1. Treatment of NF1-associated Optic Pathway/Hypothalamic Gliomas in Patients With Diencephalic Syndrome
- Author
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Weiser, Annette, Hengartner, Heinz; https://orcid.org/0000-0002-7327-1069, Kottke, Raimund; https://orcid.org/0000-0003-0166-2770, Grehten, Patrice, Toelle, Sandra P, Gerber, Nicolas U; https://orcid.org/0000-0002-1783-631X, Grotzer, Michael A, Guerreiro Stucklin, Ana S; https://orcid.org/0000-0003-3136-9241, Weiser, Annette, Hengartner, Heinz; https://orcid.org/0000-0002-7327-1069, Kottke, Raimund; https://orcid.org/0000-0003-0166-2770, Grehten, Patrice, Toelle, Sandra P, Gerber, Nicolas U; https://orcid.org/0000-0002-1783-631X, Grotzer, Michael A, and Guerreiro Stucklin, Ana S; https://orcid.org/0000-0003-3136-9241
- Abstract
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.
- Published
- 2023