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[Response to everolimus in patients with giant cell astrocytoma associated to tuberous sclerosis complex].
- Source :
-
Revista de neurologia [Rev Neurol] 2014 Dec 01; Vol. 59 (11), pp. 497-502. - Publication Year :
- 2014
-
Abstract
- Introduction: Subependymal giant cell astrocytomas (SEGA) appear in 5-20% of patients with tuberous sclerosis complex (TSC) and are the most common brain tumours in TSC. They are benign tumours, of a glioneural stock, that develop mainly in the first two decades of life, generally close to the foramen of Monro, and can trigger hydrocephalus and intracranial hypertension. It is one of the leading causes of death in TSC. Recently mTOR inhibitors have proved to be a therapeutic alternative to surgical excision. AIM. To describe our experience of using everolimus to treat patients with SEGA and TSC.<br />Patients and Methods: We conducted a prospective study of the responses of patients with TSC and at least one SEGA undergoing growth.<br />Results: Three females and three males with a mean age of 12.3 years received treatment. One patient had previously undergone surgery due to SEGA with hydrocephalus. The maximum mean diameter of the SEGA on beginning treatment was 15.3 mm (range: 11.3-24.8 mm). Treatment was established with everolimus, 2.5 mg/day administered orally in patients with a body surface area < 1.2 m2, and 5 mg/day in patients with a body surface area > 1.2 m2. Two patients presented hypertriglyceridemia; one, anorexia; another, a mouth ulcer; and one, amenorrhoea. The mean reduction in the volume of the SEGA at three months of treatment was 46%, and the reduction remained steady in later control examinations (6-25 months).<br />Conclusions: Treatment with everolimus reduces the size of SEGA associated with TSC with an adequate safety profile, and constitutes an alternative to surgery in certain cases.
- Subjects :
- Administration, Oral
Adolescent
Amenorrhea chemically induced
Anorexia chemically induced
Astrocytoma etiology
Astrocytoma pathology
Brain Neoplasms etiology
Brain Neoplasms pathology
Child
Everolimus
Female
Giant Cells pathology
Humans
Hypertriglyceridemia chemically induced
Male
Neoplasm Proteins antagonists & inhibitors
Prospective Studies
Sirolimus administration & dosage
Sirolimus adverse effects
Sirolimus therapeutic use
Stomatitis, Aphthous chemically induced
TOR Serine-Threonine Kinases metabolism
Treatment Outcome
Tuberous Sclerosis genetics
Tuberous Sclerosis Complex 2 Protein
Tumor Burden drug effects
Tumor Suppressor Proteins genetics
Tumor Suppressor Proteins metabolism
Astrocytoma drug therapy
Brain Neoplasms drug therapy
Sirolimus analogs & derivatives
TOR Serine-Threonine Kinases antagonists & inhibitors
Tuberous Sclerosis complications
Subjects
Details
- Language :
- Spanish; Castilian
- ISSN :
- 1576-6578
- Volume :
- 59
- Issue :
- 11
- Database :
- MEDLINE
- Journal :
- Revista de neurologia
- Publication Type :
- Academic Journal
- Accession number :
- 25418144