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)

A large inbred family is described in which there were seven cases of Hodgkin's disease, three of lymphosarcoma, two of thymoma, two of common variable immunodeficiency, and single cases of retinoblastoma, neuroblastoma, and rhabdomyosarcoma. There have been no other lymphoma cases in the community during the past decade. Further study of this family may help to define the genetic basis for development of Hodgkin's disease and other disorders.
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PMID:Common variable immunodeficiency, Hodgkin's disease, and other malignancies in a Newfoundland family. 4 22

Children with malignancies resistant to conventional therapy were treated with cis-diamminedichloroplatinum (PDD), 15 to 20 mg/m2, given daily by rapid intravenous infusion for 5 days at 3-wk intervals. Eleven of 24 children with acute lymphocytic leukemia (ALL) received two or more courses; among these no remissions occurred. Fifty-four children with solid tumors were treated: 25 neuroblastoma, 9 rhabdomyosarcoma, 4 Ewing sarcoma, 2 testicular embryonal carcinoma, 2 retinoblastoma, and 12 miscellaneous tumors. One complete remission, 3 partial remissions, and 2 improvements were observed in children with neuroblastoma. One girl with metastatic osteogenic sarcoma achieved a partial remission. One child with metastatic testicular embryonal carcinoma showed improvement. The side effects were vomiting controlled by antiemetics in 26 children and transient elevations of serum creatinine and BUN in 14 children. Nineteen of 39 children with solid tumors, who received more than one course of PDD, had moderately severe myelosuppression caused by PDD. In summary, PDD is a promising agent in neuroblastoma, osteogenic sarcoma, and testicular embryonal carcinoma, and an ineffective agent in ALL. The effect of PDD on other types of solid tumors should be evaluated in the future.
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PMID:Cis-diamminedichloroplatinum (NSC-119875) in childhood malignancies: a Southwest Oncology Group study. 27 32

We reviewed the records of 1,323 patients with retinoblastoma treated here between 1922 and 1978. Twenty-eight patients underwent bilateral enucleation alone and received no additional treatment for their advanced tumors. Of the 28 patients, 22 (92%) survived. Four patients were lost to follow-up, and two died of metastatic retinoblastoma. Of 22 survivors of bilateral retinoblastoma treated with enucleation alone, three developed second tumors at a mean of 15.3 years after bilateral enucleation. One patient developed a rhabdomyosarcoma in the left temple region, the second patient developed an osteogenic sarcoma of the right femur, and the third developed a malignant melanoma of the left thigh. All three patients died of their disease. The incidence of second tumors in patients who survived bilateral retinoblastoma treated without radiation was 14% (three patients), which is comparable to a series previously reported in which patients were treated with enucleation and irradiation.
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PMID:Second tumors in nonirradiated bilateral retinoblastoma. 28 50

Functionally equivalent genetic maternal can be labelled by an epigenetic marking process and used differentially depending on whether its origin is maternal or paternal. This phenomenon is known as genomic imprinting and is manifested at either the chromosomal or gene level. Genomic imprinting seems to play an important role in cancer predisposition syndromes, and phenotypic consequences are evident in constitutional deletion syndromes and uniparental disomies. Moreover, there seems to be a preferential retention of paternal alleles in sporadic tumours such as Wilms' tumour, rhabdomyosarcoma, osteosarcoma and retinoblastoma. To investigate whether chromosomes involved in acquired abnormalities of haematologic neoplasms show a similar 'parent of origin' bias, we studied the inheritance of the translocated chromosomes 9 and 22 in cases of Philadelphia-chromosome-positive leukaemia, using unique specific chromosome band polymorphisms. Here we show that the translocated chromosome 9 was of paternal origin, whereas the translocated chromosomes 22 were derived exclusively from the maternal copy, in 11 cases with reliable polymorphisms. Our data therefore provide evidence that imprinting phenomena may play an important role in acquired tumour-specific chromosome rearrangements.
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PMID:Parental origin of chromosomes involved in the translocation t(9;22). 140 53

A case of a mammary leiomyosarcoma in a 23-year-old woman is presented. The tumor appeared 16 years after successful treatment of an embryonal rhabdomyosarcoma of the orbit. Rhabdomyosarcomas are the most frequent soft tissue tumors of childhood, the orbit and the paratesticular region being the most common primary site for this tumor. In contrast, leiomyosarcomas other than those evolving from the viscera or the urogenital organs are rare neoplasms at any age. With the improvement of cancer treatment and survival rates, the risk of late effects after successful treatment for malignant tumors during childhood is increasing. Growth, development and fertility may be impaired and cosmetically disturbing facial and dental complications are common. Development of novel primary tumors is a known further consequence of successful treatment of brain tumors, retinoblastoma and acute leukemias. This is the case when high dose local radiation therapy and/or chemotherapy, especially alkylating agents, were used. Development of novel primary tumors is also known after treatment of childhood rhabdomyosarcomas. This report is intended to show that a second primary tumor may occur many years after a first successfully treated malignant neoplasm, and that young people are at risk for development of tumors at sites that are uncommon to this age group.
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PMID:[Leiomyosarcoma of the breast 16 years following successful treatment of a rhabdomyosarcoma of the orbit in childhood]. 141 91

Twenty-six infants and children with orbital and ocular pathology were examined with ultrasound (US) utilizing real-time imaging and Duplex Pulsed Doppler evaluation. Twenty-two of these patients underwent concurrent orbital computed tomography (CT) and two had magnetic resonance imaging (MRI). Orbital and periorbital lesions included hemangioma, dermoid, lymphangioma, rhabdomyosarcoma, encephalocoele and abscess. Ocular lesions included infection, trauma, retinal detachment, retinoblastoma, Coat's disease, and persistent hyperplastic primary vitreous. High resolution US with Doppler provided unique diagnostic information in patients with penetrating ocular trauma, orbital and periorbital masses, and intraocular structural abnormalities. High resolution US examination of the eye and periorbital tissues is readily performed using widely available equipment and often delineates subtle structural abnormalities not shown by CT or MRI.
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PMID:High resolution ultrasound with Doppler: a diagnostic adjunct in orbital and ocular lesions in children. 150 82

The pattern of malignancies among indigenous Zambian children is described. The study, based upon an analysis of histopathology, autopsy and haematology records for a 10-year period (1980-1989), reveals a total of 525 neoplasms with a peak prevalence in the 5-9 year age group. Non-Hodgkin's lymphoma (17.5%) was the most common disorder followed by Burkitt's lymphoma (13.9%), retinoblastoma (11.4%), Kaposi's sarcoma (8.8%), Hodgkin's disease (5.9%), Wilms' tumour (5.9%), acute lymphocytic leukaemia (4%), rhabdomyosarcoma (3.4%), nasopharyngeal carcinoma (2.7%) and osteogenic sarcoma (2.1%). Kaposi's sarcoma and Hodgkin's disease revealed a significant male dominance; the former presented mainly in nodal form. Compared to an earlier report from Zambia (1968-1972), a significant increase in the incidence of Kaposi's sarcoma and nasopharyngeal carcinoma was noted in the present series.
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PMID:The pattern of paediatric malignancy in Zambia (1980-1989): a hospital-based histopathological study. 156 Apr 80

A series of fine-needle aspiration biopsies performed in 635 children were reviewed. The diagnoses rendered in these patients included malignant lymphoma in 139 (21.9%); Hodgkin's disease, 25 (3.9%); neuroblastoma, 58 (9.1%); Wilms' Tumor, 37 (5.8%); Ewing's sarcoma, 32 (5.0%); rhabdomyosarcoma, 25 (3.9%); retinoblastoma, 22 (3.5%); leukemia infiltrate, 33 (5.2%); and miscellaneous tumors, 52 (8.2%). In 171 patients (26.9%), the biopsy was nondiagnostic. The cytomorphological characteristics of these lesions are briefly described and illustrated. Salient morphological features are further correlated with histological and ultrastructural appearances. Immunocytochemical patterns of these tumors are also discussed briefly.
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PMID:Fine-needle aspiration biopsy of pediatric neoplasms: correlation between electron microscopy and immunocytochemistry in diagnosis and classification. 160 83

Orbital abnormalities encountered in the pediatric population differ substantially from those found in adult patients. Retinoblastoma, the most serious intraocular tumor, is often difficult to diagnose, but use of computed tomography (CT) (which reveals the characteristic focal calcification) and magnetic resonance (MR) imaging allows this tumor to be differentiated from pseudogliomas, such as Coats disease, and retrolental fibroplasia. CT and MR imaging help in the differentiation of orbital cellulitis from preseptal, lacrimal, and eyelid infectious processes and of orbital pseudotumor from Graves disease. In cases of orbital trauma, CT is excellent for detecting orbital fractures and metallic foreign bodies; MR imaging is better for depicting ocular and optic nerve injuries. Both modalities allow the differentiation of rhabdomyosarcoma from dermoid, cavernous hemangioma, and lymphangioma and provide helpful information for the diagnosis of many other tumors. Since CT and MR imaging have widely expanded the capabilities of orbital imaging, it is more important than ever before for radiologists to understand pediatric orbital disease.
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PMID:CT and MR imaging of the pediatric orbit. 160 40

The L1 cell adhesion molecule was initially identified and characterized in mouse as a cell-surface glycoprotein that mediates neuron-neuron and neuron-Schwann cell adhesion. We have characterized L1 in humans using cDNA structural and mRNA expression analyses. We present the entire coding sequence for human L1, which predicts a 1253-amino acid protein displaying a signal sequence, transmembrane segment, RGD sequence, and potential glycosylation and phosphorylation sites. Nucleotide and deduced amino acid sequence identities between human and mouse L1 are 85% and 87%, respectively. In contrast, the amino acid identity between human L1 and the L1-related molecule chicken Ng-CAM is only 45%. Using Northern blot analyses, a single L1 transcript of 5.5 kb is detected in human fetal brain and in neuroblastoma (IMR-32) and retinoblastoma (Y-79) cell lines. L1 is also expressed in the rhabdomyosarcoma cell lines RD and A-204, which display several muscle characteristics. Two forms of L1, which differ by the presence or absence of a 12-bp cytoplasmic segment, are expressed in both human and mouse. This segment is encoded by a single exon that can be alternately spliced to give rise to the two forms, which appear to be expressed in tissue-specific patterns.
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PMID:Variants of human L1 cell adhesion molecule arise through alternate splicing of RNA. 162 59


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