Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome

Diencephalic syndrome is usually associated with tumors in the hypothalamic region, rarely occurring in patients with neurofibromatosis type 1 (NF1)-associated gliomas. We describe the clinical presentation and response to treatment in 3 patients with NF1 presenting with diencephalic syndrome as fir...

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Detalles Bibliográficos
Autores principales: Weiser, Annette, Hengartner, Heinz, Kottke, Raimund, Grehten, Patrice, Toelle, Sandra P., Gerber, Nicolas U., Grotzer, Michael A., Guerreiro Stucklin, Ana S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Online Articles: Clinical and Laboratory Observations
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10115491/
https://www.ncbi.nlm.nih.gov/pubmed/36730977
http://dx.doi.org/10.1097/MPH.0000000000002569
Descripción
Sumario:Diencephalic syndrome is usually associated with tumors in the hypothalamic region, rarely occurring in patients with neurofibromatosis type 1 (NF1)-associated gliomas. We describe the clinical presentation and response to treatment in 3 patients with NF1 presenting with diencephalic syndrome as first symptom of optic pathway/hypothalamic glioma (OPHG). Because of the rarity of this constellation, knowledge about the clinical course and best treatment options for patients with NF1-associated OPHG and diencephalic syndrome is still limited. All 3 patients showed good response to treatment with normalization of body mass index and decrease in tumor volume within 6 months.

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