Ad
related to: plexiform neurofibroma
Search results
Results from the WOW.Com Content Network
A neurofibroma is a benign nerve-sheath tumor in the peripheral nervous system.In 90% of cases, they are found as stand-alone tumors (solitary neurofibroma, solitary nerve sheath tumor [1] or sporadic neurofibroma [1]), while the remainder are found in persons with neurofibromatosis type I (NF1), an autosomal-dominant genetically inherited disease.
Neurofibroma (Localized, diffuse, plexiform subtype) Benign 5.3% of all benign soft tissue tumors Skin, with predominant dermal involvement, less frequently medium-sized nerves, a nerve plexus, a major nerve trunk, or spinal nerve roots; Bilateral and/or multiple spinal root involvement in NF1; Spinal cord compression
Plexiform neurofibromas (associated with neurofibromatosis type 1) have a higher risk of transforming into malignant tumors and require individual treatment plans. [1] Surgical resection is the treatment of choice for symptomatic plexiform neurofibromas, though a new drug, selumetinib was approved in 2020, as a systemic therapeutic for ...
Neurofibromatosis type I is the most common of the three types and is caused by genetic changes in the NF1 gene located on chromosome 17 (17q11.2). This gene encodes a cytoplasmic protein known the neurofibromin, which functions as a tumor suppressor and therefore serves as a signal regulator of cell proliferation and differentiation.
On the contrary, plexiform neurofibromas arise from multiple nerve fascicles and malignant transformation occurs in approximately 10% of cases. [14] They may result in significant morbidity as they may cause organ compression, vascular occlusions, bone destruction, pain and cosmetic issues. Plexiform neurofibromas are seen in 30-50% of patients ...
Neurofibromatosis type II (also known as MISME syndrome – multiple inherited schwannomas, meningiomas, and ependymomas) is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, and peripheral nerves. The types of tumors frequently associated with NF2 include vestibular ...
In neurofibromatosis type I, it has been theorized that local infiltration of the dura by plexiform neurofibromas leads to a weakening of the dural allowing the outpouching. A retrospective study found that a majority of dural ectasia were associated with nearby plexiform neurofibromas. [15]
The compound was discovered by Johnson & Johnson Pharmaceutical Research & Development, L.L.C, with registration number R115777.. For treatment of progressive plexiform neurofibromas associated with neurofibromatosis type I, it passed phase I clinical trials but was suspended (NCT00029354) in phase II.
Ad
related to: plexiform neurofibroma