Tumors of the peripheral nervous system. 1983

I M Ariel

Most tumors (benign and malignant) do not arise from the nerves per se, but from the supporting cells; tumors arising from the cells of Schwann are termed schwannoma or neurilemoma--benign or malignant. Surgical extirpation is the only way of treating these tumors. Radiation therapy can offer significant palliation and prolongation of life, but no cures have been observed. Other forms of therapy (chemotherapy, immunotherapy, etc.) have little to offer at this time. Benign tumors can be treated by local surgical extirpation; malignant tumors must be radically resected, including major amputations where indicated. Certain dermatomes have a proclivity to produce tumors, and new growths (benign and malignant) proximal to the one clinically apparent can be expected in certain patients. Neurofibromatosis (von Recklinghausen's disease) is a genetic error of metabolism with a proclivity to produce multiple neurofibrosarcomas, and in about 10 percent of the patients, malignant neurilemomas. Of 100 patients with malignant neurilemomas treated by the author, 74 were considered determinate; among them, the 10-year "cure" rate was 32 percent. Patients with von Recklinghausen's disease had almost as good a 10-year survival rate as those with solitary malignant schwannoma (30 percent vs 39 percent).

UI MeSH Term Description Entries
D008297 Male Males
D009364 Neoplasm Recurrence, Local The local recurrence of a neoplasm following treatment. It arises from microscopic cells of the original neoplasm that have escaped therapeutic intervention and later become clinically visible at the original site. Local Neoplasm Recurrence,Local Neoplasm Recurrences,Locoregional Neoplasm Recurrence,Neoplasm Recurrence, Locoregional,Neoplasm Recurrences, Local,Recurrence, Local Neoplasm,Recurrence, Locoregional Neoplasm,Recurrences, Local Neoplasm,Locoregional Neoplasm Recurrences,Neoplasm Recurrences, Locoregional,Recurrences, Locoregional Neoplasm
D009410 Nerve Degeneration Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways. Neuron Degeneration,Degeneration, Nerve,Degeneration, Neuron,Degenerations, Nerve,Degenerations, Neuron,Nerve Degenerations,Neuron Degenerations
D009442 Neurilemmoma A neoplasm that arises from SCHWANN CELLS of the cranial, peripheral, and autonomic nerves. Clinically, these tumors may present as a cranial neuropathy, abdominal or soft tissue mass, intracranial lesion, or with spinal cord compression. Histologically, these tumors are encapsulated, highly vascular, and composed of a homogenous pattern of biphasic fusiform-shaped cells that may have a palisaded appearance. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp964-5) Neurinoma,Schwannoma,Schwannomatosis, Plexiform,Neurilemoma,Neurilemmomas,Neurilemomas,Neurinomas,Plexiform Schwannomatoses,Plexiform Schwannomatosis,Schwannomas
D009455 Neurofibroma 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) Neurofibromas
D009456 Neurofibromatosis 1 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). Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D009463 Neuroma A tumor made up of nerve cells and nerve fibers. (Dorland, 27th ed) Neuromas
D010524 Peripheral Nervous System Neoplasms Neoplasms which arise from peripheral nerve tissue. This includes NEUROFIBROMAS; SCHWANNOMAS; GRANULAR CELL TUMORS; and malignant peripheral NERVE SHEATH NEOPLASMS. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp1750-1) Peripheral Nerve Neoplasms,Peripheral Nerve Neoplasms, Benign,Peripheral Nerve Neoplasms, Malignant,Peripheral Nerve Neoplastic Infiltration,Peripheral Nerve Tumors,Peripheral Nervous System Benign Neoplasms,Peripheral Nervous System Malignant Neoplasms,Neoplasm, Peripheral Nerve,Neoplasms, Peripheral Nerve,Nerve Neoplasm, Peripheral,Nerve Neoplasms, Peripheral,Nerve Tumor, Peripheral,Nerve Tumors, Peripheral,Peripheral Nerve Neoplasm,Peripheral Nerve Tumor,Tumor, Peripheral Nerve,Tumors, Peripheral Nerve
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup

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