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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]
Most peripheral nerve tumors occur for unknown reasons. Some, including schwannomatosis and neurofibromatosis (types 1 and 2), are associated with recognized hereditary disorders. Others may be caused by gene mutations. In the case of schwannomatosis and neurofibromatosis, tumors can grow on or close to nerves anywhere in the body. Frequently ...
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.
The peripheral PNET (pPNET) is now thought to be virtually identical to Ewing sarcoma: "Current evidence indicates that both Ewing's sarcoma and PNET have a similar neural phenotype and, because they share an identical chromosome translocation, they should be viewed as the same tumor, differing only in their degree of neural differentiation.
In perineural invasion, cancer cells proliferate around peripheral nerves and eventually invade them. Cancer cells migrate in response to different mediators released by autonomic and sensory fibers. Tumor cells secrete CCL2 and CSF-1 to accumulate endoneurial macrophages and, at the same time, release factors that stimulate perineural invasion.
Neurofibromatosis type I, in which the nerve tissue grows tumors (neurofibromas) that may be benign, but may cause serious damage by compressing nerves and other tissues. [ 15 ] Neurofibromatosis type II , in which bilateral acoustic neuromas (tumors of the vestibulocochlear nerve or cranial nerve 8 (CN VIII) also known as schwannoma) develop ...
Secondary CNS tumors, or metastatic tumors, occur when cancer cells spread to the brain or spinal cord from a primary tumor in another part of the body. These tumors are more common than primary CNS tumors in adults and often originate from cancers of the lung , breast , skin , kidney , or colon .
If the cancer has spread to other areas of the body, chemotherapy may be used to shrink tumors and reduce the pain and discomfort they cause, but is unlikely to eradicate the disease. A combination of docetaxel and gemcitabine could be an effective chemotherapy regimen in patients with advanced soft-tissue sarcoma.