Management and outcome of pediatric brainstem and cerebellar peduncular low-grade gliomas: a retrospective analysis of 62 cases.

PURPOSE: This study was designed to present our experience and recommendations regarding the management of pediatric brainstem and peduncular low-grade gliomas (LGGs). METHODS: Retrospective analysis was performed for pathologically proven brainstem or cerebellar peduncular LGGs in patients admitted between 2014 and 2019. These lesions were classified into the dorsal exophytic, focal brainstem, cervicomedullary, lower peduncular, and upper peduncular groups, and this classification was the basis for the surgical approach for the lesions. RESULTS: Sixty-two pediatric patients were included, and their distribution among the aforementioned groups were as follows: 12, 12, 3, 16, and 19 cases in the dorsal exophytic, focal brainstem, cervicomedullary, upper peduncular, and lower peduncular groups, respectively. Stereotactic biopsy was performed for all cases in the focal brainstem group, whereas other groups underwent open excision. Gross total resection (GTR) was achieved in 20 cases (40%), near-total resection (NTR) was achieved in 17 cases (34%), and subtotal resection (STR) was achieved in 13 cases (26%). The extent of GTR and NTR for the upper peduncular, lower peduncular, dorsal exophytic, and cervicomedullary groups were 81.2%, 68.4%,75%, and 66.6%, respectively. Then, 32 cases received chemotherapy. The 3- and 5-year progression-free survival rates were 95% (95% confidence interval (CI) 89.5-100%) and 90.3% (95% CI 79.9-100%), respectively. A significant difference in the 3-year progression-free survival rate was observed between the GTR and NTR groups (p = 0.06; 100% vs. 88.2% (95% CI 72.9-100%)). CONCLUSION: Surgery plays a definitive curative role in grossly resected cases. Additionally, the role of surgical debulking should be considered, even if GTR is impossible. Meanwhile, chemotherapy showed a beneficial role in patients with focal brainstem lesions and progressive lesions, those with STR, and some patients with NTR.

Surgical outcome of children with medulloblastoma: a retrospective study of a 405-patient series from Children's Cancer Hospital Egypt (CCHE-57357).

PURPOSE: To analyze the impact of increasing the extent of resection (EOR) on the survival rates and on the surgical outcome of children with medulloblastoma. METHODS: A series of consecutive 405 children operated for medulloblastoma between July 2007 and April 2018 was identified. The details of pre-operative data, surgical interventions, post-operative complications, and survival rates were analyzed. RESULTS: The Kaplan-Meier (KM) analysis showed no advantage of gross total resection (GTR) over near and subtotal resection regarding over all (OS) (p=0.557) and progression free survival (PFS) (p=0.146). In the same time, increasing the EOR was not associated with higher morbidity. Tumor dissemination at onset correlated to worse OS (KM: p=0.003, OR 1.999, 95% CI: 1.242-3.127; p = 0.004) and PFS (KM: p<0.001, Cox: OR 2.171, 95% CI: 1.406-3.353; p<0.001). OS was significantly affected in patients < 3 years old (KM: p=0.011, OR 2.036, 95% CI: 1.229-3.374; p = 0.006), while PFS was worse among patients who had pre-op seizures (KM: p=0.036, Cox: OR 2.852, 95% CI: 1.046-7.773; p=0.041) or post-op pseudomeningocele (KM: p=0.021, Cox: OR 2.311, 95% CI: 1.123-4.754; p=0.023). CONCLUSIONS: Although surgical excision of medulloblastoma is the standard of care, there was no significant benefit for GTR over near or subtotal resection on the OS or PFS rates that are mainly influenced by the patient's age and tumor dissemination. However, GTR should be targeted, as it is not associated with increased incidence of mutism or other surgery-related complications.

Telovelar Approach to Pediatric Fourth Ventricle Tumors: Feasibility and Outcome.

AIM: To describe our institution’s experience with the telovelar approach as well as factors governing radicality and outcome for pediatric fourth ventricle tumors. MATERIAL AND METHODS: During March 2015â€"January 2017, 44 children with fourth ventricular tumors were operated using the telovelar approach. Radicality, neurological outcome, complications, and survival rates were evaluated. Statistical analysis was performed to determine factors associated with radicality and neurological outcome. RESULTS: Gross tumor removal was achieved in 37 patients (84.1%). All patients required cerebrospinal fluid (CSF) diversion through a ventriculoperitoneal shunt. Six patients (13.6%) developed cerebellar mutism, 4 patients (9.1%) developed bulbar paralysis (lower cranial nerve palsy), and 13 patients (30.2%) had tumor recurrence. Medulloblastoma was associated with the highest risk of cerebellar mutism (p=0.040). A poor prognosis (according to overall and progression-free survival rates) was associated with subtotal resection (p=0.020), large cell/anaplastic medulloblastoma and anaplastic ependymoma (p=0.038), highrisk medulloblastoma (p=0.005), and CSF seeding (p < 0.001). CONCLUSION: The telovelar approach provides an adequate anatomical exposure of the fourth ventricle and allows early visualization and protection of its floor (brainstem). It facilitates adequate radicality and is associated with a lower incidence of cerebellar mutism and other approach-related complications. The working angle, particularly to the rostral part of the fourth ventricle, can be increased by removing the posterior arch of the atlas.