the genital rhabdomyoma and the fibroblastic lesion, called fibro-epithelial polyp with atypical stroma. The treatment actually includes both chemotherapy and surgery which is often limited to a conization.

Adolescent↗
PubMed1997

Subependymal giant cell astrocytoma: a clinicopathologic study with HMB45 and MIB-1 immunohistochemical analysis.

Subependymal giant cell astrocytomas (SEGAs) occur in approximately 6% of patients with tuberous sclerosis (TS) and are often considered to be a forme fruste or partial expression of this disorder. Recently, a number of other TS-associated lesions, including pulmonary and uterine lymphangiomyomatosis, renal angiomyolipoma, and cardiac rhabdomyoma, were shown to stain with the monoclonal antibody HMB45, suggesting a pathogenetic relationship among these lesions. Seven SEGAs in six patients (three men and three women; age range, 2-55 yr; mean, 24 yr) were studied. Cutaneous and other central nervous system features of TS were present in two patients. All of the SEGAs were intraventricular in location and were characterized by large, round, eosinophilic and spindle-shaped cells, with nuclear pseudoinclusions and mild nuclear pleomorphism, arranged in a vaguely perivascular pattern. All of the SEGAs were negative with HMB45 immunostaining. MIB-1 immunostaining was also performed in all of the cases, and MIB-1 indices (the percentage of MIB-1-positive nuclei) ranged from 0.1 to 3.8 (mean, 1.1). Two patients required an additional surgical procedure for residual/recurrent tumor; three patients are alive without evidence of disease 5 to 11 years after diagnosis; and one patient was lost to follow-up. SEGAs are generally slowly growing intraventricular neoplasms, as evidenced by their low MIB-1 labeling. Unlike other TS-associated lesions, SEGAs do not stain with HMB45.

Adolescent↗
PubMed1996

Fine-needle aspiration of the head and neck.

Fine-needle aspiration cytology is a valuable technique in the work-up of nodules and masses arising within the head and neck. Squamous cell carcinoma is present most often, and because of this relative frequency, the primary utility of needle-aspiration cytology is in the confirmation or exclusion of this diagnosis. FNA is particularly helpful in the work-up of cervical masses and nodules because biopsy of cervical adenopathy should be avoided unless all other diagnostic modalities have failed to establish a diagnosis. As such, needle-aspiration cytology represents an accurate, inexpensive, and rapid technique for elucidation of the etiology of cervical adenopathy. The majority of aspirates from cervical lymph nodes will disclose either reactive lymphadenopathy or metastatic squamous cell carcinoma. Occasional nodules will be due to lymphoma. While primary diagnosis of lymphoma by needle-aspiration cytology is generally not considered definitive, it is helpful in clarifying the nature of the process and the direction additional diagnostic tests should take. Similarly, establishing the presence of carotid body tumors, brachial cleft cysts or epidermal inclusion cysts excludes metastatic carcinoma and negates the need for open biopsy as well as allaying concerns on the part of both clinician and patient. Fine-needle aspiration of lesions within the mouth, oral pharynx, nasopharynx, and nasal sinuses has similar diagnostic goals, in that eliminating squamous cell carcinoma is its paramount objective. Fine-needle aspiration cytology can also establish a specific diagnosis for many lesions within this area. This technique can make specific diagnoses of angiofibroma, primary adenocarcinoma of the nasal sinuses, rhabdomyoma, granular cell tumor, and rhabdomyosarcoma. Each of these represents an important clinical entity with a specific therapy. Utilizing electron microscopy and immunohistochemical techniques, along with flow cytometry, can greatly broaden the diagnostic range and specificity of needle-aspiration cytology. Flow cytometry and immunohistochemistry are particularly useful in the establishment of monoclonality in lymphoproliferative processes and, hence, aid in the separation of reactive from lymphomatous lymphadenopathy. Immunohistochemistry can establish the precise nature of lesions as variable as rhabdomyosarcoma, olfactory neuroblastoma, and granular cell tumor. The prudent use of these techniques can be cost-effective and negate the need for more invasive diagnostic procedures. Needle-aspiration cytology represents a cost-effective and rapid technique for the assessment of nodules and masses within the head and neck area. Limitations in accuracy exist. In particular, the separation of reactive atypia in benign squamous epithelium from well-differentiated squamous cell carcinoma may be exceedingly difficult, if not impossible. Nonetheless, the technique has a high degree of accuracy for the diagnosis of both primary and metastatic disease.

Biopsy, Needle↗
PubMed1998

Hamartoma of mature cardiac myocytes: a case report.

A 24-year-old man presented with hypertension, palpitations, and premature atrial and ventricular contractions. A mass was discovered in the distal interventricular septum that was composed of dense collagenous tissue, fat, and disorganized, hypertrophic, mature cardiac myocytes indicative of a cardiac hamartoma. This entity has only rarely been reported and must be distinguished from the much more common rhabdomyoma and from oncocytic cardiomyopathy, which is also referred to as "cardiac hamartoma."

Adult↗
PubMed1998

Cardiac dysrrhthmia in systemic disease.

This is an ECG of a 6-year-old girl. She presented to us for management of epilepsy. Magnetic resonance imaging (MRI) of the brain showed multiple lesions consistent with cortical and subcortical tubers. There were also achromic spots on her skin and echocardiography demonstrated a rhabdomyoma near the right ventricular outflow tract. A diagnosis of tuberous sclerosis, an autosomal-dominantly inherited condition, was made. What is abnormal about the ECG?

Child↗