Symptoms edit
-Neurological symptoms.[1] -Pain or discomfort: numbness, burning, or “pins and needles.”[2] -Dizzyness and/or loss of balance.[3]
Genetics edit
A malignant peripheral nerve sheath tumor is rare, but is one of the most common frequent soft tissue sarcoma in the pediatrics population. About half of these cases also happen to occur along with NF1 which is a genetic mutation on the 17th chromosome which causes tumors along the nervous system. The lifetime risk of having both of these conditions is at 8-13% while those with only MPNST have a 0.001% in the general population.[4] NF1 and MPNST are categorized as autosomal dominant disorders. This means when one receives an abnormal gene from a one of their parents, they will ultimately have that disorder. That person has a 50/50 chance of passing on that gene to their offspring. The pedigree to the right describes this genetic pattern.
Prognosis edit
MNPSTs are extremely threatening in NF1. In a 10 year instututional review for the treatment of chemotherapy for MPNST in NF1, which followed the cases of 1 per 2,500 in 3,300 live births, chemotherapy did not seem to reduce mortality, and its effectiveness should be questioned. Although with recent approaches with the molecular biology of MPNSTs, new therapies and prognostic factors are being examined. [5]
- ^ Valeyrie-Allanore, L. (2005). ""Symptoms Associated With Malignancy Of Peripheral Nerve Sheath Tumours: A Retrospective Study Of 69 Patients With Neurofibromatosis 1."". British Journal of Dermatology. 153 (1): 79–82. doi:10.1111/j.1365-2133.2005.06558.x. PMID 16029330.
- ^ Valeyrie-Allanore, L. (2005). ""Symptoms Associated With Malignancy Of Peripheral Nerve Sheath Tumours: A Retrospective Study Of 69 Patients With Neurofibromatosis 1."". British Journal of Dermatology. 153 (1): 79–82. doi:10.1111/j.1365-2133.2005.06558.x. PMID 16029330.
- ^ Valeyrie-Allanore, L. (2005). ""Symptoms Associated With Malignancy Of Peripheral Nerve Sheath Tumours: A Retrospective Study Of 69 Patients With Neurofibromatosis 1."". British Journal of Dermatology. 153 (1): 79–82. doi:10.1111/j.1365-2133.2005.06558.x. PMID 16029330.
- ^ Ferrari, Andrea (2007). ""Management Of Childhood Malignant Peripheral Nerve Sheath Tumor."". Pediatic Drugs. 9 (4): 239.
{{cite journal}}: More than one of|pages=and|page=specified (help); Unknown parameter|coauthors=ignored (|author=suggested) (help) - ^ Zehou, Ouidad (2013). ""Chemotherapy For The Treatment Of Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis 1: A 10-Year Institutional Review."". Orphanet Journal of Rare Diseases. 8 (1): 1. doi:10.1186/1750-1172-8-127. PMC 3766199. PMID 23972085.
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