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Survival in Malignant Peripheral Nerve Sheath Tumours: A Comparison between Sporadic and Neurofibromatosis Type 1-Associated Tumours.

Porter DE, Prasad V, Foster L, Dall GF, Birch R, Grimer RJ - Sarcoma (2009)

Bottom Line: However, 90 occurred sporadically whereas 33 were associated with neurofibromatosis type 1 (NF1).Tumour stage and site were significant prognostic indicators after univariate analysis.After multivariate analysis, however, only NF1 (P = .007) and tumour volume more than 200 m (P = .015) remained independent predictors of poor outcome.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedic Surgery, University of Edinburgh, Royal Infirmary of Edinburgh, Little France Crescent, Edinburgh EH16 4SU, UK.

ABSTRACT
We studied 123 patients with malignant peripheral nerve sheath tumours (MPNSTs) between 1979 and 2002. However, 90 occurred sporadically whereas 33 were associated with neurofibromatosis type 1 (NF1). Survival was calculated using Kaplan-Meier survival curves and we used Cox's proportional hazards model to identify independent prognostic factors. A 5-year survival for 110 nonmetastatic patients was 54%; (33% NF1 and 63% sporadic P = .015). Tumour stage and site were significant prognostic indicators after univariate analysis. After multivariate analysis, however, only NF1 (P = .007) and tumour volume more than 200 m (P = .015) remained independent predictors of poor outcome. We recommend that NF1 be taken into account during MPNST staging. As the survival rate in the NF group was dependant on tumour volume, routine screening of these patients with FDG PET and/or MRI may be warranted, thereby staging and controlling them at the earliest possible opportunity.

No MeSH data available.


Related in: MedlinePlus

Kaplan-Meier survival in patients without metastases at diagnosis.
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fig2: Kaplan-Meier survival in patients without metastases at diagnosis.

Mentions: Overall 5-year survival for all123 patients was 51% and was significantly worse for patients with NF1 thanthose with sporadic MPNSTs (32% versus 60%; P = .01). 13 patients (11%)had IUCC-TNM stage IV disease (metastases at diagnosis). Stage IVdisease was more common in NF1 patients (15%) than those with sporadic tumours(9%) but NF1 was still associated with a significantly worse 5-year survival ifpatients with stage IV disease were removed from the analysis (33% versus 63%; P = .015)(see Figure 2).


Survival in Malignant Peripheral Nerve Sheath Tumours: A Comparison between Sporadic and Neurofibromatosis Type 1-Associated Tumours.

Porter DE, Prasad V, Foster L, Dall GF, Birch R, Grimer RJ - Sarcoma (2009)

Kaplan-Meier survival in patients without metastases at diagnosis.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2666272&req=5

fig2: Kaplan-Meier survival in patients without metastases at diagnosis.
Mentions: Overall 5-year survival for all123 patients was 51% and was significantly worse for patients with NF1 thanthose with sporadic MPNSTs (32% versus 60%; P = .01). 13 patients (11%)had IUCC-TNM stage IV disease (metastases at diagnosis). Stage IVdisease was more common in NF1 patients (15%) than those with sporadic tumours(9%) but NF1 was still associated with a significantly worse 5-year survival ifpatients with stage IV disease were removed from the analysis (33% versus 63%; P = .015)(see Figure 2).

Bottom Line: However, 90 occurred sporadically whereas 33 were associated with neurofibromatosis type 1 (NF1).Tumour stage and site were significant prognostic indicators after univariate analysis.After multivariate analysis, however, only NF1 (P = .007) and tumour volume more than 200 m (P = .015) remained independent predictors of poor outcome.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedic Surgery, University of Edinburgh, Royal Infirmary of Edinburgh, Little France Crescent, Edinburgh EH16 4SU, UK.

ABSTRACT
We studied 123 patients with malignant peripheral nerve sheath tumours (MPNSTs) between 1979 and 2002. However, 90 occurred sporadically whereas 33 were associated with neurofibromatosis type 1 (NF1). Survival was calculated using Kaplan-Meier survival curves and we used Cox's proportional hazards model to identify independent prognostic factors. A 5-year survival for 110 nonmetastatic patients was 54%; (33% NF1 and 63% sporadic P = .015). Tumour stage and site were significant prognostic indicators after univariate analysis. After multivariate analysis, however, only NF1 (P = .007) and tumour volume more than 200 m (P = .015) remained independent predictors of poor outcome. We recommend that NF1 be taken into account during MPNST staging. As the survival rate in the NF group was dependant on tumour volume, routine screening of these patients with FDG PET and/or MRI may be warranted, thereby staging and controlling them at the earliest possible opportunity.

No MeSH data available.


Related in: MedlinePlus