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A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. [2]
Surgery is the most common method of treating peripheral nerve sheath tumors. [11] In malignant tumors, complete resection is the only known curative treatment (with a sufficiently wide margin or even amputation to improve prognosis). [12] For larger lesions or those with a more aggressive histology, adjuvant radiation is recommended. Novel or ...
Each nerve sheath tumor type has a few different associated features on MRI imaging. Neurofibromas and malignant peripheral nerve sheath tumors can be difficult to distinguish from each other and may require additional testing, including PET scans (18 FDG-PET). [1] Image-guided needle biopsies may be performed if there is concern for malignancy ...
A nervous system tumor is a tumor that arises within the nervous system, either the central nervous system (CNS) or the peripheral nervous system (PNS). [1] [2] Nervous system primary tumors include various types of brain tumor and spinal tumors, such as gliomas, and meningiomas (of the CNS), and schwannomas (of the PNS) and can be either benign or malignant.
These tumors are often benign but can sometimes be malignant. Common types of primary PNS tumors include: Schwannomas: Tumors arising from Schwann cells, which produce the myelin sheath surrounding peripheral nerves. A common subtype is the vestibular schwannoma (acoustic neuroma). [5] Neurofibromas: Benign tumors that grow on nerves, commonly ...
Neuromas tend to be benign (i.e. not cancerous); many nerve tumors, including those that are commonly malignant, are nowadays referred to by other terms. Neuromas can arise from different types of nervous tissue, including the nerve fibers and their myelin sheath, as in the case of genuine neoplasms (growths) like ganglioneuromas and neurinomas.
Malignant melanotic nerve sheath tumor (previously known as melanotic schwannoma) is a rare aggressive peripheral nerve sheath tumor that typically develops in conjunction with spinal or visceral autonomic nerves, consisting uniformly of Schwann cells displaying melanocytic differentiation.
For example, people with neurofibromatosis type I (also called von Recklinghausen disease, associated with alterations in the NF1 gene) are at an increased risk of developing soft-tissue sarcomas known as malignant peripheral nerve-sheath tumors.
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