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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

Tumour frequency by site.
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fig1: Tumour frequency by site.

Mentions: Of the 123 patients in this study with MPNSTs, 33patients (27%) had NF1. NF1 patientswere significantly younger at diagnosis than those with sporadic tumours with amedian age of 26 years compared with 53 years for sporadic MPNSTs, (X2 = 23.65, P < .001). There were alsosignificant differences in the distribution of the site of tumours between thetwo groups with relative overrepresentation of peripheral limb tumours in the sporadicgroup and axial tumours in the NF1 group (X2 = 24.3, P < .001) (see Figure 1). There were no significantdifferences in the tumour volumes found in the NF1 and sporadic groups (P = .36).


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)

Tumour frequency by site.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig1: Tumour frequency by site.
Mentions: Of the 123 patients in this study with MPNSTs, 33patients (27%) had NF1. NF1 patientswere significantly younger at diagnosis than those with sporadic tumours with amedian age of 26 years compared with 53 years for sporadic MPNSTs, (X2 = 23.65, P < .001). There were alsosignificant differences in the distribution of the site of tumours between thetwo groups with relative overrepresentation of peripheral limb tumours in the sporadicgroup and axial tumours in the NF1 group (X2 = 24.3, P < .001) (see Figure 1). There were no significantdifferences in the tumour volumes found in the NF1 and sporadic groups (P = .36).

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