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MCID: PRP025
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Peripheral Primitive Neuroectodermal Tumor malady |
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Sources: 17Genetics Home Reference, 30NIH Rare Diseases, 33OMIM, 22MalaCards See all sources Export this MalaCard |
Genetics Home Reference: Ewing sarcoma is a cancerous tumor that occurs in bones or soft tissues, such as cartilage or nerves. There are several types of Ewing sarcoma, including Ewing sarcoma of bone, extraosseous Ewing sarcoma, peripheral primitive neuroectodermal tumor (pPNET), and Askin tumor. These tumors are considered to be related because they have similar genetic causes. These types of Ewing sarcoma can be distinguished from one another by the tissue in which the tumor develops. Approximately 87 percent of Ewing sarcomas are Ewing sarcoma of bone, which is a bone tumor that usually occurs in the thigh bones (femurs), pelvis, ribs, or shoulder blades. Extraosseous (or extraskeletal) Ewing sarcoma describes tumors in the soft tissues around bones, such as cartilage. pPNETs occur in nerve tissue and can be found in many parts of the body. A type of pPNET found in the chest is called Askin tumor.17
MalaCards: Peripheral Primitive Neuroectodermal Tumor, also known as ewing sarcoma, is related to extraosseous ewing's sarcoma and ewing's family of tumors. An important gene associated with Peripheral Primitive Neuroectodermal Tumor is IGLC7 (immunoglobulin lambda constant 7). The drugs prednisone and prednisolone tebutate and the compounds askin and 12-o-tetradecanoylphorbol 13-acetate have been mentioned in the context of this disorder. Affiliated tissues include lung, ovary and cervix, and related mouse phenotype mortality/aging. NIH Rare Diseases: Ewing's sarcoma is a malignant (cancerous) bone tumor that affects children. It can occur any time during childhood and young adulthood, but usually develops during puberty, when bones are growing rapidly. The tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. It may also develop in the skull or the flat bones of the trunk. There are few symptoms. The most common is pain and occasionally swelling at the site of the tumor. Fever may also be present. The tumor often spreads (metastasis) to the lungs and other bones. The cause of Ewing's sarcoma is unknown.30 OMIM: 612219 |
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Sources: 6Disease Ontology, 8DISEASES, 30NIH Rare Diseases, 7diseasecard, 17Genetics Home Reference, 33OMIM, 32Novoseek , 43UMLS, 27NCIt, 40SNOMED-CT, 24MeSH See all sources |
Aliases & Descriptions:
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Sources: 33OMIM See all sources |
Clinical features from OMIM: 612219
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Sources: 4CenterWatch, 29NIH Clinical Center, 5ClinicalTrials, 43UMLS, 28NDF-RT See all sources |
Approved drugs:Search CenterWatch for peripheral primitive neuroectodermal tumor Drug clinical trials:Search ClinicalTrials for peripheral primitive neuroectodermal tumor Search NIH Clinical Center for peripheral primitive neuroectodermal tumor Search CenterWatch for peripheral primitive neuroectodermal tumor Inferred drug relations via UMLS/NDF-RT:43 28 cyclophosphamide, dactinomycin, dexamethasone, dexamethasone acetate, dexamethasone acetate pwdr [va product], dexamethasone phosphate, dexamethasone sodium phosphate, dexamethasone sodium phosphate pwdr [va product], etoposide, etoposide phosphate, linolenic acid, methylprednisolone, methylprednisolone acetate, methylprednisolone sodium succinate, prednisolone, prednisolone acetate, prednisolone acetate pwdr [va product], prednisolone pwdr [va product], prednisolone sodium phosphate, prednisolone tebutate, prednisone, prednisone pwdr [va product] |
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Sources: 22MalaCards See all sources |
MalaCards organs/tissues related to peripheral primitive neuroectodermal tumor:22Lung, Ovary, Cervix
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Sources: 25MGI See all sources |
Publications for genes affiliated with Peripheral Primitive Neuroectodermal Tumor
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Sources: 35PubMed See all sources |
Articles related to peripheral primitive neuroectodermal tumor:(show all 18)
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Sources: 1BioGPS See all sources |
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Sources: 32Novoseek , 18HMDB, 9DrugBank, 34PharmGKB, 42Tocris Bioscience See all sources |
Compounds related to peripheral primitive neuroectodermal tumor according to GeneDecks:(show all 32)
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Sources: 12Gene Ontology See all sources |
Cellular components related to peripheral primitive neuroectodermal tumor according to GeneDecks:
Molecular functions related to peripheral primitive neuroectodermal tumor according to GeneDecks:
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