The relationship between neurofibromatosis type 1, juvenile xanthogranuloma, and malignancy: A retrospective case-control study - 14/12/17

Doi : 10.1016/j.jaad.2016.12.012

Carmen Liy-Wong, MD ^a,^b, Javed Mohammed, MD, FRCPC ^c, Amanda Carleton, MD, MSc ^d, Elena Pope, MD, MSc, FRCPC ^a,^b, Patricia Parkin, MD, FRCPC ^b, Irene Lara-Corrales, MD, MSc ^a,^b,^⁎
^a Section of Pediatric Dermatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
^b Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
^c Department of Pediatrics, Children's Hospital at London Health Science Centre, Western University, London
^d Department of Family Medicine, University of Toronto, Toronto, Ontario, Canada

^∗Correspondence to: Irene Lara-Corrales, MD, MSc, University of Toronto, The Hospital for Sick Children, 555 University Ave, Toronto, ON M5G1X8.University of Toronto, The Hospital for Sick Children, 555 University AveTorontoONM5G1X8

Abstract

Background

Neurofibromatosis type 1 (NF-1) predisposes individuals to the development of benign and malignant tumors. The association of NF-1, juvenile xanthogranuloma (JXG), and juvenile myelomonocytic leukemia has been described in the literature. It is unclear whether JXG alone constitute a risk factor for leukemia or other malignancies in children with NF-1.

Objective

To determine if there is an association between NF-1, JXG, and malignancy.

Methods

We conducted a retrospective case-control study comparing children with NF-1 and malignancy (cases) with sex- and age-matched children with NF-1 without malignancy (controls).

Results

We identified 739 patients with NF-1 over a 20-year period, 14 of whom also had a diagnosis of malignancy. These cases include 9 (64%) boys and 5 (36%) girls. JXG were found in 4/14 (28.5%) cases and 6/29 (21%) controls (odds ratio 1.5, 95% confidence interval 0.35-6.6, P = .56).

Limitations

Retrospective design, small number of cases, and inconsistent documentation of clinical findings, including age at disappearance of JXG.

Conclusions

Juvenile xanthogranulomas do not appear to confer an increased risk for malignancy in children with NF-1.

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Key words : juvenile xanthogranuloma, leukemia, malignancy, malignant tumors, neurofibromatosis type 1

Abbreviations used : JMML, JXG, NF-1

Plan

	Funding sources: Supported by The Hospital for Sick Children Foundation Neurofibromatosis Research Fund.
	Conflicts of interest: None declared.
	Reprints not available from the authors.

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Vol 76 - N° 6

P. 1084-1087 - juin 2017 Retour au numéro

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The relationship between neurofibromatosis type 1, juvenile xanthogranuloma, and malignancy: A retrospective case-control study - 14/12/17

Abstract

Background

Objective

Methods

Results

Limitations

Conclusions

Plan

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