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NAME Syndrome Nevi, Atrial myxoma, Myxoid neurofibroma, Ephelides Syndrome
NAME National Association of Medical Examiners; nevi, atrial myxoma, myxoid neurofibroma, ephelides [synd...
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NF neurofibroma
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  • JrId: 1630
    JournalTitle: Neurofibromatosis.
    MedAbbr: Neurofibromatosis
    ISSN: 1010-5662
    ESSN:
    IsoAbbr:
    NlmId: 8807525
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    Neurofibromatosis(nonmalignant)
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CancerWEB ¿µ¿µ ÀÇÇлçÀü ¸ÂÃã °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
neurofibroma <oncology, tumour> A neurofibroma is a smooth, polypoid, soft or firm tumour that arises from the Schwann cells and fibroblasts of the neurilemmal sheath. They may grow anywhere in the myelinated nervous system.
(27 Sep 1997)
neurofibroma, plexiform A type of neurofibroma representing an anomaly rather than a true neoplasm, in which the proliferation of schwann cells occurs from the inner aspect of the nerve sheath, thereby resulting in an irregularly thickened, distorted, tortuous structure. In some instances the process extends along the course of the nerve and may eventually involve the spinal roots and the spinal cord.
(12 Dec 1998)
neurofibromatosis <oncology> One of the most common disorders in genetics, neurofibromatosis encompasses at least two diseases, designated NF-1 and NF-2.
NF-1 or classic neurofibromatosis, is characterised by the familiar cafe- au-lait spots, axillary freckling, cutaneous and visceral neurofibromas (which sometimes undergo malignant transformation), gliomas, scoliosis, and Lisch nodules of the iris. NF-1 is associated with the the von Recklinghausen Neurofibromatosis locus that encodes the NF-1 protein, a GTPase activating protein which interacts with the ras proteins. The gene is located on chromosome 17.
NF-2, also called acoustic or central neurofibromatosis, features neurofibromas restricted to the acoustic nerve (usually bilateral) and the central nervous system, skin lesions may or may not be present. The gene is located on chromosome 22.
There are no biochemical markers of the disorder, but the cloning of both the NF-1 and NF-2 genes makes DNA-based diagnosis possible in some families. Both genes appear to be tumour suppressor genes. Both conditions are autosomal dominant, but the variable penetrance and expressivity and high frequency of new mutations make genetic counseling difficult.
Inheritance: autosomal dominant.
(29 Dec 1997)
neurofibromatosis 1 A congenital autosomal dominant disorder characterised by developmental changes in the nervous system, muscles, bones, and skin especially in those derived from the embryonic neural crest. There are multiple cutaneous tumours and tumours of the peripheral and central nervous system. The disease has been linked to mutations of the nf1 gene on chromosome 17.
(12 Dec 1998)
neurofibromatosis 2 Severe autosomal dominant disorder characterised especially by bilateral acoustic neuromas as well as other multiple tumours including meningiomas, ependymomas, spinal neurofibromas, and gliomas. The disease has been linked to mutations of the nf2 gene on chromosome 22.
(12 Dec 1998)
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storiform neurofibroma An uncommon variant of dermatofibrosarcoma protuberans containing heavily pigmented dendritic melanocytes scattered between spindle cells of the tumour.
Synonym: Bednar tumour, storiform neurofibroma.
(05 Mar 2000)
MeSH(Medical Subject Headings) ¸ÂÃã °Ë»ö (http://www.nlm.nih.gov) °á°ú : 5 ÆäÀÌÁö: 1
  • Neurofibroma - »õâ A moderately firm, benign, encapsulated tumor resulting from proliferation of SCHWANN CELLS and FIBROBLASTS that includes portions of nerve fibers. The tumors usually develop along peripheral or cranial nerves and are a central feature of NEUROFIBROMATOSIS 1, where they may occur intracranially or involve spinal roots. Pathologic features include fusiform enlargement of the involved nerve. Microscopic examination reveals a disorganized and loose cellular pattern with elongated nuclei intermixed with fibrous strands. (From Adams et al., Principles of Neurology, 6th ed, p1016)
    Synonyms : Neurofibromas
  • Neurofibroma, Plexiform - »õâ A type of neurofibroma manifesting as a diffuse overgrowth of subcutaneous tissue, usually involving the face, scalp, neck, and chest but occasionally occurring in the abdomen or pelvis. The tumors tend to progress, and may extend along nerve roots to eventually involve the spinal roots and spinal cord. This process is almost always a manifestation of NEUROFIBROMATOSIS 1. (From Adams et al., Principles of Neurology, 6th ed, p1016; J Pediatr 1997 Nov;131(5):678-82)
    Synonyms : Tumor Royale, Elephantiasis Neuromatoses, Neurofibromas, Plexiform, Neuroma, Plexiform, Pachydermatoceles, Plexiform Neurofibroma, Plexiform Neurofibromas, Plexiform Neuroma, Plexiform Neuromas
  • Neurofibromatoses - »õâ A group of disorders characterized by an autosomal dominant pattern of inheritance with high rates of spontaneous mutation and multiple neurofibromas or neurilemmomas. NEUROFIBROMATOSIS 1 (generalized neurofibromatosis) accounts for approximately 95% of cases, although multiple additional subtypes (e.g., NEUROFIBROMATOSIS 2, neurofibromatosis 3, etc.) have been described. (From Neurochirurgie 1998 Nov;44(4):267-72)
    Synonyms : Multiple Neurofibromas, Neurofibromatosis 3, Neurofibromatosis Syndrome, Multiple Neurofibroma, Neurofibroma, Multiple, Neurofibromas, Multiple, Neurofibromatosis 3s, Neurofibromatosis Syndromes, Syndrome, Neurofibromatosis, Syndromes, Neurofibromatosis
  • Neurofibromatosis 1 - »õâ An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).
    Synonyms : NF1 (Neurofibromatosis 1), Neurofibromatosis I, Neurofibromatosis Type 1, Neurofibromatosis Type I, Neurofibromatosis, Peripheral, NF 1, Neurofibromatosis, Peripheral, NF1, Neurofibromatosis, Type 1, Neurofibromatosis, Type I, Recklinghausen's Disease of Nerve
  • Neurofibromatosis 2 - »õâ An autosomal dominant disorder characterized by a high incidence of bilateral acoustic neuromas as well as schwannomas (NEURILEMMOMA) of other cranial and peripheral nerves, and other benign intracranial tumors including meningiomas, ependymomas, spinal neurofibromas, and gliomas. The disease has been linked to mutations of the NF2 gene (GENES, NEUROFIBROMATOSIS 2) on chromosome 22 (22q12) and usually presents clinically in the first or second decade of life.
    Synonyms : Bilateral Acoustic Neurofibromatosis, Familial Acoustic Neuromas, NF2 (Neurofibromatosis 2), Neurofibromatosis II, Neurofibromatosis Type 2, Neurofibromatosis Type II, Neurofibromatosis, Central NF2, Neurofibromatosis, Central, NF 2, Neurofibromatosis, Type 2
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neurofibroma tumor of the fibrous covering of a peripheral nerve
Ãâó: wordnet.princeton.edu/perl/webwn
neurofibromatosis autosomal dominant disease characterized by numerous neurofibromas and by spots on the skin and often by developmental abnormalities
Ãâó: wordnet.princeton.edu/perl/webwn
neurofibroma A benign tumor that develops from the cells and tissues that cover nerves.
Ãâó: www.stjude.org/glossary
neurofibromatosis a disease characterized by the growth of tumors (abnormal tissue growths) on various nerves in the body. The effects of these tumors vary, from going unnoticed to causing serious disability.
Ãâó: https://www.healthforums.com/library/1,1277,articl...
neurofibromatosis A genetic condition that affects the nervous system, muscles, bones and skin.
Ãâó: www.cnn.com/HEALTH/library/BN/00023.html
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neurofibroma tumor of the fibrous covering of a peripheral nerve
neurofibroma autosomal dominant disease characterized by numerous neurofibromas and by spots on the skin and often by developmental abnormalities
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