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Neurosurgical treatment of subependymal giant cell astrocytomas in tuberous sclerosis complex: a series of 44 surgical procedures in 31 patients

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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.

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Authors and Affiliations

  1. Department of Neurosurgery, Children’s Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy

    Flavio Giordano, Matteo Lenge & Lorenzo Genitori

  2. Neuro-oncology Unit, Children’s Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy

    Carla Moscheo & Iacopo Sardi

  3. 3. Pediatric Neurology, Children’s Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy

    Matteo Lenge, Francesco Mari & Renzo Guerrini

  4. Division of Prenatal Diagnosis, Children’s Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy

    Roberto Biagiotti

  5. Pathology Unit, Children’s Hospital A. Meyer, University of Florence, viale Pieraccini 24, Florence, 50139, Italy

    Anna Maria Buccoliero

  6. Neurosurgery, Sant’Anna Hospital, Via Aldo Moro, Ferrara, 44124, Italy

    Lorenzo Mongardi

  7. Department of (Neuro) Pathology, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands

    Eleonora Aronica

  8. Stichting Epilepsie Instellingen Nederland (SEIN), Heemstede, The Netherlands

    Eleonora Aronica

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  1. Flavio Giordano
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Correspondence to Flavio Giordano.

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Giordano, F., Moscheo, C., Lenge, M. et al. Neurosurgical treatment of subependymal giant cell astrocytomas in tuberous sclerosis complex: a series of 44 surgical procedures in 31 patients. Childs Nerv Syst 36, 951–960 (2020). https://doi.org/10.1007/s00381-019-04449-w

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