Neurosurgery Department, Kasr Al-Ainy School of Medicine, Cairo University, Cairo, Egypt.
Neurosurgery Department, Children's Cancer Hospital Egypt (CCHE, 57357), Cairo, Egypt.
Childs Nerv Syst. 2022 Mar;38(3):565-575. doi: 10.1007/s00381-021-05405-3. Epub 2021 Nov 17.
This study was designed to present our experience and recommendations regarding the management of pediatric brainstem and peduncular low-grade gliomas (LGGs).
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.
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%)).
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.
本研究旨在介绍我们在儿童脑干和桥脑脚低级别胶质瘤（LGG）治疗方面的经验和建议。
回顾性分析了 2014 年至 2019 年间收治的经病理证实的脑干或小脑桥脑脚 LGG 患者。这些病变分为背外侧外生型、局灶性脑干型、颈髓型、下桥脑脚型和上桥脑脚型，并以此为病变的手术入路依据。
共纳入 62 例儿科患者，其在上述各组中的分布如下：背外侧外生型 12 例、局灶性脑干型 12 例、颈髓型 3 例、上桥脑脚型 16 例和下桥脑脚型 19 例。局灶性脑干组所有病例均行立体定向活检，其他组行开放性切除。20 例（40%）实现大体全切除（GTR），17 例（34%）实现近全切除（NTR），13 例（26%）实现次全切除（STR）。上桥脑脚、下桥脑脚、背外侧外生型和颈髓型的 GTR 和 NTR 程度分别为 81.2%、68.4%、75%和 66.6%。随后，32 例接受了化疗。3 年和 5 年无进展生存率分别为 95%（95%可信区间（CI）89.5-100%）和 90.3%（95% CI 79.9-100%）。GTR 组与 NTR 组 3 年无进展生存率有显著差异（p=0.06；100%比 88.2%（95% CI 72.9-100%））。
手术在大体切除病例中具有明确的治愈作用。此外，即使无法实现 GTR，也应考虑手术去瘤的作用。同时，化疗对局灶性脑干病变和进展性病变、STR 患者和部分 NTR 患者具有有益作用。
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