Massimino Maura, Solero Carlo Lazzaro, Garrè Maria Luisa, Biassoni Veronica, Cama Armando, Genitori Lorenzo, Di Rocco Concezio, Sardi Iacopo, Viscardi Elisabetta, Modena Piergiorgio, Potepan Paolo, Barra Salvina, Scarzello Giovanni, Galassi Ercole, Giangaspero Felice, Antonelli Manila, Gandola Lorenza
Pediatric Unit, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Nazionale Tumori, Milano, Italy.
J Neurosurg Pediatr. 2011 Sep;8(3):246-50. doi: 10.3171/2011.6.PEDS1142.
Complete ependymoma resection ensures a better prognosis for children with this tumor, but the complete excision of infratentorial ependymomas involves serious risks. Second-look surgery for tumor remnants may be less harmful and enable complete removal. There is a potential, although still unclear, role for neoadjuvant chemotherapy in preparation for further surgery.
Since 1994, the authors have adopted two successive protocols for intracranial ependymoma, both including a phase of adjuvant chemotherapy for children with surgical tumor remnants with a plan for potential second-look surgery before radiotherapy.
In the first protocol, 9 of 63 children underwent further surgery, and 6 became tumor free with no additional sequelae. Their prognosis for progression-free survival and freedom from local relapse was comparable to that of children who were operated on only once. In the second protocol, efforts were made to achieve complete resection and 29 of 110 patients underwent reoperations: 9 after the first surgery, 17 after chemotherapy, and 3 soon after radiotherapy. Fourteen of the 29 patients became tumor free, 1 of them with worsening neurological symptoms. The outcome of the 66 patients who became tumor free after 1 operation was compared with that of the 14 who became tumor free after reoperation. The 3-year progression-free survival of the 66 patients compared with the 14 other patients was 71.4% ± 6.9% and 90% ± 9.5%, respectively; the 3-year freedom from local relapse was 84.7% ± 5.9% and 90% ± 9.5%, respectively; and the 3-year overall survival was 85.9% ± 5.4% and 87.5% ± 11.7%, respectively.
Second-look surgery proved feasible with no major morbidity, and results improved with time. Local tumor control was comparable in patients undergoing 1 or more resections.
完全切除室管膜瘤可确保患此肿瘤的儿童有更好的预后，但幕下室管膜瘤的完全切除涉及严重风险。对肿瘤残留进行二次手术可能危害较小，并能实现完全切除。新辅助化疗在为进一步手术做准备方面可能有潜在作用，尽管仍不明确。
自1994年以来，作者采用了两个连续的颅内室管膜瘤治疗方案，均包括对有手术肿瘤残留的儿童进行辅助化疗阶段，并计划在放疗前进行潜在的二次手术。
在第一个方案中，63名儿童中有9名接受了进一步手术，6名实现无瘤状态且无其他后遗症。他们的无进展生存期和无局部复发情况的预后与仅接受一次手术的儿童相当。在第二个方案中，努力实现完全切除，110名患者中有29名接受了再次手术：9名在第一次手术后，17名在化疗后，3名在放疗后不久。29名患者中有14名实现无瘤状态，其中1名出现神经症状恶化。将66名一次手术后实现无瘤状态的患者的结果与14名二次手术后实现无瘤状态的患者的结果进行比较。66名患者与另外14名患者的3年无进展生存率分别为71.4%±6.9%和90%±9.5%；3年无局部复发率分别为84.7%±5.9%和90%±9.5%；3年总生存率分别为85.9%±5.4%和87.5%±11.7%。
二次手术被证明是可行的，且无重大并发症，结果随时间推移有所改善。接受1次或更多次切除术的患者的局部肿瘤控制情况相当。
