Clinical characteristics predicting internal neurofibromas in 357 children with neurofibromatosis-1: results from a cross-selectional study.

Emilie Sbidian 1, 2, 3 Smaïl Hadj-Rabia 4 Vincent Riccardi 5 Laurence Valeyrie-Allanore 1, 2, 6 Sébastien Barbarot 7 Olivier Chosidow 2 Salah Ferkal 8, 6 Diana Rodriguez 9 Pierre Wolkenstein 1, 2, 6 Sylvie Bastuji-Garin 1, 3, 6, 10, 11, *
* Auteur correspondant
1 LIC - Laboratoire d'Investigation Clinique
2 Service de Dermatologie
3 Pôle Recherche Clinique-Santé Publique
4 Service de Dermatologie
5 Internal Medicine, Clinical Genetics & Clinical Cytogenetics
6 Centre de référence des Neurofibromatoses - Ile-de-France
7 Service de Dermatologie
8 CIC - Centre d'Investigation Clinique
9 Service de neuropédiatrie [Trousseau]
10 URC - Unité de Recherche Clinique
11 Service de Santé Publique
Abstract : UNLABELLED: ABSTRACT: OBJECTIVE: To identify clinical characteristics associated with internal neurofibromas in children with NF1, as a means of ensuring the early identification of patients at high risk for malignant peripheral nerve-sheath tumors developed from preexisting internal neurofibromas. PATIENTS AND METHODS: We used data from two NF1 populations, in France and North America, respectively. The French database comprised 1083 patients meeting NIH diagnostic criteria for NF1 and the Neurofibromatosis Institute Database of North America comprised 703 patients. Patients younger than 17 years of age were eligible for our study if they had been evaluated for internal neurofibromas using computed tomography and/or magnetic resonance imaging. Clinical characteristics associated with internal neurofibromas by univariate analysis (P ≤ 0.15) were entered into a multiple logistic regression model after checking for potential interactions and confounding. Multiple imputation was used for missing values. RESULTS: Among the 746 children in the two databases, 357 (48%) met our inclusion criteria. Their mean age was 7.7 ± 5.0 years and there were 192 (53.8%) males. Internal neurofibromas were present in 35 (9.8%) patients. Internal neurofibromas developed earlier in females than in males and their prevalence increased during adolescence. Factors independently associated with internal neurofibromas were age (OR = 1.16 [1.07-1.27]), xanthogranulomas (OR = 5.85 [2.18-15.89]) and presence of both subcutaneous and plexiform neurofibromas (OR = 6.80 [1.52-30.44]). CONCLUSIONS: Several easily recognizable clinical characteristics indicate a high risk of internal neurofibromas in children with NF1 and, therefore, a need for very close monitoring.
Keywords : Neurofibromatosis 1 Internal neurofibromas Subcutaneous neurofibromas Children Cross-sectional study
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Orphanet Journal of Rare Diseases, BioMed Central, 2012, 7 (1), pp.62. 〈10.1186/1750-1172-7-62〉
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Emilie Sbidian, Smaïl Hadj-Rabia, Vincent Riccardi, Laurence Valeyrie-Allanore, Sébastien Barbarot, et al.. Clinical characteristics predicting internal neurofibromas in 357 children with neurofibromatosis-1: results from a cross-selectional study.. Orphanet Journal of Rare Diseases, BioMed Central, 2012, 7 (1), pp.62. 〈10.1186/1750-1172-7-62〉. 〈inserm-00748122〉

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