Growth dynamics of plexiform neurofibromas

A retrospective cohort study of 201 patients with neurofibromatosis 1

Rosa Nguyen, Eva Dombi, Brigitte C. Widemann, Jeffrey Solomon, Carsten Fuensterer, Lan Kluwe, Jan M. Friedman, Victor Felix Mautner

Research output: Contribution to journalArticle

Abstract

Background: To examine the natural growth dynamics of internal plexiform neurofibromas (PNs) in patients with neurofibromatosis 1 (NF1). Methods. Two hundred and one NF1 patients underwent whole body MRI (WBMRI). Tumour burden was estimated volumetrically. Non-parametric Spearman's rho correlation coefficients were used to analyse the relationship of growth rate to tumour volume and age. Chi-squared and Mann-Whitney U tests were used for analysing the association of tumour occurrence with sex or age. Chi-squared tests were used to analyse the association of tumour growth with age group. Results: Seventy-one of 171 patients with serial WBMRI exams had internal PNs (median follow up 2.2 years [1.1 to 4.9 years]). Median whole body tumour volume was 86.4 mL [5.2 to 5878.5 mL]) with a median growth rate of 3.7%/year (-13.4 to 111%/year) that correlated with larger whole body tumour volume (P

Original languageEnglish (US)
Article number75
JournalOrphanet Journal of Rare Diseases
Volume7
Issue number1
DOIs
StatePublished - 2012
Externally publishedYes

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Plexiform Neurofibroma
Neurofibromatosis 1
Tumor Burden
Cohort Studies
Retrospective Studies
Growth
Nonparametric Statistics
Neoplasms
Age Groups

Keywords

  • MRI scan
  • Neurofibroma
  • Neurofibromatosis 1
  • Plexiform
  • Tumour burden

ASJC Scopus subject areas

  • Medicine(all)
  • Genetics(clinical)
  • Pharmacology (medical)

Cite this

Nguyen, R., Dombi, E., Widemann, B. C., Solomon, J., Fuensterer, C., Kluwe, L., ... Mautner, V. F. (2012). Growth dynamics of plexiform neurofibromas: A retrospective cohort study of 201 patients with neurofibromatosis 1. Orphanet Journal of Rare Diseases, 7(1), [75]. https://doi.org/10.1186/1750-1172-7-75

Growth dynamics of plexiform neurofibromas : A retrospective cohort study of 201 patients with neurofibromatosis 1. / Nguyen, Rosa; Dombi, Eva; Widemann, Brigitte C.; Solomon, Jeffrey; Fuensterer, Carsten; Kluwe, Lan; Friedman, Jan M.; Mautner, Victor Felix.

In: Orphanet Journal of Rare Diseases, Vol. 7, No. 1, 75, 2012.

Research output: Contribution to journalArticle

Nguyen, R, Dombi, E, Widemann, BC, Solomon, J, Fuensterer, C, Kluwe, L, Friedman, JM & Mautner, VF 2012, 'Growth dynamics of plexiform neurofibromas: A retrospective cohort study of 201 patients with neurofibromatosis 1', Orphanet Journal of Rare Diseases, vol. 7, no. 1, 75. https://doi.org/10.1186/1750-1172-7-75
Nguyen, Rosa ; Dombi, Eva ; Widemann, Brigitte C. ; Solomon, Jeffrey ; Fuensterer, Carsten ; Kluwe, Lan ; Friedman, Jan M. ; Mautner, Victor Felix. / Growth dynamics of plexiform neurofibromas : A retrospective cohort study of 201 patients with neurofibromatosis 1. In: Orphanet Journal of Rare Diseases. 2012 ; Vol. 7, No. 1.
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