التفاصيل البيبلوغرافية
العنوان: |
Neoadjuvant B-RAF and MEK Inhibitor Targeted Therapy for Adult Papillary Craniopharyngiomas: A New Treatment Paradigm |
المؤلفون: |
Calvanese, Francesco, Jacquesson, Timothée, Manet, Romain, Vasiljevic, Alexandre, Lasolle, Hélène, Ducray, Francois, Raverot, Gerald, Jouanneau, Emmanuel |
المصدر: |
Frontiers in Endocrinology ; volume 13 ; ISSN 1664-2392 |
بيانات النشر: |
Frontiers Media SA |
سنة النشر: |
2022 |
المجموعة: |
Frontiers (Publisher - via CrossRef) |
الوصف: |
Background Surgical and clinical management of craniopharyngiomas is associated with high long-term morbidity especially in the case of hypothalamic involvement. Improvements in knowledge of craniopharyngioma molecular biology may offer the possibility of safe and effective medical neoadjuvant treatments in a subset of patients harboring papillary subtype tumors with a BRAFV600E mutation. Method We report herein two cases of tubero-infundibular and ventricular Papillary Craniopharyngiomas in which BRAF/MEK inhibitor combined therapy was used as adjuvant (Case 1) or neoadjuvant (Case 2) treatment, with a 90% reduction in tumor volume observed after only 5 months. In Case 2 the only surgical procedure used was a minimal invasive biopsy by the trans-ventricular neuroendoscopic approach. As a consequence, targeted therapy was administered in purely neoadjuvant fashion. After shrinkage of the tumor, both patients underwent fractionated radiotherapy on the small tumor remnant to achieve long-term tumor control. A review of a previously reported case has also been performed. Result This approach led to tumor control with minimal long-term morbidity in both cases. No side effects or complications were reported after medical treatment and adjuvant radiotherapy. Conclusion Our experience and a review of the literature argue for a change in the current treatment paradigm for Craniopharyngiomas (CPs). In giant and invasive tumors, confirmation of BRAFV600E mutated PCPs by biopsy and BRAF/MEK inhibitor therapy before proposing other treatments may be useful to improve long term outcomes for patients. |
نوع الوثيقة: |
article in journal/newspaper |
اللغة: |
unknown |
DOI: |
10.3389/fendo.2022.882381 |
DOI: |
10.3389/fendo.2022.882381/full |
الإتاحة: |
https://doi.org/10.3389/fendo.2022.882381Test |
حقوق: |
https://creativecommons.org/licenses/by/4.0Test/ |
رقم الانضمام: |
edsbas.C9AB41AD |
قاعدة البيانات: |
BASE |