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Endocrine Long-Term Follow-Up of Children with Neurofibromatosis Type 1 and Optic Pathway Glioma .
- Source :
-
Hormone research in paediatrics [Horm Res Paediatr] 2017; Vol. 87 (3), pp. 179-188. Date of Electronic Publication: 2017 Mar 27. - Publication Year :
- 2017
-
Abstract
- Background/aims: Children with optic pathway glioma (OPG) face sequelae related to tumour location and treatment modalities. We aimed to assess the prevalence of hypothalamic-pituitary dysfunctions in children with neurofibromatosis type 1 (NF1) and OPG who did not receive radiotherapy or surgical resection. The causative role of tumour location on endocrinopathy development is investigated.<br />Methods: A retrospective follow-up study of 40 children with NF1 and OPG evaluated between August 1996 and May 2015 was undertaken. Patients who underwent radiotherapy or surgical resection were excluded and 36 patients were studied. Tumour location was classified according to the Dodge criteria: stage I, optic nerve alone; stage II, optic chiasm with or without optic nerve involvement; and stage III, involvement of the hypothalamus or other adjacent structures.<br />Results: Endocrinopathies were diagnosed in 20/36 (55.6%) children during a mean follow-up of 9.1 (0.2-13.6) years: 0/4 OPGs were Dodge stage I, 12/21 (57.1%) stage II, and 8/11 (72.7%) stage III. The first endocrinopathy was found at a mean age of 7.4 (5.0-13.2) years, 2.4 (0-6.7) years after tumour diagnosis. We found growth hormone deficiency (GHD; 36.1%), central precocious puberty (33.3%), obesity with insulin resistance/impaired glucose tolerance (11.1%), early puberty (5.5%), GH excess (5.5%), ACTH deficiency (5.5%), hypogonadotropic hypogonadism (2.7%), and thyrotropin deficiency (2.7%). GHD was transient in all of those who were retested.<br />Conclusion: This population is at high risk of endocrinopathies due to tumour location. Lifelong endocrine follow-up is recommended. .<br /> (© 2017 S. Karger AG, Basel.)
- Subjects :
- Child
Child, Preschool
Female
Follow-Up Studies
Humans
Male
Endocrine System Diseases epidemiology
Endocrine System Diseases etiology
Glioma drug therapy
Glioma epidemiology
Neurofibromatosis 1 drug therapy
Neurofibromatosis 1 epidemiology
Optic Nerve Neoplasms drug therapy
Optic Nerve Neoplasms epidemiology
Retrospective Studies
Subjects
Details
- Language :
- English
- ISSN :
- 1663-2826
- Volume :
- 87
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Hormone research in paediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 28346917
- Full Text :
- https://doi.org/10.1159/000458525