Katarzyna Kotulska1, Julita Borkowska2, Marcin Roszkowski3, Marek Mandera4, Paweł Daszkiewicz3, Krzysztof Drabik3, Elzbieta Jurkiewicz5, Magdalena Larysz-Brysz6, Katarzyna Nowak5, Wiesława Grajkowska7, Dorota Domańska-Pakieła2, Sergiusz Jóźwiak2. 1. Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland; Department of Science, The Children's Memorial Health Institute, Warsaw, Poland. Electronic address: k.kotulska@czd.pl. 2. Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland. 3. Department of Neurosurgery, The Children's Memorial Health Institute, Warsaw, Poland. 4. Department of Pediatric Neurosurgery, Silesian Medical University, Katowice, Poland. 5. Department of Radiology, The Children's Memorial Health Institute, Warsaw, Poland. 6. Department of Physiology, Silesian Medical University, Katowice, Poland. 7. Department of Pathology, The Children's Memorial Health Institute, Warsaw, Poland.
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 <2 cm, between 2 and 3 cm, between 3 and 4 cm, >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.
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 complexpatients 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 <2 cm, between 2 and 3 cm, between 3 and 4 cm, >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 complexpatients should be thoroughly screened for subependymal giant cell astrocytoma growth, and early treatment should be considered in selected patients.
Authors: Paolo Curatolo; Marit Bjørnvold; Patricia E Dill; José Carlos Ferreira; Martha Feucht; Christoph Hertzberg; Anna Jansen; Sergiusz Jóźwiak; J Christopher Kingswood; Katarzyna Kotulska; Alfons Macaya; Romina Moavero; Rima Nabbout; Bernard A Zonnenberg Journal: Drugs Date: 2016-04 Impact factor: 9.546
Authors: Krinio Giannikou; Zachary Zhu; Jaegil Kim; Kellen D Winden; Magdalena E Tyburczy; David Marron; Joel S Parker; Zachary Hebert; Anika Bongaarts; Len Taing; Henry W Long; William V Pisano; Sanda Alexandrescu; Brianna Godlewski; Mark Nellist; Katarzyna Kotulska; Sergiusz Jozwiak; Marcin Roszkowski; Marek Mandera; Elizabeth A Thiele; Hart Lidov; Gad Getz; Orrin Devinsky; Michael S Lawrence; Keith L Ligon; David W Ellison; Mustafa Sahin; Eleonora Aronica; David M Meredith; David J Kwiatkowski Journal: Mod Pathol Date: 2020-10-13 Impact factor: 8.209