Endocrine involvement in pediatric-onset langerhans' cell histiocytosis: a population-based study

Donadieu, Jean; Rolon, Maria-Alejandra; Thomas, Caroline; Brugières, Laurence; Plantaz, Dominique; Emile, Jean François; Frappaz, Didier; Dávid, M; Brauner, Raja; Généreau, T; Debray, Dominique; Cabrol, Sylvie; Barthez, Marie-Anne; Hoang-Xuân, Khê; Polak, Michel; Group, for the French LCH Study

doi:10.1016/j.jpeds.2003.12.030

Cited by 177 publications

(129 citation statements)

References 17 publications

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“…1,8,12,13 Recent studies indicate that the radiologic ND-LCH is more frequently detected in patients with craniofacial lesions or neuroendocrine problems. 14,15 The routine use of MR imaging in the evaluation of such patients led to an increasing number of reported cases. The wide range of radiologic findings in ND-LCH has been extensively described but not in the context of the course of the disease.…”

Section: Discussionmentioning

confidence: 99%

Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis

Prosch¹,

Grois²,

Wnorowski³

et al. 2007

American Journal of Neuroradiology

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BACKGROUND AND PURPOSE: MR imaging signal intensity abnormalities in the cerebellum, the pons, and the basal ganglia, compatible with a neurodegenerative process (ND) were reported in up to 10% of patients with Langerhans cell histiocytosis (LCH). Although the imaging features of ND-LCH have been extensively described, the temporal course of ND-LCH has not been assessed as of yet. The purpose of this study was to describe the long-term course of MR imaging signal intensity abnormalities in ND-LCH on T1-and T2-weighted images.

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Section: Discussionmentioning

confidence: 99%

Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis

Prosch¹,

Grois²,

Wnorowski³

et al. 2007

American Journal of Neuroradiology

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“…Clinical manifestations of histiocytoses are highly variable, although certain tissues and organs are more frequently affected such as bones, skin, lungs, and liver (1). While local control and combination chemotherapy can be curative in some, many patients experience disease recurrence, and others develop irreversible end-organ injury associated with significant morbidity such as diabetes insipidus (DI) and anterior pituitary dysfunction, neurodegeneration, and end-stage hepatic cirrhosis (3)(4)(5)(6). The etiopathogenesis of LCH and the other histiocytoses remained unclear for decades, until the identification of recurring BRAF V600E mutations (7,8) and more recently mutations in the downstream gene MAP2K1 in LCH and ECD (9,10).…”

Section: Introductionmentioning

confidence: 99%

Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes

et al. 2017

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“…All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem involvement. Apart from hypothalamic-pituitary dysfunction, mostly diabetes insipidus, central nervous system (CNS) complications occur in about 5% of cases [1,2].…”

Section: Introductionmentioning

confidence: 99%

MRI features of neurodegenerative Langerhans cell histiocytosis

et al. 2006

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CNS complications of LCH include "space occupying" lesions corresponding to histiocytic granulomas and "neurodegenerative" presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH.

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Endocrine involvement in pediatric-onset langerhans' cell histiocytosis: a population-based study

Cited by 177 publications

References 17 publications

Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis

Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis

Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes

MRI features of neurodegenerative Langerhans cell histiocytosis

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