Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignancy occurring during the neonatal period (defined as the first 28 days of life) is over 3 times the incidence of other pediatric age groups. Of all neoplasia occurring in infants, benign and malignant, 25% are soft tissue tumors. Differentiating the benign lesions from the 15% that are malignant can be difficult. This article discusses the epidemiology, differential diagnosis, evaluation, and treatment of infants with soft tissue sarcomas. Fibrosarcoma and rhabdomyosarcoma are also discussed at length. The authors review other rare tumors as well. The impact on diagnosis of molecular techniques is included when appropriate. A multidisciplinary team approach for treatment of these infants is recommended.
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PMID:Soft tissue sarcomas of infancy. 1047 43

We report a case of congenital primary cutaneous rhabdomyosarcoma, solid alveolar type, presenting as a solitary skin lesion on the right upper lip of a 2-week-old infant boy. Rhabdomyosarcoma originates from the embryonic mesenchyme precursor of striated muscle. Histologically it belongs to the group of "small round cell tumors." Its myogenic origin is ascertained by immunohistochemical studies positive for myogenin, muscle-specific actin, desmin, and myoglobin. Malignancy in the neonatal period is uncommon and the clinical management presents considerable challenges. Congenital alveolar rhabdomyosarcoma is a highly malignant tumor with no record of long-term survivors. Treatment options include chemotherapy, excision, and radiotherapy. This infant's tumor was responsive to chemotherapy and surgery and he was free of disease at the 6-month follow-up.
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PMID:Congenital primary cutaneous rhabdomyosarcoma in a neonate. 1286 57

To identify predictive findings in children with nontraumatic acquired cavernous sinus lesions, a retrospective study of the clinical course of 4 of our own patients and 17 more children found in an extensive literature search was performed. Mean age was 8.7 years. Malignancy was found in 11 of 21 children (6 female, 15 male). Of these 21 children, 9 were cured, 8 are either in remission or their course is unknown, and 4 died. Eight of the nine cured patients were diagnosed as having had Tolosa-Hunt syndrome. Of the four deceased children, three had a very short course (1 to 4 months) and were diagnosed with malignant lymphoma (n = 2) and rhabdomyosarcoma (n = 1). One patient died from a brain tumor other than the initially diagnosed T-cell lymphoma in the cavernous sinus after a follow-up of 8 years. MRI should be the preferred imaging technique-even if it is not conclusive in many cases-and every possible diagnostic effort should be made before using corticosteroids. No clinical or radiological signs other than rapid deterioration seem to be predictable of a malignant cavernous sinus lesion with poor outcome. Thus, close follow-up is recommended in children with signs and symptoms indicative of an acquired lesion in the cavernous sinus.
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PMID:Prediction of the clinical outcome of cavernous sinus lesions in children. 2356 17