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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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
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author Weiser, Annette
Hengartner, Heinz
Kottke, Raimund
Grehten, Patrice
Toelle, Sandra P.
Gerber, Nicolas U.
Grotzer, Michael A.
Guerreiro Stucklin, Ana S.
author_facet Weiser, Annette
Hengartner, Heinz
Kottke, Raimund
Grehten, Patrice
Toelle, Sandra P.
Gerber, Nicolas U.
Grotzer, Michael A.
Guerreiro Stucklin, Ana S.
author_sort Weiser, Annette
collection PubMed
description 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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spelling pubmed-101154912023-04-20 Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome Weiser, Annette Hengartner, Heinz Kottke, Raimund Grehten, Patrice Toelle, Sandra P. Gerber, Nicolas U. Grotzer, Michael A. Guerreiro Stucklin, Ana S. J Pediatr Hematol Oncol Online Articles: Clinical and Laboratory Observations 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. Lippincott Williams & Wilkins 2023-05 2022-10-20 /pmc/articles/PMC10115491/ /pubmed/36730977 http://dx.doi.org/10.1097/MPH.0000000000002569 Text en Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/)
spellingShingle Online Articles: Clinical and Laboratory Observations
Weiser, Annette
Hengartner, Heinz
Kottke, Raimund
Grehten, Patrice
Toelle, Sandra P.
Gerber, Nicolas U.
Grotzer, Michael A.
Guerreiro Stucklin, Ana S.
Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome
title Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome
title_full Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome
title_fullStr Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome
title_full_unstemmed Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome
title_short Treatment of NF1-Associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome
title_sort treatment of nf1-associated optic pathway/hypothalamic gliomas in patients with diencephalic syndrome
topic Online Articles: Clinical and Laboratory Observations
url 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
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