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BACKGROUND: Brainstem glioma is the leading cause of morbidity and mortality among all central nervous system tumors, especially in childhood as it represents about 20% of all pediatric brain tumors. Therefore, this study aimed to present our experience in a tertiary center in a developing country with limited resources for the surgical management of exophytic brainstem gliomas. METHODS: This retrospective study included pediatric patients with brainstem (midbrain, pontine, or medullary) focal or diffuse gliomas whether low or high grade that had dorsal, ventral, or lateral exophytic component who were presented to our hospitals from January 2019 to January 2021. The patients' data were collected, such as age, sex, preoperative and postoperative clinical condition, radiological data, surgical approach, extent of tumor removal, histopathology, follow-up period, and adjuvant therapy. RESULTS: A total of 23 patients were included in this study. The telovelar approach was used in 17 patients, the supracerebellar infratentorial approach in three patients, and the retrosigmoid, transcerebellar, and occipital transtentorial approach once for each patient. Twenty patients underwent near-total excision, and three underwent subtotal excision. Two-thirds of our cases (17 patients) were low-grade gliomas, with the remaining one-third comprising entirely of either anaplastic astrocytoma (five patients) or glioblastoma multiforme (one patient). The follow-up period of the patients extended from 3 months to 24 months. CONCLUSION: Exophytic brainstem glioma surgery can result in good outcomes with minimal complications when near-total excision is attempted through a properly chosen approach and adherence to some surgical techniques and considerations.
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INTRODUCTION: Optic pathway gliomas (OPGs) are rare neoplasms in children with an unpredictable clinical course. There is significant controversy regarding the optimal management and outcome of these patients. METHODS: Charts of all patients with OPG diagnosed and treated at Children's Cancer Hospital Egypt between July 2007 and July 2014 were retrospectively reviewed. We evaluated the roles of surgical, ophthalmologic, endocrinologic, neurologic, and treatment aspects of care. RESULTS: Sixty-five patients were included in this study, with a mean age of 5.3 years. OPGs were chiasmatic (n = 25), optic nerve (n = 18), hypothalamic (n = 7), and chiasmatic/hypothalamic (n = 7). Extensive involvement of the optic pathway was seen in an additional 8 patients. Twenty cases had neurofibromatosis type 1. Four cases underwent surgical debulking, and 28 were biopsied (16 open, 11 stereotactic, and 1 endoscopic). Nine of the 18 optic nerve tumors were managed by total excision. Twenty-four patients did not undergo any surgical intervention. Forty-five patients received chemotherapy. Histopathology revealed pilocytic (n = 20), pilomyxoid (n = 15), fibrillary astrocytoma (n = 4), and grade I papillary-glioneuronal tumor (n = 1). Nonrepresentative sample (n = 1). The 4-year overall survival rate was 86.3% with mean follow-up period of 32.2 months. CONCLUSION: The initial role of surgery in newly developed OPG is biopsy for tissue diagnosis and relief of the hydrocephalus, if present, followed by chemotherapy. Chemotherapy decreases or stabilizes the tumor size in most cases, leading to preservation of both visual and endocrinal functions. The most significant prognostic factor confirmed in this study was the age of the patient.
