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Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: A population based study
Author(s) -
Laurencikas Evaldas,
Gavhed Désirée,
Stålemark Helen,
Hooft Ingrid van't,
Prayer Daniela,
Grois Nicole,
Henter JanInge
Publication year - 2011
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22791
Subject(s) - medicine , langerhans cell histiocytosis , incidence (geometry) , radiological weapon , histiocytosis , central nervous system , population , pathology , pediatrics , dermatology , radiology , disease , environmental health , physics , optics
Background Patients with Langerhans cell histiocytosis (LCH) may develop neurodegeneration and other central nervous system (CNS) dysfunctions revealed by brain magnetic resonance imaging (MRI). We estimated the incidence and pattern of pathological brain MRI findings in a well‐defined, population‐based cohort of children with LCH. Methods Among children under 15 years of age diagnosed with LCH in the Stockholm County during 1992–2001, brain MRI was performed at a single center in children with clinical and/or laboratory signs of CNS involvement, including endocrine dysfunction. Results Out of the 29 children (16 males, 13 females) diagnosed with LCH, brain MRI was performed based on clinical indications in 16 children (55%) with either abnormal endocrine findings (n = 6), such as diabetes insipidus (n = 5), low IGF‐1 (n = 1), or panhypopituitarism (n = 1), or clinical CNS symptoms (n = 10). CNS MRI abnormalities were demonstrated in eight children (28%), at a median time of 3.5 years after LCH diagnosis (range 1–11.4 years). Altogether 7 of the 29 children (24%) had MRI findings associated with neurodegeneration, corresponding to a minimal incidence of 2.1/10 6 children per year. Neurodegenerative abnormalities tended to be more frequent in patients with craniofacial involvement ( P = 0.12). Conclusions The minimal annual incidence rate of neurodegenerative associated radiographic findings in LCH is estimated at 2.1/10 6 children (24% of all children with LCH). An important question is whether all patients with LCH, or certain forms of LCH, should be recommended for a late follow‐up examination including MRI. In patients with CNS‐LCH, neurological, neuropsychological, neurophysiological, neurochemical and neuroradiological follow‐up assessment is suggested. Pediatr Blood Cancer 2011;56:250–257. © 2010 Wiley‐Liss, Inc.