A Comprehensive Overview of Pediatric Neoplasms at the Craniocervical Junction: Meningiomas, Schwannomas, and Chordomas

Abstract

Tumors of the craniocervical junction (CCJ) are complicated pathologies with high patient mortality or low quality of life. In the pediatric population, these tumors are less prevalent, with various symptomatic presentations that include motor and neurological manifestations. Three of the most common neoplasms at the CCJ in children are meningiomas, schwannomas, and chordomas. In this review, we will characterize the tissue biomarkers, clinical presentation, treatment methods, and surgical outcomes for these pediatric tumors at the CCJ. A comprehensive literature review was used using the PubMed Database. Keywords used were "craniocervical junction", "pediatric", "meningiomas", schwannomas", and "meningiomas". Articles that were not related to the CCJ, included only adult cases, and non-English studies were filtered. Our search yielded a total of 11 studies, with a total of 239 pediatric patients with tumors at the CCJ. These studies were broken down as five for meningiomas, one for schwannomas, and eight for chordomas. In conclusion, resection of pediatric neoplasms at the CCJ is challenging due to anatomical limitations and the size of the patient. Within the CCJ, chordomas were the most prevalent tumor type, with schwannomas being the least prevalent. Literature findings indicate that genetic mutations of the NF2 gene associated with neurofibromatosis type II, as well as incomplete tumor resection, are predictors of poor outcomes. Further developments of monoclonal antibody chemotherapy and endoscopic approaches could expand treatment options for aggressive pediatric neoplasms at the skull base.

Craniovertebral Junction Instability after Oncological Resection: A Narrative Review

“…Chordomas, meningiomas and nerve sheath tumors (schwannomas and neurofibromas) represent the most common primary neoplasms [ 33 , 34 ].…”

“…However, a score between 7 and 12, which indicates a potentially unstable lesion, is an indication for surgical consultation [ 78 ]. A lytic destruction or resection of 70% of a unilateral condyle, 50% of bilateral condyles or extensive removal of the posterior elements and facets are also suggested in the literature as indications for occipitocervical fixation (OCF) [ 34 ].…”

Deep learning–based automatic segmentation of meningioma from T1-weighted contrast-enhanced MRI for preoperative meningioma differentiation using radiomic features

“…Recently, radiomics and deep learning (DL), two main categories of machine learning (ML), have rapidly developed into a research hotspot in medical image analysis, enabling the extraction of high-throughput quantitative imaging features from medical image [ 10 – 12 ]. It captures relationships between image voxels that may not be perceived by the naked eye of physicians-even experienced radiologists, which can contribute to the diagnostic and predictive accuracy of the disease.…”