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)

A Head and Neck Sarcoma Registry was established by the Society of Head and Neck Surgeons to review treatment results of a rare tumor by surgeons with special interest in this anatomic site. Two hundred fourteen patients were analyzed. There were 194 adult tumors and 20 pediatric tumors. The major sites included parotid and neck, 20%; face and forehead, 18%; maxilla and palate, 13%; scalp, 12%; mandible, 11%; paranasal sinuses, 7%; larynx, 2%; and oral cavity, 5%. Eighty-four percent were resectable. The disease-free survival was 56%; overall survival was 70% at 5 years. Major determinants of survival were adequacy of resection (margins free of tumor) and tumor type. Survival differed according to tumor cell type (tumor grade was not available). Patients with chondrosarcoma and dermatofibrosarcoma had survival approaching 100%. Patients with malignant fibrous histiocytoma (MFH) and fibrosarcoma (FSA) had intermediate survival of 60% to 70%. The worst survival, less than 50% at 5 years, occurred in patients with osteosarcoma, angiosarcoma, and rhabdomyosarcoma in decreasing order. This suggests a rationale for identifying high-risk patients for prospective adjuvant protocols. This study emphasizes the value of recording uncommon tumors to provide relevant information for future study and possibly therapy.
 ...
PMID:Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. 162 88

The incidence of primary and metastatic cutaneous malignant solid tumors was investigated in a pediatric dermatology department. Among 25,000 first time patients seen between 1971 and 1985, 19 had cutaneous malignant solid tumors with an annual incidence of 0.7 for every 1,000 pediatric dermatology patients. Nine cases had primary cutaneous tumors and 10 cases metastatic tumors. The majority of patients were infants (zero to two years). The tumors found were rhabdomyosarcoma, six cases; basal-cell carcinoma, four cases; neuroblastoma, three cases; malignant melanoma, two cases, squamous-cell carcinoma, dermatofibrosarcoma, atypical fibroxanthoma and myxopapillary ependymoma, one case of each. Predisposing factors for the developing of malignancy were present in 42% of patients.
 ...
PMID:[Incidence of malignant, primary and metastatic solid skin tumors at a pediatric dermatology service]. 266 91

The distribution of the neural cell adhesion molecule (N-CAM; CD56) was studied immunohistochemically in acetone-fixed frozen sections of 83 soft tissue tumors and selected normal mesenchymal tissue using Leu-19 monoclonal antibody and avidin-biotin peroxidase immunostaining. Positive N-CAM immunostaining was found in gastrointestinal and uterine cells but not in vascular smooth muscle cells. Normal skeletal muscle was negative, but atrophic muscle fibers within tumors were positive. Strong and consistent immunoreactivity was seen in nerves, pheochromocytoma, malignant schwannoma, and spindle cells, but not in epithelial-like cells of synovial sarcoma, hemangiopericytoma, benign leiomyoma, and rhabdomyosarcoma. Variable staining was seen in benign schwannoma and malignant fibrous histiocytoma. Limited, usually focal N-CAM immunoreactivity was seen in desmoid tumor, dermatofibrosarcoma, and leiomyosarcoma. Although the N-CAM is consistently seen in neural tumors, it is not cell lineage specific. Changes on malignant transformation (comparing corresponding benign and malignant lesions) do not show any consistent pattern. Rather, there is neoexpression of the N-CAM in rhabdomyosarcoma, whereas there is loss of the N-CAM in leiomyosarcoma. Immunohistochemistry of the N-CAM can be a useful adjunct for characterization of soft tissue tumors, and its possible correlation with tumor behavior has to be examined in further studies.
 ...
PMID:Neural cell adhesion molecule distribution in soft tissue tumors. 767 94

Adult head and neck soft tissue sarcomas (AHNSTS) are rare, and data concerning treatment results are spare. To assess clinico-pathological characteristics, management prognostic factors, and survival of AHNSTS, we reviewed our experience of 28 recent successive new cases. Data were collected from a retrospective database (1997-2002). Aggressive fibromatosis, dermatofibrosarcoma, Kaposi sarcoma, chondrosarcoma and osteogenic sarcoma were excluded. Univariate analysis for prognostic factors was performed with chi2 test with Yates correction. The median age was 45.7 years (range: 18-86). The male/female ratio was 15/13. The most common subtypes was rhabdomyosarcoma (seven cases). Twenty-two patients presented with previous inadequate resection performed elsewhere before admission. The most common location was neck muscles (11 cases). Nineteen patients had surgery (complete resection in 13 cases). Associated treatments were neoadjuvant chemotherapy, adjuvant chemotherapy and postoperative radiotherapy in respectively, 4, 3 and 10 cases. The 2-year overall survival was 56%. Rhabdomyosarcomas (p = 0.005) and inadequate resection (p = 0.04) were associated with poor outcome. Large resection of AHNSTS in a multimodality approach may afford the best chance of disease control.
 ...
PMID:Head and neck soft tissue sarcomas of adult: prognostic value of surgery in multimodal therapeutic approach. 1538 Jan 66