Flavio Giordano1, Carla Moscheo2, Matteo Lenge3,4, Roberto Biagiotti5, Francesco Mari4, Iacopo Sardi2, Anna Maria Buccoliero6, Lorenzo Mongardi7, Eleonora Aronica8,9, Renzo Guerrini4, Lorenzo Genitori3. 1. Department of Neurosurgery, Children's Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy. flavio.giordano@meyer.it. 2. Neuro-oncology Unit, Children's Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy. 3. Department of Neurosurgery, Children's Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy. 4. 3. Pediatric Neurology, Children's Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy. 5. Division of Prenatal Diagnosis, Children's Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy. 6. Pathology Unit, Children's Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy. 7. Neurosurgery, Sant'Anna Hospital, Via Aldo Moro, Ferrara, 44124, Italy. 8. Department of (Neuro) Pathology, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands. 9. Stichting Epilepsie Instellingen Nederland (SEIN), Heemstede, The Netherlands.
Abstract
BACKGROUND: Subependymal giant cell astrocytomas (SEGA) are benign tumors characteristic of tuberous sclerosis complex (TSC) that may cause hydrocephalus. Various treatments are nowadays available as mTOR inhibitors or surgery. Surgery is still a valid option especially for symptomatic and larger tumors. METHODS: From January 1994 to December 2015, 31 TSC patients harboring SEGA underwent surgery at the Department of Neurosurgery of the Meyer Pediatric Hospital, Florence. Indications for surgery were tumor size and location, growth and cystization/hemorrhage, and hydrocephalus. Clinical data, preoperative and postoperative MRI, recurrence rate, further surgical procedures, and related complications were analyzed. RESULTS: A total of 44 surgeries were performed in 31 TSC patients affected by SEGA, achieving gross total removal (GTR) and subtotal removal (STR), respectively, in 36 and 8 patients. Recurrences occurred in 11 patients; 9 of them underwent further surgical procedures and 2 were treated with mTOR pathway inhibitors. Surgical morbidity and mortality were, respectively, 22.7% and 2.3%. After a mean follow-up of 4.9 years, 90% of patients were tumor-free with good neurological status in 93.3%; twelve (40%) had a ventriculo-peritoneal shunt (VPS) for hydrocephalus. CONCLUSIONS: The present series confirms that the surgical approach, combined with mTOR inhibitors, is still a valid option for the treatment of SEGAs.
BACKGROUND:Subependymal giant cell astrocytomas (SEGA) are benign tumors characteristic of tuberous sclerosis complex (TSC) that may cause hydrocephalus. Various treatments are nowadays available as mTOR inhibitors or surgery. Surgery is still a valid option especially for symptomatic and larger tumors. METHODS: From January 1994 to December 2015, 31 TSCpatients harboring SEGA underwent surgery at the Department of Neurosurgery of the Meyer Pediatric Hospital, Florence. Indications for surgery were tumor size and location, growth and cystization/hemorrhage, and hydrocephalus. Clinical data, preoperative and postoperative MRI, recurrence rate, further surgical procedures, and related complications were analyzed. RESULTS: A total of 44 surgeries were performed in 31 TSCpatients affected by SEGA, achieving gross total removal (GTR) and subtotal removal (STR), respectively, in 36 and 8 patients. Recurrences occurred in 11 patients; 9 of them underwent further surgical procedures and 2 were treated with mTOR pathway inhibitors. Surgical morbidity and mortality were, respectively, 22.7% and 2.3%. After a mean follow-up of 4.9 years, 90% of patients were tumor-free with good neurological status in 93.3%; twelve (40%) had a ventriculo-peritoneal shunt (VPS) for hydrocephalus. CONCLUSIONS: The present series confirms that the surgical approach, combined with mTOR inhibitors, is still a valid option for the treatment of SEGAs.
Authors: David M Feliciano; Tiffany V Lin; Nathaniel W Hartman; Christopher M Bartley; Cathryn Kubera; Lawrence Hsieh; Carlos Lafourcade; Rachel A O'Keefe; Angelique Bordey Journal: Int J Dev Neurosci Date: 2013-02-26 Impact factor: 2.457
Authors: Jonathan Roth; E Steve Roach; Ute Bartels; Sergiusz Jóźwiak; Mary Kay Koenig; Howard L Weiner; David N Franz; Henry Z Wang Journal: Pediatr Neurol Date: 2013-10-15 Impact factor: 3.372