Academic Journal

Experience using mTOR inhibitors for subependymal giant cell astrocytoma in tuberous sclerosis complex at a single facility

التفاصيل البيبلوغرافية
العنوان: Experience using mTOR inhibitors for subependymal giant cell astrocytoma in tuberous sclerosis complex at a single facility
المؤلفون: Kyoichi Tomoto, Ayataka Fujimoto, Chikanori Inenaga, Tohru Okanishi, Shin Imai, Masaaki Ogai, Akiko Fukunaga, Hidenori Nakamura, Keishiro Sato, Akira Obana, Takayuki Masui, Yoshifumi Arai, Hideo Enoki
المصدر: BMC Neurology, Vol 21, Iss 1, Pp 1-7 (2021)
بيانات النشر: BMC, 2021.
سنة النشر: 2021
المجموعة: LCC:Neurology. Diseases of the nervous system
مصطلحات موضوعية: Subependymal giant cell astrocytoma (SEGA), Tuberous sclerosis complex (TSC), Mammalian target of rapamycin inhibitor (mTORi), Surgery, Hydrocephalus, Interdisciplinary team, Neurology. Diseases of the nervous system, RC346-429
الوصف: Abstract Background Subependymal giant cell astrocytoma (SEGA) is occasionally seen in tuberous sclerosis complex (TSC). Two main options are currently available for treating SEGA: surgical resection or pharmacotherapy using mammalian target of rapamycin inhibitors (mTORi). We hypothesized that opportunities for surgical resection of SEGA would have reduced with the advent of mTORi. Methods We retrospectively reviewed the charts of patients treated between August 1979 and July 2020, divided into a pre-mTORi era group (Pre-group) of patients treated before November 2012, and a post-mTORi era group (Post-group) comprising patients treated from November 2012, when mTORi became available in Japan for SEGA. We compared groups in terms of treatment with surgery or mTORi. We also reviewed SEGA size, rate of acute hydrocephalus, recurrence of SEGA, malignant transformation and adverse effects of mTORi. Results In total, 120 patients with TSC visited our facility, including 24 patients with SEGA. Surgical resection was significantly more frequent in the Pre-group (6 of 7 patients, 86 %) than in the Post-group (2 of 17 patients, 12 %; p = 0.001). Acute hydrocephalus was seen in 1 patient (4 %), and no patients showed malignant transformation of SEGA. The group treated using mTORi showed significantly smaller SEGA compared with the group treated under a wait-and-see policy (p = 0.012). Adverse effects of pharmacotherapy were identified in seven (64 %; 6 oral ulcers, 1 irregular menstruation) of the 11 patients receiving mTORi. Conclusions The Post-group underwent surgery significantly less often than the Pre-group. Since the treatment option to use mTORi in the treatment of SEGA in TSC became available, opportunities for surgical resection have decreased in our facility.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1471-2377
Relation: https://doaj.org/toc/1471-2377
DOI: 10.1186/s12883-021-02160-5
URL الوصول: https://doaj.org/article/9f1ef9c91fe84761b969e82177b8ed86
رقم الانضمام: edsdoj.9f1ef9c91fe84761b969e82177b8ed86
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:14712377
DOI:10.1186/s12883-021-02160-5