Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature

Objectives: Pediatric intracranial tumors, particularly at the skull base, are rare and present unique challenges to pediatric neurosurgeons and oncologists, owing to their complex anatomy and diverse histopathology. Robust evidence is still marginal concerning their clinical and surgical courses. O...

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Main Authors: M. Grutza, P. Lenga, M. Issa, A. Seitz, F. Sahm, T. Milde, A.W. Unterberg, S.M. Krieg, A. El Damaty
Format: Article
Language:English
Published: Elsevier 2024-01-01
Series:Brain and Spine
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Online Access:http://www.sciencedirect.com/science/article/pii/S2772529424013924
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author M. Grutza
P. Lenga
M. Issa
A. Seitz
F. Sahm
T. Milde
A.W. Unterberg
S.M. Krieg
A. El Damaty
author_facet M. Grutza
P. Lenga
M. Issa
A. Seitz
F. Sahm
T. Milde
A.W. Unterberg
S.M. Krieg
A. El Damaty
author_sort M. Grutza
collection DOAJ
description Objectives: Pediatric intracranial tumors, particularly at the skull base, are rare and present unique challenges to pediatric neurosurgeons and oncologists, owing to their complex anatomy and diverse histopathology. Robust evidence is still marginal concerning their clinical and surgical courses. Our aim is to describe our experience regarding surgical approaches, with special focus on surgical features, postoperative outcomes, adverse events as well as adjuvant therapeutic concepts. Methods: Patients aged <18 years undergoing skull base surgery between 2017 and 2023 at our institution were retrospectively enrolled. Patient demographics, tumor characteristics, surgical approach, pre -and postoperative clinical status and adjuvant therapy as well as overall and progression free survival were assessed. Results: Twelve children aged 6.1 ± 4.1 years were analyzed. There was a predominance of the female gender (7/12, 58.4%). Mean tumor diameter was 4.0 ± 2.9 cm. In three children the tumor was located suprasellar, temporobasal in one, adjacent to the cerebellar pontine angle in 4, clival in 3 and petroclival in 2 children. A subfrontal approach was performed in two patients, a subtemporal approach in one, a retrosigmoidal approach in 5 and in one patient two-staged approach; retrosigmoidal and later in a second operation pterional approach was conducted. One clival tumor was removed via an endonasal endoscopic approach and in another case via a transoral endoscopic approach. Gross total resection (GTR) and near total resection were achieved in 7 patients (58.3%). Tumor types included meningioma, clivus-chordoma, epidermoid cyst, anaplastic ependymoma, Ewing's sarcoma and Atypical Teratoid Rhabdoid Tumor (ATRT) as well as embryonal tumor with multilayered rosettes (ETMR). 4 patients (33.3%) died of disease due to tumor progression in average after 15 months. Hydrocephalus developed in two patients; a ventriculoperitoneal shunt was inserted in one patient, while an endoscopic third ventriculostomy (ETV) was performed in the other. Moreover, one child suffered from a residual neurological deficit at last follow-up evaluation. Adjuvant therapy protocols were applied in six patients (50.0%). Conclusion: Skull base tumors in children present a therapeutic challenge due to their rarity and unique pathological composition and can lead to considerable morbidity and mortality. An interdisciplinary approach involving neurosurgeons, pediatric oncologists and radiotherapists is mandatory to guarantee the best clinical course.
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spelling doaj-art-ca9153e2ab0048e5847dba7d049fb6e42025-08-20T02:37:53ZengElsevierBrain and Spine2772-52942024-01-01410413610.1016/j.bas.2024.104136Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literatureM. Grutza0P. Lenga1M. Issa2A. Seitz3F. Sahm4T. Milde5A.W. Unterberg6S.M. Krieg7A. El Damaty8Department of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany; Medical Faculty of Heidelberg University, Heidelberg, Germany; Corresponding author. Department of Neurosurgery Heidelberg University Hospital Im Neuenheimer Feld 400, 69120, Heidelberg, Germany.Department of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany; Medical Faculty of Heidelberg University, Heidelberg, GermanyDepartment of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany; Medical Faculty of Heidelberg University, Heidelberg, GermanyDepartment of Neuroradiology, Heidelberg University Hospital, Heidelberg, GermanyDepartment of Neuropathology, Institute of Pathology, Heidelberg University Hospital, Heidelberg, Germany; Hopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany; Clinical Cooperation Unit Pediatric Oncology, German Cancer Research Center (DKFZ) and German Consortium for Translational Cancer Research (DKTK), Heidelberg, GermanyHopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany; Clinical Cooperation Unit Pediatric Oncology, German Cancer Research Center (DKFZ) and German Consortium for Translational Cancer Research (DKTK), Heidelberg, Germany; National Center for Tumor Diseases (NCT), Heidelberg, Germany; University Hospital Jena, Department of Pediatrics and Adolescent Medicine, Friedrich Schiller University Jena, Jena, GermanyDepartment of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany; Medical Faculty of Heidelberg University, Heidelberg, GermanyDepartment of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany; Medical Faculty of Heidelberg University, Heidelberg, GermanyDepartment of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany; Medical Faculty of Heidelberg University, Heidelberg, GermanyObjectives: Pediatric intracranial tumors, particularly at the skull base, are rare and present unique challenges to pediatric neurosurgeons and oncologists, owing to their complex anatomy and diverse histopathology. Robust evidence is still marginal concerning their clinical and surgical courses. Our aim is to describe our experience regarding surgical approaches, with special focus on surgical features, postoperative outcomes, adverse events as well as adjuvant therapeutic concepts. Methods: Patients aged <18 years undergoing skull base surgery between 2017 and 2023 at our institution were retrospectively enrolled. Patient demographics, tumor characteristics, surgical approach, pre -and postoperative clinical status and adjuvant therapy as well as overall and progression free survival were assessed. Results: Twelve children aged 6.1 ± 4.1 years were analyzed. There was a predominance of the female gender (7/12, 58.4%). Mean tumor diameter was 4.0 ± 2.9 cm. In three children the tumor was located suprasellar, temporobasal in one, adjacent to the cerebellar pontine angle in 4, clival in 3 and petroclival in 2 children. A subfrontal approach was performed in two patients, a subtemporal approach in one, a retrosigmoidal approach in 5 and in one patient two-staged approach; retrosigmoidal and later in a second operation pterional approach was conducted. One clival tumor was removed via an endonasal endoscopic approach and in another case via a transoral endoscopic approach. Gross total resection (GTR) and near total resection were achieved in 7 patients (58.3%). Tumor types included meningioma, clivus-chordoma, epidermoid cyst, anaplastic ependymoma, Ewing's sarcoma and Atypical Teratoid Rhabdoid Tumor (ATRT) as well as embryonal tumor with multilayered rosettes (ETMR). 4 patients (33.3%) died of disease due to tumor progression in average after 15 months. Hydrocephalus developed in two patients; a ventriculoperitoneal shunt was inserted in one patient, while an endoscopic third ventriculostomy (ETV) was performed in the other. Moreover, one child suffered from a residual neurological deficit at last follow-up evaluation. Adjuvant therapy protocols were applied in six patients (50.0%). Conclusion: Skull base tumors in children present a therapeutic challenge due to their rarity and unique pathological composition and can lead to considerable morbidity and mortality. An interdisciplinary approach involving neurosurgeons, pediatric oncologists and radiotherapists is mandatory to guarantee the best clinical course.http://www.sciencedirect.com/science/article/pii/S2772529424013924Skull base tumorsPediatric populationPediatric neurooncology
spellingShingle M. Grutza
P. Lenga
M. Issa
A. Seitz
F. Sahm
T. Milde
A.W. Unterberg
S.M. Krieg
A. El Damaty
Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature
Brain and Spine
Skull base tumors
Pediatric population
Pediatric neurooncology
title Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature
title_full Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature
title_fullStr Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature
title_full_unstemmed Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature
title_short Pediatric skull base tumors: Clinical features and surgical outcomes; a single center retrospective study with a review of literature
title_sort pediatric skull base tumors clinical features and surgical outcomes a single center retrospective study with a review of literature
topic Skull base tumors
Pediatric population
Pediatric neurooncology
url http://www.sciencedirect.com/science/article/pii/S2772529424013924
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