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PubMedDate not supplied

Cerebral embolism resulting from an intracardiac tumor in tuberous sclerosis.

Tuberous sclerosis was diagnosed in an infant girl during the first 3 days of life on the basis of electrocardiographic and cranial computed tomographic abnormalities. At 19 months of age, neurologic deterioration occurred and repeat computed tomography documented several new cortical lesions while the subaortic mass was markedly reduced in size. The sequence of events suggested cerebral embolization by the cardiac tumor.

Echocardiography↗
PubMed2004

Median sternotomy as an exit procedure in a child with massive pericardial tumor.

Fetuses can survive with complete airway obstruction caused by placental gas exchange until birth when full ventilatory function is required. The authors present a case in which prenatal scans suggested that adequate ventilation would not be achievable because of the presence of an intrathoracic tumor. An EXIT procedure (exutero intrapartum treatment) was therefore performed, which permitted sufficient lung expansion for adequate ventilation.

Adult↗
PubMed2005

Malignant stromal tumor, so called "gastrointestinal stromal tumor", with rhabdomyomatous differentiation occurring in the gallbladder.

Gastrointestinal stromal tumors (GISTs) constitute the largest category of primary nonepithelial neoplasms of the gastrointestinal tract. It is extremely rare that this tumor occurs in the bile tract, and only a few cases have been reported. Immunohistochemically, the tumor cells revealed a phenotype similar to Cajal cells, occasionally with differentiation to smooth muscle cells or neural cells. We present a case of malignant stromal tumor similar to GISTs with rhabdomyomatous differentiation of the gallbladder in a 68-year-old woman. The resected tumor was predominantly composed of spindle cells with rhabdomyomatous differentiation. Immunohistochemical study revealed diffuse staining of tumor cells using vimentin despite negative staining for desmin or S-100. This indicated a mesenchymal origin of the cells without smooth muscle or neuronal differentiation. Myoglobin-positive cells, in which phosphotungstic acid hematoxylin staining revealed cross striations of the cytoplasm, suggested rhabdomyomatous differentiation. Diffuse positivity for KIT in the cells suggested that the pathogenesis of this tumor may resemble that of GIST. The tumor may have derived from a mesenchymal stem cell that had undergone partial rhabdomyomatous differentiation.

Aged↗
PubMed2006

The diverse clinical manifestations of tuberous sclerosis complex: a review.

Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem neurocutaneous disorder. TSC results in hamartomatous lesions primarily involving the skin, central nervous system, kidneys, eyes, heart, and lungs. The clinical findings and severity of TSC are highly variable. Recent advances in our understanding of the complexities of the TSC1 and TSC2 genes are making genotype-phenotype correlations possible. While managing seizures, cognitive dysfunction, and behavioral abnormalities are the primary responsibility of the neurologist, familiarity with all aspects of this disease helps provide better comprehensive care for affected individuals.

Central Nervous System↗
PubMed2006

Transbasal approach to skull base tumors: evaluation and proposal of classification.

A clinical study of the TBA was performed in 22 patients harboring tumors of the skull base. The follow-up ranged from 3 to 89 months (average, 30.5 months). The main complications were intracerebral hematoma, ptosis, and infection. One patient died (4.5%) because of an extradural hematoma. Eight patients died during the follow-up because of tumor complications. Among the survivals, the median of the Karnofsky index was 96.4. Based on this study, we propose a classification for the TBA, according to its extension.

Angiofibroma↗
PubMed1983

Rhabdomyomatous dysplasia of lung and congenital diaphragmatic hernia.

A previously unrecognized association of pulmonary rhabdomyomatous dysplasia and congenital posterolateral diaphragmatic hernia is reported. The authors subscribe to the concept of ectopia and postulate an anomalous migration of cervical "diaphragmatic" rhabdomyoblasts as the most probable origin of the pulmonary muscle fibers.

Female↗
PubMed1983

Fetal rhabdomyomatous nephroblastoma.

A case of fetal rhabdomyomatous nephroblastoma is reported. The neoplasm distended the renal pelvis, resembling a sarcoma botryoides. The patient died postoperatively of pneumonia and an autopsy showed no residual neoplasm.

Child, Preschool↗
PubMed1984

Malignant "triton" tumors: a clinicopathologic and immunohistochemical study of nine cases.

Nine cases of malignant "triton" tumors, based on the coexistence of rhabdomyoblasts and Schwann cell elements, were analyzed clinicopathologically and immunocytochemically. All tumors were stained for myoglobin and S-100 protein by the immunoperoxidase technique. Six of the nine patients were in the third or fourth decade of life. Six cases were associated with von Recklinghausen's disease, and the tumors in two cases grew along nerve trunks. The malignant tumors showed a predilection for the thigh and buttock. Six of the seven deaths occurred within two years of the initial treatments. Strongly positive staining for S-100 protein was observed in three tumors, with transitional zones between the sarcomas and peripheral neurofibroma-like areas, as well as in two tumors composed predominantly of rhabdomyoblastic elements. In four other cases the tumors were only weakly positive for protein S-100. Intracytoplasmic myoglobin was present in all cases. Tumors composed predominantly of rhabdomyosarcomatous elements occurred in four patients, including two children with von Recklinghausen's disease. These results, considered with other findings, suggest that malignant "triton" tumors may not be as rare as previously believed.

Abdominal Muscles↗
PubMed1986

Tumors of skeletal muscle.

The most common tumors arising in muscle are soft tissue sarcomas, fibromatoses, and hemangiomas. Rhabdomyosarcoma is primarily a tumor of childhood and adolescence and arises most commonly in extramuscular sites. Most intramuscular rhabdomyosarcomas are alveolar. Increased diagnostic accuracy and the recognition of malignant fibrous histiocytoma have changed understanding of adult, intramuscular, pleomorphic rhabdomyosarcoma. Immunohistochemistry is playing an increasingly important role in the diagnosis of rhabdomyosarcoma, and the correlation between the histologic features and clinical behavior of rhabdomyosarcoma is under investigation. Because of their diversity and overlapping histologic features, muscle tumors are a challenge for the pathologist and require intensive study by current techniques.

Adolescent↗