Schneider Christian, Ramaswamy Vijay, Kulkarni Abhaya V, Rutka James T, Remke Marc, Tabori Uri, Hawkins Cynthia, Bouffet Eric, Taylor Michael D
Developmental and Stem Cell Biology, Division of Neurosurgery, Neurooncology and Neuropathology, Arthur and Sonia Labatt Brain Tumor Research Center, The Hospital for Sick Children, Toronto, Ontario, Canada.
J Neurosurg Pediatr. 2015 Mar;15(3):236-42. doi: 10.3171/2014.9.PEDS14280. Epub 2014 Dec 19.
While medulloblastoma was initially thought to comprise a single homogeneous entity, it is now accepted that it in fact comprises 4 discrete subgroups, each with its own distinct demographics, clinical presentation, transcriptomics, genetics, and outcome. Hydrocephalus is a common complication of medulloblastoma and not infrequently requires CSF diversion. The authors report the incidence of CSF diversion surgery in each of the subgroups of medulloblastoma (Wnt, Shh, Group 3, and Group 4).
The medical and imaging records for patients who underwent surgery for medulloblastoma at The Hospital for Sick Children were retrospectively reviewed. The primary outcome was the requirement for CSF diversion surgery either before or within 60 days of tumor resection. The modified Canadian Preoperative Prediction Rule for Hydrocephalus (mCPPRH) was compared among subgroups.
Of 143 medulloblastoma patients, treated from 1991 to 2013, sufficient data were available for 130 patients (15 with Wnt, 30 with Shh, 30 with Group 3, and 55 with Group 4 medulloblastomas). Of these, 28 patients (22%) ultimately underwent CSF diversion surgery: 0% with Wnt, 29% with Shh, 29% with Group 3, and 43% with Group 4 tumors. Patients in the Wnt subgroup had a lower incidence of CSF diversion than all other patients combined (p = 0.04). Wnt patients had a lower mCPPRH score (lower risk of CSF diversion, p = 0.045), were older, had smaller ventricles at diagnosis, and had no leptomeningeal metastases.
The overall rate of CSF diversion surgery for Shh, Group 3, and Group 4 medulloblastomas is around 30%, but no patients in the present series with a Wnt medulloblastoma required shunting. The low incidence of hydrocephalus in patients with Wnt medulloblastoma likely reflects both host factors (age) and disease factors (lack of metastases). The absence of hydrocephalus in patients with Wnt medulloblastomas likely contributes to their excellent rate of survival and may also contribute to a higher quality of life than for patients in other subgroups.
虽然髓母细胞瘤最初被认为是一个单一的同质实体，但现在人们认识到它实际上由4个不同的亚组组成，每个亚组都有其独特的人口统计学特征、临床表现、转录组学、遗传学和预后。脑积水是髓母细胞瘤的常见并发症，常常需要进行脑脊液分流。作者报告了髓母细胞瘤各亚组（Wnt、Shh、3组和4组）中脑脊液分流手术的发生率。
对在病童医院接受髓母细胞瘤手术的患者的医疗和影像记录进行回顾性审查。主要结局是在肿瘤切除前或切除后60天内是否需要进行脑脊液分流手术。对各亚组的改良加拿大术前脑积水预测规则（mCPPRH）进行比较。
在1991年至2013年接受治疗的143例髓母细胞瘤患者中，有130例患者（15例Wnt型、30例Shh型、30例3组和55例4组髓母细胞瘤）有足够的数据。其中，28例患者（22%）最终接受了脑脊液分流手术：Wnt型为0%，Shh型为29%，3组为29%，4组为43%。Wnt亚组患者脑脊液分流的发生率低于所有其他患者的总和（p = 0.04）。Wnt患者的mCPPRH评分较低（脑脊液分流风险较低，p = 0.045），年龄较大，诊断时脑室较小，且无软脑膜转移。
Shh、3组和4组髓母细胞瘤的脑脊液分流手术总体发生率约为30%，但本系列中没有Wnt髓母细胞瘤患者需要分流。Wnt髓母细胞瘤患者脑积水发生率低可能反映了宿主因素（年龄）和疾病因素（无转移）。Wnt髓母细胞瘤患者无脑积水可能有助于其良好的生存率，也可能有助于其生活质量高于其他亚组患者。
