Malignant peripheral nerve sheath tumor natural History, complications and prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]
Overview
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%. Large tumor size at presentation (typically >5 cm) has been the most consistently determined adverse prognostic factor. Other reported factors include tumor grade, truncal location, surgical margin status, local recurrence, and heterologous rhabdomyoblastic differentiation. The true prognostic impact of NF1 syndrome in MPNST remains somewhat in flux. Several large series report significantly worse outcomes for MPNST arising in the setting of NF1 compared with sporadic disease, with inferior responses to cytotoxic chemotherapy and 5-year survivals that are up to 50% worse.
Complications
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
- 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%.
- Large tumor size at presentation (typically >5 cm) has been the most consistently determined adverse prognostic factor.
- Other reported factors include tumor grade, truncal location, surgical margin status, local recurrence, and heterologous rhabdomyoblastic differentiation.
- The true prognostic impact of NF1 syndrome in MPNST remains somewhat in flux.
- Several large series report significantly worse outcomes for MPNST arising in the setting of NF1 compared with sporadic disease, with inferior responses to cytotoxic chemotherapy and 5-year survivals that are up to 50% worse.
References
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.
- ↑ 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.
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