Surgical Treatment of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex Patients

• Published in: Pediatric neurology 2014-04, Vol.50 (4), p.307-312
• Main Authors: Kotulska, Katarzyna, MD, PhD, Borkowska, Julita, MD, Roszkowski, Marcin, MD, PhD, Mandera, Marek, MD, PhD, Daszkiewicz, Paweł, MD, PhD, Drabik, Krzysztof, MD, Jurkiewicz, Elzbieta, MD, PhD, Larysz-Brysz, Magdalena, PhD, Nowak, Katarzyna, MD, Grajkowska, Wiesława, MD, PhD, Domańska-Pakieła, Dorota, MD, PhD, Jóźwiak, Sergiusz, MD, PhD
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Age Factors; Astrocytoma - complications; Astrocytoma - genetics; Astrocytoma - pathology; Astrocytoma - surgery; Brain - pathology; Brain - surgery; Brain Neoplasms - complications; Brain Neoplasms - genetics; Brain Neoplasms - pathology; Brain Neoplasms - surgery; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Magnetic Resonance Imaging; Male; mTOR inhibitors; Mutation; Neurology; Neurosurgical Procedures - adverse effects; outcome; Pediatrics; prognostic factor; Risk Factors; subependymal giant cell astrocytoma; surgery; Treatment Outcome; Tuberous Sclerosis - complications; Tuberous Sclerosis - genetics; Tuberous Sclerosis - pathology; tuberous sclerosis complex; Tumor Suppressor Proteins - genetics; Young Adult
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/j.pediatrneurol.2013.12.004

Abstract Background Subependymal giant cell astrocytoma is a brain tumor associated with tuberous sclerosis complex. There are two treatment options for subependymal giant cell astrocytomas: surgery or mammalian target of rapamycin inhibitor. The analysis of outcome of subependymal giant cell astrocytoma surgery may help characterize the patients who may benefit from pharmacotherapy. Methods Sixty-four subependymal giant cell astrocytoma surgeries in 57 tuberous sclerosis complex patients with at least a 12-month follow-up were included in the study. The tumor size, age of the patients, mutation in the TSC1 or TSC2 gene, indication for the surgery, and postsurgical complications were analyzed. Results The mean age of patients at surgery was 9.7 years. Mean follow-up after surgery was 63.7 months. Thirty-seven (57.8%) tumors were symptomatic and 27 (42.2%) were asymptomatic. Patients with TSC2 mutations developed subependymal giant cell astrocytoma at a significantly younger age than individuals with TSC1 mutations. Four patients (6.2% of all surgeries) died after surgery. Surgery-related complications were reported in 0%, 46%, 83%, 81%, and 67% of patients with tumors 4 cm, and bilateral subependymal giant cell astrocytomas, respectively, and were most common in children younger than 3 years of age. The most common complications included hemiparesis, hydrocephalus, hematoma, and cognitive decline. Conclusions Our study indicates that subependymal giant cell astrocytoma surgery is associated with significant risk in individuals with bilateral subependymal giant cell astrocytomas, tumors bigger than 2 cm, and in children younger than 3 years of age. Therefore, tuberous sclerosis complex patients should be thoroughly screened for subependymal giant cell astrocytoma growth, and early treatment should be considered in selected patients.