Juvenile Xanthogranuloma, Neurofibromatosis, and Juvenile Chronic Myelogenous Leukemia

Zvulunov, Alex; Barak, Yigal; Metzker, Aryeh

doi:10.1001/archderm.1995.01690200040007

Cited by 217 publications

(17 citation statements)

References 39 publications

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“…Finally, xanthogranulomas were independently associated with internal neurofibromas in our study. NF1 children with xanthogranulomas are at increased risk for juvenile chronic myelogenous leukemia [34]. Although it should be noted that this study included a small number of patients and has not been confirmed since.…”

Section: Discussionmentioning

confidence: 99%

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

Sbidian

Hadj‐Rabia

Riccardi³

et al. 2012

Orphanet J Rare Dis

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ObjectiveTo 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 methodsWe 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.ResultsAmong 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]).ConclusionsSeveral easily recognizable clinical characteristics indicate a high risk of internal neurofibromas in children with NF1 and, therefore, a need for very close monitoring.

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Section: Discussionmentioning

confidence: 99%

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

Sbidian

Hadj‐Rabia

Riccardi³

et al. 2012

Orphanet J Rare Dis

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“…JCML is a rare type of leukaemia, accounting for about 1% of childhood leukaemias, characterized by an onset in early childhood, an absence of Philadelphia chromosome and a poor prognosis [3]. The association of JXG and NF 1 is established and represents a true association [3].…”

Section: Discussionmentioning

confidence: 99%

“…The association of JXG and NF 1 is established and represents a true association [3]. Another well documented association is that of JXG and childhood leukaemia [3]. The triple association of JXG, NF 1 and leukaemia was found in a recent review to have a frequency 30- to 40-fold higher than expected, and children with JXG and NF1 were estimated to have a 20- to 32-fold higher risk of JCML than patients with NF 1 and no JXG [3].…”

Section: Discussionmentioning

confidence: 99%

“…Another well documented association is that of JXG and childhood leukaemia [3]. The triple association of JXG, NF 1 and leukaemia was found in a recent review to have a frequency 30- to 40-fold higher than expected, and children with JXG and NF1 were estimated to have a 20- to 32-fold higher risk of JCML than patients with NF 1 and no JXG [3]. The reason for this association is unknown but localization of the gene that encodes for granulocyte colony-stimulating factor to chromosome 17q 11-q 22 close to the NF gene provides a mechanism for the increased risk for tumourigenesis in NF 1 [6].…”

Section: Discussionmentioning

confidence: 99%

“…The association of JXG and neurofibromatosis type 1 (NF 1) is now established and represents a true association; another well documented association is that of JXG and childhood leukaemia (xantholeukaemia) [3]. The triple association of JXG, NF 1 and juvenile chronic myelogenous leukaemia (JCML) also represents a true association and the finding of JXG and NF 1 in children should alert the physician to a possible development of JCML.…”

Section: Introductionmentioning

confidence: 99%

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