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[The echocardiographic findings of cardiac rhabdomyomas in a female patient with tuberous sclerosis and Down's syndrome].

This report described the case of a patient with cardiac rhabdomyomas and tuberous sclerosis (TS), associated with Down's syndrome. Diagnosis of TS was made subsequently to sebaceus adenomas, peri-ungual fibromas and ash leaf macules, mental retardation, epilepsy, renal and cerebral calcification findings. Physical examination, ECG and Holter ECG monitoring revealed normal cardiac findings. Two dimensional echocardiography disclosed the presence of two areas of increased acoustic density; one of which was at the level of postero-medial papillar muscle and the second appeared to be adherent to ventricular septum. Left ventricular size, function and intracavitary blood flow were normal.

Adult↗

Submandibular rhabdomyoma.

A case of recurrent adult rhabdomyoma of the submandibular gland is presented. This is a rare tumor of the skeletal muscle. Approximately 80 cases have been reported. The salient pathologic features are reviewed and a discussion and review of the literature is presented.

Aged↗

[Giant intracardiac Rhabdomyoma in neonatal stage. Report of one case].

Primary heart tumors are not very common, frequent ones during childhood are the rhabdomyomas. This diagnostic can be made since fetal life. Evolution variates, in some cases there are no symptoms and the diagnostic represents an echocardiographic finding, other cases are detected associated with tuberous sclerosis and a small group express by arrhythmias or low output. Because of the strange of this presentation this case is presented.

Cardiac Surgical Procedures↗

[Rhabdomyoma of the mouth floor--a case report].

We report a rare case of an adult rhabdomyoma of the floor of the mouth in a 52-year-old woman. To date only 25 cases of such tumors have been described in the oral cavity. In spite of its large size (approximately 10 x 8 x 6 cm), the tumor could be completely removed. Up to now, 20 months after the removal of tumor, no recurrences, have occurred.

Biomarkers, Tumor↗

[Cardiac rhabdomyoma in tuberous sclerosis. Presentation of a case].

We report of a case of cardiac tumor in a three months old infant, examined with echocardiography because a murmur was noted on routine examination. Further investigations brought out the diagnosis of tuberous sclerosis. Spontaneous regression of the tumor mass occurred in the following months. No signs of cardiac dysfunction of CNS involvement appeared. The peculiarity of rhabdomyoma as an early manifestation of tuberous sclerosis is emphasized.

Echocardiography↗

Giant aortic aneurysm and rhabdomyomas in infant with tuberous sclerosis (case report).

We present the case of a giant aneurysm and dissection of the abdominal aorta in an eight month old infant. Imaging studies especially a helicoid computerized axial tomography with 3D reconstruction, showed a lesion which extended from the diaphragm to the iliac arteries. The clinical findings and the above mentioned studies revealed non-obstructive heart rhabdomyomas, tubers in the brain and the abdominal aortic aneurysm, all of which were consistent with the diagnosis of tuberous sclerosis.

Aortic Aneurysm, Abdominal↗

Tuberous sclerosis with cardiac rhabdomyoma manifested by fetal bradycardia: report of a case.

This is a case of neonatal tuberous sclerosis associated with cardiac rhabdomyoma and manifested by fetal cardiac arrhythmia-bradycardia. The prenatal echocardiography showed multiple cardiac tumors which occupied the left and right ventricles. The largest one measured 5.2 cm in diameter. It was found to be 4.5 x 4.1 x 3.5 cm in size at autopsy. A postnatal cranial echogram showed multiple subependymal nodules. The patient expired after 7 hours of life. The autopsy findings confirmed the diagnosis. The mother of the patient had adenoma seb'aceum on the face and an ungual fibroma on the left little finger. Cranial computerized tomography revealed a small calcified tubercle.

Adult↗

[A case of left ventricular rhabdomyoma in a child].

A three-year-old girl was admitted to our hospital for surgical treatment of cardiac tumor. At three days after birth, ECG showed bradycardia about 70/min. UCG revealed intracardiac tumor in left ventricle. During operation, the tumor was detected by intraoperative UCG. Left ventricle was opened and tumor was removed from interventricular septum. Histological examination revealed rhabdomyoma. Postoperative course was uneventful and she is doing well.

Child, Preschool↗

[Cardiac rhabdomyoma and tuberous sclerosis. Survival after the surgical resection of the cardiac tumor].

A 3-month-old boy with tuberous sclerosis was diagnosed of biventricular heart tumor by means of 2D-echocardiogram. Three masses of tumor were excised from the right ventricle with the aid of cardiopulmonary bypass. Pathologic examination disclosed a cardiac rhabdomyoma. The boy is symptoms-free after 5 years of the operation. An echocardiogram taken at this time showed no-evidence of heart tumor. Pathology, diagnostic methods and surgical indications are commented. A review of selected surgically resected cases is also presented.

Biopsy↗

[Tuberous sclerosis associated with left ventricle rhabdomyoma].

Female infant, presenting with flexion spasms, skin hypochromatic spots and systolic ejection murmur. A clinical diagnosis of tuberous sclerosis, supported by computerized brain tomography and neurosonographic data, was made. Echocardiographic and cineangiocardiographic studies disclosed an associated tumor in left ventricle outlet. After resection of the rhabdomyoma, the patient is asymptomatic with neural manifestations controlled under valproic acid.

Echocardiography↗

Aspiration cytology of adult rhabdomyoma.

The distinct cytologic picture of a fine needle aspirate from a case of adult rhabdomyoma is described. The presence of cytoplasmic cross striations was a diagnostic feature that could be demonstrated in some tumor cells with all routine stains used (Papanicolaou, May-Grünwald-Giemsa and hematoxylin and eosin).

Biopsy, Needle↗

[Prenatal diagnosis of heart rhabdomyoma].

A fetal heart rhabdomyoma diagnosed at 31 weeks of gestational age, is presented. The tumor, located in the right ventricle, is asymptomatic. The diagnosis is confirmed postnatal.

Adult↗

Multifocal rhabdomyoma of the neck. Report of a case studied by fine-needle aspiration, light and electron microscopy, histochemistry, and immunohistochemistry.

A 32-year-old Caucasian man presented with a tumor in the right side of the neck. Preoperative fine-needle aspiration showed large multinucleated cells with abundant granular cytoplasm that were consonant with the features of a granular cell tumor. At surgery, two separate tumors were found. Histologically, the tumor proved to be an adult rhabdomyoma, the 11th such multifocal case reported. The striated muscle origin of this benign tumor was confirmed by immunohistochemical and ultrastructural studies. The tumor cells were desmin-, myoglobin-, and actin-positive. They showed variable numbers of thick and thin filaments, as well as hypertrophic Z-band material. Histochemical studies showed the presence of basophilic muscle cells, vesicular nuclei, ragged red fibers, and diffuse acid phosphatase positivity. These features, together with the absence of actual muscle cell proliferation and the assumption that the mass of the tumor could be explained by the enormous swelling of the muscle cells, lead us to conclude that an adult rhabodomyoma is merely the result of a process of disorderly degeneration and regeneration rather than a real neoplasm or a hamartomatous lesion.

Acid Phosphatase↗

[Rhabdomyoma in a newborn infant. Echocardiographic diagnosis].

With the 2D echocardiography, is easier the diagnosis of intracardiac tumors nowadays. We present a case of a newborn girl with a rhabdomyoma intra-myocardiac and intracavitary. We discuss the clinical manifestations, surgical indications and the association with tuberous sclerosis.

Angiocardiography↗

[Rhabdomyoma of the vagina].

Three cases of benign vaginal rhabdomyoma were examined by light and electron microscopy. Frequency, localization, genesis, nomenclature, and differential diagnosis of the rare tumour were discussed.

Adult↗

[Rhabdomyoma of the interventricular septum in a one-day-old newborn infant. Echocardiographic diagnosis].

A case of interventricular septal rhabdomyoma in a one day-old newborn is presented. The newborn was moderately cyanosed. A 3/6 systolic murmur in basal area was present. An electrocardiogram showed the features of Wolff-Parkinson-White syndrome. A chest x-ray showed distinct cardiomegaly. M-Mode (MME) and Two-dimensional echocardiography (2DE) identified an interventricular septal mass within left ventricle and suggested the presence of left ventricular outflow obstruction. We emphasize the usefulness of MME and 2DE in accurately diagnosing intramural ventricular tumors in newborns.

Echocardiography↗

Contribution by tomodensitometry to the understanding and diagnosis of benign non-epithelial endolaryngeal tumours. In connection with a case of chondroma, one of lipoma and one of rhabdomyoma.

The authors report on 3 cases of submucosal, non-epithelial tumour of the larynx: one chondroma, one lipoma and rhabdomyoma. A review of the literature is presented. These three cases have benefited by radiological imagery proper to tomodensitometry. This technique is certainly the preferential radiological examination, since it enables an appraisal to be made of the interrelationship of the tumour and its surrounding structures. It gives a probability diagnosis through comparative measurement of the tumorous density.

Adolescent↗

Adult-type of rhabdomyoma of the cheek. A case report with emphasis on the histological and immunohistochemical features.

A case of adult-type rhabdomyoma of the cheek is reported. The tumor was composed of cells presenting striated muscle differentiation, which was confirmed immunohistochemically by the positive reaction with alpha 1-sarcomeric actin demonstrating the cytoplasmic cross striations. A careful evaluation of the light microscopic features of this rare tumor, as well as the histochemical and immunohistochemical characteristics of the tumor cells would greatly help the differential diagnosis.

Humans↗