Malignant peripheral nerve sheath tumor natural History, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
==Prognosis== | |||
Patient response to treatment will vary based on age, health, and the tolerance to medications and therapies. | |||
[[Metastasis]] occurs in about 39% of patients, most commonly to the lung. Features associated with poor prognosis include a large primary tumor (over 5 cm across), high [[grading (tumors)|grade]] disease, co-existent neurofibromatosis, and the presence of metastases.<ref name="Panigrahi"/> | |||
It is a rare tumor type, with a relatively poor prognosis in children.<ref name="pmid12632346">{{cite journal |author=Neville H, Corpron C, Blakely ML, Andrassy R |title=Pediatric neurofibrosarcoma |journal=J. Pediatr. Surg. |volume=38 |issue=3 |pages=343–6; discussion 343–6 |year=2003 |pmid=12632346 |doi=10.1053/jpsu.2003.50105}}</ref> | |||
In addition, MPNSTs are extremely threatening in NF1. In a 10-year institutional 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. | |||
<ref>{{cite journal|last=Zehou|first=Ouidad|title=Chemotherapy For The Treatment Of Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis 1: A 10-Year Institutional Review.|journal=Orphanet Journal Of Rare Diseases|date=2013|volume=8|issue=1|page=1|accessdate=7 Apr 2014}}</ref> | |||
Revision as of 16:17, 1 December 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Prognosis
Patient response to treatment will vary based on age, health, and the tolerance to medications and therapies.
Metastasis occurs in about 39% of patients, most commonly to the lung. Features associated with poor prognosis include a large primary tumor (over 5 cm across), high grade disease, co-existent neurofibromatosis, and the presence of metastases.[1]
It is a rare tumor type, with a relatively poor prognosis in children.[2]
In addition, MPNSTs are extremely threatening in NF1. In a 10-year institutional 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. [3]
References
- ↑
- ↑ Neville H, Corpron C, Blakely ML, Andrassy R (2003). "Pediatric neurofibrosarcoma". J. Pediatr. Surg. 38 (3): 343–6, discussion 343–6. doi:10.1053/jpsu.2003.50105. PMID 12632346.
- ↑ 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.
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