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

203 cancers of the eyelid were operated on in 193 patients. 65% of the tumours occurred in males and the peak incidence was at 75 years. 60% afflicted the lower eyelids and 21% the medial canthi. Basal cell carcinoma were found in 182 specimens and spinocellular carcinoma in only 8. Malignant melanoma, Meibomian gland carcinoma and rhabdomyosarcoma appeared each in one patient, the latter representing the only fatal case in eyelid cancer. All cancers were excised with a free margin around 5 mm. The standard procedures of reconstruction of the eyelid defect were a fullthickness skin graft in cases where conjunctiva and tarsus could be preserved (97 cases), and a tarsoconjunctival flap in full-thickness defects of the lower eyelid (58 cases). Other methods used were wedge excision and direct approximation in very small lesions involving the lid margin, an infratarsal island flap from the lower eyelid for medium-sized to large defects in the upper lid and a forehead or scalp flap after exenteration of the orbit. 18/203 cancers recurred and 12 of these were primarily regarded as radically treated. Seven of the latter were reoperated after more than 3 years and may in fact be new tumours. No metastases were found. The various reconstructive procedures are discussed in detail
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PMID:Surgical treatment of eyelid cancer with special reference to tarsoconjunctival flaps. A follow-up on 193 patients. 110 77

The production and detailed immunostaining properties of a new rat monoclonal antibody (ICR.2) to epithelial membrane antigen are reported. The antibody was selected for its ability to compete with the polyclonal antiserum (M7), used in the original immunohistological studies, in order that it might serve as a direct replacement in diagnosing epithelial tumours. Most of the staining reactions on normal tissues were identical to those previously reported with M7 but there were some important differences. They included: positivity of renal and adrenal capsular fibroblasts, perineurium, some myoepithelial and smooth muscle cells, occasional osteoblasts and squamous and thyroid follicular epithelium in the normal state. The intercellular canaliculi of sweat glands and secretory canaliculi of gastric oxyntic cells were clearly demonstrated. These staining reactions could be obtained with M7 when a sensitive detection system was used although the results were usually weak and inconsistent. Nearly all adenosquamous and transitional carcinomas were positive. The remaining tumours fell into three major groups: (1) those which were consistently or nearly consistently negative--melanoma, seminoma, rhabdomyosarcoma, alveolar soft part sarcoma, adrenal cortical carcinoma, granulocytic sarcoma, paraganglioma, non-Hodgkin's lymphoma. Hodgkin's disease and embryonal carcinoma: (2) those which were either negative or positive with distinctive patterns of staining--basal cell carcinoma, embryonal tumours: and (3) non-epithelial tumours that were consistently positive--epithelioid sarcoma, synovial sarcoma, osteosarcoma, chordoma and myeloma--or positive in a significant minority of cases--leiomyosarcoma, malignant fibrous histiocytoma, clear cell sarcoma of tendon sheath, various neuroectodermal tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detailed investigation of the diagnostic value in tumour histopathology of ICR.2, a new monoclonal antibody to epithelial membrane antigen. 169 88

We reviewed patient records of 99 consecutive orbital exenterations performed between 1969 and 1988. Patients ranged in age from 2 to 86 years (mean, 55.9 years). Classification of cases on histopathologic criteria showed 32 exenterations were performed for squamous cell carcinoma originating in the paranasal sinus (13), skin (12), conjunctiva (six), and lacrimal sac (one). Orbital exenteration was performed for treatment of other epithelial malignancy in basal cell carcinoma (eight), sebaceous carcinoma (six), adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor (one), and transitional cell carcinoma (one). Exenterations were performed for melanoma of the conjunctiva (ten), nasosinus (three), skin (two), orbit (two), and choroid (one). Exenterations were also performed as treatment for mucormycosis (five), meningioma (three), fibrosarcoma (two), rhabdomyosarcoma (two), hemangiopericytoma (two), orbital cellulitis (one), fibrous histiocytoma (one), schwannoma (one), lymphangioma (one), benign lymphoepithelial lesion (one), and undifferentiated malignancy (one).
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PMID:A 20-year series of orbital exenteration. 195 84

A 4-year-old girl presented with a nasopharyngeal rhabdomyosarcoma and had the characteristic features of the naevoid basal cell carcinoma syndrome. She was treated with a modified standard combination of chemotherapy and radiotherapy. However, radiotherapy had to be interrupted because of severe radiation recall.
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PMID:Case report: nasopharyngeal rhabdomyosarcoma and Gorlin's naevoid basal cell carcinoma syndrome. 685 99

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.
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PMID:Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital. 828 84

Between 1982 and 1994, 21 patients with malignant tumors of the external auditory canal and middle ear were treated at the Department of Otolaryngology, Niigata University. Eleven patients with tumors of the external auditory canal and 10 tumors of the middle ear were registered. There were 9 males and 12 females, and their ages ranged from 10 to 80 years (median: 61). Otalgia, otorrhea or bloody otorrhea were the chief complaints of most patients with external auditory canal of middle ear tumors. Pathological examination revealed squamous cell carcinoma in 16 patients, adenoid cystic carcinoma in 3 patients, and basal cell carcinoma and rhabdomyosarcoma in 1 patient each. External auditory canal tumors were surgically excised, while radical mastoidectomy and subsequent irradiation were performed for the middle ear tumors. The five-year survival rate determined by the Kaplan-Meier method, was 77.8% for patients with external auditory canal tumors and 40% for those with middle ear tumors.
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PMID:[Twenty-one cases of malignant tumor of the external auditory canal or middle ear]. 869 1

Basal cell carcinoma, medulloblastoma, rhabdomyosarcoma and other human tumours are associated with mutations that activate the proto-oncogene Smoothened (SMO) or that inactivate the tumour suppressor Patched (PTCH). Smoothened and Patched mediate the cellular response to the Hedgehog (Hh) secreted protein signal, and oncogenic mutations affecting these proteins cause excess activity of the Hh response pathway. Here we show that the plant-derived teratogen cyclopamine, which inhibits the Hh response, is a potential 'mechanism-based' therapeutic agent for treatment of these tumours. We show that cyclopamine or synthetic derivatives with improved potency block activation of the Hh response pathway and abnormal cell growth associated with both types of oncogenic mutation. Our results also indicate that cyclopamine may act by influencing the balance between active and inactive forms of Smoothened.
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PMID:Effects of oncogenic mutations in Smoothened and Patched can be reversed by cyclopamine. 1098 33

Hedgehog signalling is a key regulator of embryonic development controlling proliferation and/or cell fate determination. With identification of the Hedgehog receptor PTCH1 as a tumour suppressor gene that underlies the human nevoid basal cell carcinoma syndrome (NBCCS), the Hedgehog signalling pathway was firmly linked to cancer. It now appears that constitutive activation of Hedgehog signalling, by inactivating mutations in PTCH1 or activating mutations in the coreceptor SMOH, is required and possibly sufficient for basal cell carcinoma development and also contributes to the formation of a variety of other tumour types, including medulloblastoma and rhabdomyosarcoma. Several lines of evidence, including transgenic mice experiments, suggest that the critical cellular effect is stimulation of proliferation mediated by the transcriptional effector GLI1. Additional components of the signal transduction machinery as well as essential target genes remain to be identified, and involvement of the Hedgehog signalling pathway in other tumour types and/or hereditary cancer predisposition syndromes is to be expected.
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PMID:Hedgehog signalling in cancer. 1113 Jan 78

The zinc finger transcription factor GLI1, which mediates Sonic hedgehog signaling during development, is expressed in several human cancers, including basal cell carcinoma, medulloblastoma, and sarcomas. We identified 147 genes whose levels of expression were significantly altered in RNA obtained from cells demonstrating a transformed phenotype with stable GLI1 expression or stable Ha-ras expression. Comparison of expression profiles from GLI1- and Ha-ras-expressing cells established a set of genes unique to GLI1-induced cell transformation. Thirty genes were altered by stable GLI1 expression, and 124 genes were changed by stable Ha-ras expression. Seven genes had altered expression levels in both GLI1- and Ha-ras-expressing cells. Genes whose expression was altered by GLI1 included cell cycle genes, cell adhesion genes, signal transduction genes, and genes regulating apoptosis. GLI1 consensus DNA-binding sequences were identified in the 5' regions of cyclin D2, IGFBP-6, osteopontin, and plakoglobin, suggesting that these genes represent immediate downstream targets. Gel shift analysis confirmed the ability of the GLI1 protein to bind these sequences. Up-regulation of cyclin D2 and down-regulation of plakoglobin were demonstrated in GLI1-amplified compared with non-amplified human rhabdomyosarcoma cells. Many of the GLI1 targets with known function identified in this study increase cell proliferation, indicating that GLI1-induced cell transformation occurs through multiple downstream pathways.
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PMID:Gene expression profiling leads to identification of GLI1-binding elements in target genes and a role for multiple downstream pathways in GLI1-induced cell transformation. 1171 6

Inherited mutations of Patched (PTCH) in the nevoid basal cell carcinoma syndrome (NBCCS) lead to several developmental defects and contribute to tumor formation in a variety of tissues. PTCH mutations have been also identified in sporadic tumors associated with NBCCS including basal cell carcinoma (BCC) and medulloblastoma. Mice heterozygous for Ptch recapitulate the typical developmental symptoms of NBCCS and develop rhabdomyosarcoma (RMS) and medulloblastoma. PTCH is assumed to act as a tumor suppressor gene although inactivation of both alleles has been demonstrated only in a fraction of tumors. We have investigated the status of Ptch in RMS of heterozygous Ptch neo67/+ mice. Although the wild-type Ptch allele was retained in tumor tissue, the high levels of Ptch mRNA in these tumors result from overexpression of the mutant Ptch transcript. Our results suggest that the wild-type Ptch allele might be selectively silenced in RMS tissue or, alternatively, that haploinsufficiency of Ptch is sufficient to promote RMS formation in mice.
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PMID:Unbalanced overexpression of the mutant allele in murine Patched mutants. 1201 44


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