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15 pages, 3680 KiB

Open AccessArticle

Neurosurgical Treatment and Outcome of Pediatric Skull Base Lesions: A Case Series and Review of the Literature

by Ladina Greuter, Tim Hallenberger, Raphael Guzman and Jehuda Soleman

Children 2023, 10(2), 216; https://doi.org/10.3390/children10020216 - 26 Jan 2023

Cited by 1 | Viewed by 1889

Abstract

Introduction: Pediatric skull base lesions occur rarely and are of various etiologies. Traditionally, open craniotomy has been the treatment of choice; however, nowadays, endoscopic approaches are increasingly applied. In this retrospective case series, we describe our experience in treating pediatric skull base lesions [...] Read more.

Introduction: Pediatric skull base lesions occur rarely and are of various etiologies. Traditionally, open craniotomy has been the treatment of choice; however, nowadays, endoscopic approaches are increasingly applied. In this retrospective case series, we describe our experience in treating pediatric skull base lesions and provide a systematic overview of the literature on the treatment and outcome of pediatric skull base lesions. Methods: We conducted a retrospective data collection of all pediatric patients (<18 years) treated for a skull base lesion at the Division of Pediatric Neurosurgery, University Children’s Hospital Basel, Switzerland, between 2015 and 2021. Descriptive statistics and a systematic review of the available literature were additionally conducted. Results: We included 17 patients with a mean age of 8.92 (±5.76) years and nine males (52.9%). The most common entity was sellar pathologies (n = 8 47.1%), with craniopharyngioma being the most common pathology (n = 4, 23.5%). Endoscopic approaches, either endonasal transsphenoidal or transventricular, were used in nine (52.9%) cases. Six patients (35.3%) suffered from transient postoperative complications, while in none of the patients these were permanent. Of the nine (52.9%) patients with preoperative deficits, two (11.8%) showed complete recovery and one (5.9%) partial recovery after surgery. After screening 363 articles, we included 16 studies with a total of 807 patients for the systematic review. The most common pathology reported in the literature confirmed our finding of craniopharyngioma (n = 142, 18.0%). The mean PFS amongst all the studies included was 37.73 (95% CI [36.2, 39.2]) months, and the overall weighted complication rate was 40% (95% CI [0.28 to 0.53] with a permanent complication rate of 15% (95% CI [0.08 to 0.27]. Only one study reported an overall survival of their cohort of 68% at five years. Conclusion: This study highlights the rarity and heterogeneity of skull base lesions in the pediatric population. While these pathologies are often benign, achieving GTR is challenging due to the deep localization of the lesions and eloquent adjacent structures, leading to high complication rates. Therefore, skull base lesions in children require an experienced multidisciplinary team to provide optimal care. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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11 pages, 836 KiB

Open AccessArticle

Management of Sporadic Vestibular Schwannomas in Children—Volumetric Analysis and Clinical Outcome Assessment

by Julian Zipfel, Mykola Gorbachuk, Isabel Gugel, Marcos Tatagiba and Martin U. Schuhmann

Children 2022, 9(4), 490; https://doi.org/10.3390/children9040490 - 1 Apr 2022

Cited by 3 | Viewed by 2466

Abstract

Vestibular schwannomas (VS) usually manifest between the 5th and 8th decade of life. Most pediatric cases are associated with Neurofibromatosis type 2 and sporadic VS are rare in this age group. Few case series have been published. We report on our institutional series [...] Read more.

Vestibular schwannomas (VS) usually manifest between the 5th and 8th decade of life. Most pediatric cases are associated with Neurofibromatosis type 2 and sporadic VS are rare in this age group. Few case series have been published. We report on our institutional series of sporadic VS in children. We included all cases between 2003 and 2021; 28 of 1635 patients harbored a sporadic VS and were younger than 21 years old. A retrospective review of clinical parameters and surgical data as well as outcomes was performed. All procedures were performed via a retrosigmoid approach. Preoperative imaging was assessed, and tumor volumetry was performed. Mean follow-up was 28 months, symptomatology was diverse. Most children and adolescents presented with hearing loss and tinnitus. All cases with multiple preoperative magnetic resonance imaging scans showed volumetric tumor growth between 1 and 18%/month (mean 8.9 ± 5.6%). Cystic tumor morphology and bone erosion was seen in larger tumors. Gross total resection was possible in 78% of patients and no recurrence was observed. All patients with subtotal resection showed tumor regrowth. Sporadic VS in children are rare and present with a high clinical variability. Surgical resection is the primary therapy and is feasible with favorable results comparable to the adult age group. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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13 pages, 3083 KiB

Open AccessArticle

Surgical Management of Pre-Chiasmatic Intraorbital Optic Nerve Gliomas in Children after Loss of Visual Function—Resection from Bulbus to Chiasm

by Julian Zipfel, Jonas Tellermann, Dorothea Besch, Eckart Bertelmann, Martin Ebinger, Pablo Hernáiz Driever, Jens Schittenhelm, Rudi Beschorner, Arend Koch, Ulrich-Wilhelm Thomale and Martin Ulrich Schuhmann

Children 2022, 9(4), 459; https://doi.org/10.3390/children9040459 - 24 Mar 2022

Cited by 7 | Viewed by 3789

Abstract

Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the subgroup of pre-chiasmatic gliomas, intraorbital and intradural resection is a curative option after blindness. We present a two-center cohort using different surgical approaches. A retrospective analysis was performed, including 10 [...] Read more.

Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the subgroup of pre-chiasmatic gliomas, intraorbital and intradural resection is a curative option after blindness. We present a two-center cohort using different surgical approaches. A retrospective analysis was performed, including 10 children. Mean age at surgery was 6.8 years. Interval between diagnosis and surgery was 1–74 (mean 24 ± 5.5, median 10) months. Indications for surgery were exophthalmos, pain, tumor progression, or a combination. Eight patients underwent an extradural trans-orbital-roof approach to resect the intra-orbital tumor, including the optic canal part plus intradural pre-chiasmatic resection. Gross total resection was achieved in 7/8, and none had a recurrence. One residual behind the bulbus showed progression, treated by chemotherapy. In two patients, a combined supra-orbital mini-craniotomy plus orbital frame osteotomy was used for intraorbital tumor resection + intradural pre-chiasmatic dissection. In these two patients, remnants of the optic nerve within the optic canal remained stable. No patient had a chiasmatic functional affection nor permanent oculomotor deficits. In selected patients, a surgical resection from bulb to chiasm ± removal of optic canal tumor was safe without long-term sequela and with an excellent cosmetic result. Surgery normalizes exophthalmos and provides an effective tumor control. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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11 pages, 1258 KiB

Open AccessArticle

Educational Attainment and Employment Outcome of Survivors of Pediatric CNS Tumors in Switzerland—A Report from the Swiss Childhood Cancer Survivor Study

by Maria Otth, Gisela Michel, Nicolas U. Gerber, Ana S. Guerreiro Stücklin, André O. von Bueren, Katrin Scheinemann and on behalf of the Swiss Pediatric Oncology Group (SPOG)

Children 2022, 9(3), 411; https://doi.org/10.3390/children9030411 - 14 Mar 2022

Cited by 5 | Viewed by 2532

Abstract

Background: Childhood cancer survivors diagnosed with a central nervous system (CNS) tumor are at risk for educational and vocational challenges. This study compared educational attainment and employment outcome in survivors of CNS tumors to survivors of other malignancies. Methods: The questionnaire-based Swiss Childhood [...] Read more.

Background: Childhood cancer survivors diagnosed with a central nervous system (CNS) tumor are at risk for educational and vocational challenges. This study compared educational attainment and employment outcome in survivors of CNS tumors to survivors of other malignancies. Methods: The questionnaire-based Swiss Childhood Cancer Survivor Study (SCCSS) included cancer patients diagnosed between 1976 and 2010, aged ≤20 years, who survived ≥5 years after diagnosis. We classified participants aged ≥16 years into three groups: CNS tumor and non-CNS malignancy with and without CNS-directed treatment. We analyzed educational attainment, employment outcome and special schooling. Subgroup analyses included survivors aged ≥25 years. Results: We analyzed 2154 survivors, including 329 (15%) CNS tumor survivors, 850 (40%) non-CNS tumor survivors with and 975 (45%) without CNS-directed treatment. Fewer CNS tumor survivors aged ≥25 years reached tertiary education (44%) compared to those without CNS-directed treatment (51%) but performed similar to survivors with CNS-directed treatment (42%). Among CNS tumor survivors, 36 (14%) received special schooling. Higher parental education was associated with higher levels in survivors. Employment outcome did not significantly differ between the three diagnostic groups. A higher proportion of CNS tumor survivors received disability pension or were unemployed. Conclusions: Our findings suggest that CNS tumor survivors need more time to achieve their highest educational level. This should influence clinical care of these survivors by offering vocational counseling. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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Review

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15 pages, 306 KiB

Open AccessReview

Advances in the Treatment of Pediatric Brain Tumors

by Edwin S. Kulubya, Matthew J. Kercher, H. Westley Phillips, Reuben Antony and Michael S. B. Edwards

Children 2023, 10(1), 62; https://doi.org/10.3390/children10010062 - 27 Dec 2022

Cited by 11 | Viewed by 3706

Abstract

Pediatric brain tumors are the most common solid malignancies in children. Advances in the treatment of pediatric brain tumors have come in the form of imaging, biopsy, surgical techniques, and molecular profiling. This has led the way for targeted therapies and immunotherapy to [...] Read more.

Pediatric brain tumors are the most common solid malignancies in children. Advances in the treatment of pediatric brain tumors have come in the form of imaging, biopsy, surgical techniques, and molecular profiling. This has led the way for targeted therapies and immunotherapy to be assessed in clinical trials for the most common types of pediatric brain tumors. Here we review the latest efforts and challenges in targeted molecular therapy, immunotherapy, and newer modalities such as laser interstitial thermal therapy. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

16 pages, 364 KiB

Open AccessReview

Endocrine Disorders in Children with Brain Tumors: At Diagnosis, after Surgery, Radiotherapy and Chemotherapy

by Fabien Claude, Graziamaria Ubertini and Gabor Szinnai

Children 2022, 9(11), 1617; https://doi.org/10.3390/children9111617 - 25 Oct 2022

Cited by 7 | Viewed by 2574

Abstract

Introduction: Brain tumors are the second most frequent type of all pediatric malignancies. Depending on their localization, patients with brain tumors may present neurological or ophthalmological symptoms, but also weight anomalies and endocrine disorders ranging from growth hormone deficiency, anomalies of puberty, diabetes [...] Read more.

Introduction: Brain tumors are the second most frequent type of all pediatric malignancies. Depending on their localization, patients with brain tumors may present neurological or ophthalmological symptoms, but also weight anomalies and endocrine disorders ranging from growth hormone deficiency, anomalies of puberty, diabetes insipidus to panhypopituitarism. Immediately at diagnosis, all patients with brain tumors require a complete assessment of the hypothalamic–pituitary function in order to address eventual endocrine disorders. Moreover, children and adolescents undergoing brain surgery must receive peri- and postoperative hydrocortisone stress therapy. Post-operative disorders of water homeostasis are frequent, ranging from transient diabetes insipidus, as well as syndrome of inappropriate antidiuretic hormone secretion to persistent diabetes insipidus. Late endocrine disorders may result from surgery near or within the hypothalamic–pituitary region. Pituitary deficits are frequent after radiotherapy, especially growth hormone deficiency. Thyroid nodules or secondary thyroid cancers may arise years after radiotherapy. Gonadal dysfunction is frequent after chemotherapy especially with alkylating agents. Conclusion: Early detection and treatment of specific endocrine disorders at diagnosis, perioperatively, and during long-term follow-up result in improved general and metabolic health and quality of life. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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10 pages, 1558 KiB

Open AccessReview

Special Anaesthetic Considerations for Brain Tumour Surgery in Children

by Sandra Jeker, Maria Julia Beck and Thomas O. Erb

Children 2022, 9(10), 1539; https://doi.org/10.3390/children9101539 - 9 Oct 2022

Viewed by 2277

Abstract

Brain tumours are among the most common neoplasm in children. Therefore, paediatric anaesthesiologists face the challenge of neurosurgical interventions in all age groups. To minimize perioperative mortality and morbidity, a comprehensive understanding of age-dependent differences in anatomy and cerebrovascular physiology is a mandatory [...] Read more.

Brain tumours are among the most common neoplasm in children. Therefore, paediatric anaesthesiologists face the challenge of neurosurgical interventions in all age groups. To minimize perioperative mortality and morbidity, a comprehensive understanding of age-dependent differences in anatomy and cerebrovascular physiology is a mandatory prerequisite. Advances in subspeciality training in paediatric neurosurgery and paediatric anaesthesia may improve clinical outcomes and advance communication between the teams. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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14 pages, 317 KiB

Open AccessReview

Supratentorial Pediatric Midline Tumors and Tumor-like Lesions: Clinical Spectrum, Natural History and Treatment Options

by Luca Paun, Alexandre Lavé, Gildas Patet and Andrea Bartoli

Children 2022, 9(4), 534; https://doi.org/10.3390/children9040534 - 9 Apr 2022

Cited by 2 | Viewed by 2135

Abstract

Childhood Central Nervous System tumors account for 25% of all pediatric tumors. Large availability and broadening of indications to imaging has made incidental findings more common. Among these, midline lesions have different clinical relevance depending on their intrinsic pattern of behaviour and on [...] Read more.

Childhood Central Nervous System tumors account for 25% of all pediatric tumors. Large availability and broadening of indications to imaging has made incidental findings more common. Among these, midline lesions have different clinical relevance depending on their intrinsic pattern of behaviour and on their specific location. In this narrative review we describe the natural history and treatment options of midline lesions in children. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

26 pages, 1627 KiB

Open AccessEditor’s ChoiceReview

Essential Management of Pediatric Brain Tumors

by Katharina Lutz, Stephanie T. Jünger and Martina Messing-Jünger

Children 2022, 9(4), 498; https://doi.org/10.3390/children9040498 - 2 Apr 2022

Cited by 15 | Viewed by 6057

Abstract

Brain tumors are the most common solid tumors in children and are associated with high mortality. The most common childhood brain tumors are grouped as low-grade gliomas (LGG), high grade gliomas (HGG), ependymomas, and embryonal tumors, according to the World Health Organization (WHO). [...] Read more.

Brain tumors are the most common solid tumors in children and are associated with high mortality. The most common childhood brain tumors are grouped as low-grade gliomas (LGG), high grade gliomas (HGG), ependymomas, and embryonal tumors, according to the World Health Organization (WHO). Advances in molecular genetics have led to a shift from pure histopathological diagnosis to integrated diagnosis. For the first time, these new criteria were included in the WHO classification published in 2016 and has been further updated in the 2021 edition. Integrated diagnosis is based on molecular genomic similarities of the tumor subclasses, and it can better explain the differences in clinical courses of previously histopathologically identical entities. Important advances have also been made in pediatric neuro-oncology. A growing understanding of the molecular-genetic background of tumorigenesis has improved the diagnostic accuracy. Re-stratification of treatment protocols and the development of targeted therapies will significantly affect overall survival and quality of life. For some pediatric tumors, these advances have significantly improved therapeutic management and prognosis in certain tumor subgroups. Some therapeutic approaches also have serious long-term consequences. Therefore, optimized treatments are greatly needed. Here, we discuss the importance of multidisciplinary collaboration and the role of (pediatric) neurosurgery by briefly describing the most common childhood brain tumors and their currently recognized molecular subgroups. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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14 pages, 317 KiB

Open AccessReview

Long-Term Follow-Up of Pediatric CNS Tumor Survivors—A Selection of Relevant Long-Term Issues

by Maria Otth, Johanna Wyss and Katrin Scheinemann

Children 2022, 9(4), 447; https://doi.org/10.3390/children9040447 - 22 Mar 2022

Cited by 7 | Viewed by 2948

Abstract

Introduction: Survivors of pediatric central nervous system (CNS) tumors are at high risk for late effects and long-term morbidity. The quality of survival became increasingly important, as advances in diagnostics, multimodal treatment strategies, and supportive care have led to significant increases in long-term [...] Read more.

Introduction: Survivors of pediatric central nervous system (CNS) tumors are at high risk for late effects and long-term morbidity. The quality of survival became increasingly important, as advances in diagnostics, multimodal treatment strategies, and supportive care have led to significant increases in long-term survival. Aim: This review aims to provide a global overview of the potential late effects and long-term follow-up care of CNS tumor survivors, directed to trainees and practitioners with less targeted training in pediatric oncology. Late effects in CNS tumor survivors: A specific focus on CNS tumor survivors relies on cognitive and psychosocial late effects, as they may have an impact on education, professional career, independent living, and quality of life. Further important late effects in CNS tumor survivors include endocrine, metabolic, cardiovascular, and cerebrovascular diseases. Conclusions: Comprehensive long-term follow-up care is essential for pediatric CNS tumor survivors to improve their quality of survival and quality of life. An individualized approach, taking all potential late effects into account, and carried out by an interdisciplinary team, is recommended, and should continue into adulthood. Existing recommendations and guidelines on long-term follow-up care guide the multidisciplinary teams. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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9 pages, 2625 KiB

Open AccessTechnical Note

Endoscope-Assisted Extreme Lateral Supracerebellar Infratentorial Approach for Resection of Superior Cerebellar Peduncle Pilocytic Astrocytoma: Technical Note

by Kyriakos Papadimitriou, Giulia Cossu, Ekkehard Hewer, Manuel Diezi, Roy Thomas Daniel and Mahmoud Messerer

Children 2022, 9(5), 640; https://doi.org/10.3390/children9050640 - 29 Apr 2022

Cited by 1 | Viewed by 2610

Abstract

(1) Background: Superior cerebellar peduncle (SCP) lesions are sparsely reported in the literature. The surgical approaches to the cerebello-mesencephalic region remain challenging. In this article, we present the extreme lateral supracerebellar infratentorial (ELSI) approach to treat a large hemorrhagic pilocytic astrocytoma of the [...] Read more.

(1) Background: Superior cerebellar peduncle (SCP) lesions are sparsely reported in the literature. The surgical approaches to the cerebello-mesencephalic region remain challenging. In this article, we present the extreme lateral supracerebellar infratentorial (ELSI) approach to treat a large hemorrhagic pilocytic astrocytoma of the SCP. (2) Methods: An 11-year-old boy, known for neurofibromatosis Type I, presented to the emergency department of our institution with symptoms and signs of intracranial hypertension. The cerebral magnetic resonance imaging (MRI) revealed a large hemorrhagic lesion centered on the SCP provoking obstructive hydrocephalus. Following an emergency endoscopic third ventriculocisternostomy (ETV), he underwent a tumor resection via an endoscope-assisted ELSI approach. (3) Results: ELSI approach allows for a wide exposure with direct access to lesions of the SCP. The post-operative course was uneventful, and the patient was discharged home on post-operative day 5. Post-operative MRI revealed a near total resection with a small residual tumor within the mesencephalon. (4) Conclusion: ELSI approach offers an excellent exposure with the surgical angles necessary for median and paramedian lesions. The park-bench position with appropriate head flexion and rotation offers a gravity-assisted relaxation of the tentorial and petrosal cerebellar surfaces. The endoscope can be an adjunct to illuminate the blind areas of the surgical corridor for an improved tumor resection without significant cerebellar retraction. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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11 pages, 426 KiB

Open AccessSystematic Review

Transitional Care in Pediatric Brain Tumor Patients: A Systematic Literature Review

by Florian Ebel, Ladina Greuter, Raphael Guzman and Jehuda Soleman

Children 2022, 9(4), 501; https://doi.org/10.3390/children9040501 - 2 Apr 2022

Cited by 8 | Viewed by 2156

Abstract

Background: Due to advances in the treatment of pediatric brain tumors (PBT), an increasing number of patients are experiencing the transition from the pediatric to the adult health care system. This requires efficient transitional models. Methods: We systematically reviewed the literature regarding PBT [...] Read more.

Background: Due to advances in the treatment of pediatric brain tumors (PBT), an increasing number of patients are experiencing the transition from the pediatric to the adult health care system. This requires efficient transitional models. Methods: We systematically reviewed the literature regarding PBT concerning different transitional models and aspects of the transitional period. For this purpose, PubMed, Medline, and Embase databases were searched systematically through January 2022. Results: We reviewed a total of 304 studies, of which 15 were ultimately included. We identified five transition models described within the literature, while the most frequently mentioned ones were the “adult caregiver model” (45.5%), “joint caregiver model” (45.5%), “continued caregiver model” (27.3%), and the “specialized clinic model” (27.3%). During the transition, the most frequent challenges mentioned by the patients were the lack of knowledge about the disease by the adult health care professionals (62.5%) and the difficulty of establishing a new relationship with the new physician, environment, or hospital (37.5%). Conclusions: An efficient transitional model is mandatory for patients with PBT. Continuity in the treatment and care of the patient and their family is essential. For this purpose, in patients with PBT, the “continued caregiver model”, and for NF1 and TSC patients, the “specialized clinic model” seems optimal to offer continuity of care. If such models are unavailable, efficient communication with patients, families, and specialists in a multidisciplinary network is even more critical. Full article

(This article belongs to the Special Issue Management of Brain Tumors in Children)

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