Malignant peripheral nerve sheath tumor natural History, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]
Overview
In general Malignant peripheral nerve sheath tumor is known to have high metastatic potential and poor prognosis..[1] long-term outcomes vary widely across multiple series, with 5-year survival ranging between 15% and 50%.
Complications
- A common complication of malignant peripheral nerve sheath tumor is metastasis.
- Metastasis occurs in about 39% of patients, most commonly to the lung.[2]
Prognosis
- Prognosis of malignant peripheral nerve sheath tumor is generally poor.[1]
- 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.[2] Malignant peripheral nerve sheath tumor is extremely threatening in NF1.[3]
References
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 Panigrahi S, Mishra SS, Das S, Dhir MK (2013). "Primary malignant peripheral nerve sheath tumor at unusual location". J Neurosci Rural Pract. 4 (Suppl 1): S83–6. doi:10.4103/0976-3147.116480. PMC 3808069. PMID 24174807.
- ↑ 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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