Ishikawa Mariko, Sumita Kazutaka, Tamura Kaoru, Kobayashi Daisuke, Tsukahara Yusuke, Inaji Motoki, Tanaka Yoji, Nariai Tadashi, Maehara Taketoshi
Department of Neurosurgery, Tokyo Medical and Dental University.
No Shinkei Geka. 2020 Mar;48(3):237-244. doi: 10.11477/mf.1436204169.
Primary cerebellar glioblastoma is a rare disease that accounts for 0.4-3.4% of glioblastoma multiforme(GBM)cases. The clinicopathological characteristics and prognosis of primary cerebellar GBM are not well understood due to its rarity and the lack of an established treatment strategy. To elucidate the prognostic factors and dissemination pattern, we retrospectively assessed four cases of cerebellar GBM that we treated between 2003 and 2013. All cases involved men, and the age range was 53 to 76 years(median 69.5 years);each patient underwent surgical removal and received adjuvant chemotherapy or radiotherapy. Every cerebellar GBM patient developed intrathecal dissemination at every stage of cerebellar GBM. Two patients had spinal metastases with tumor recurrence, and no patient had brain stem invasion. mutation and MGMT expression were both negative in three cases. The median overall survival of cerebellar GBM patients was 13.8 years, and the median progression-free survival was 5.5 years, which is similar to that reported in previous reports-and similar in terms of results-for supratentorial GBM treated at the same time at our institution. In conclusion, the prognosis of cerebellar GBM appears to be similar to that of supratentorial GBM;however, the pattern of tumor progression, such as intrathecal dissemination, is different. Craniospinal irradiation on cerebellar GBM should be carefully considered with frequent follow-up by whole spine survey using MRI.
原发性小脑胶质母细胞瘤是一种罕见疾病,占多形性胶质母细胞瘤(GBM)病例的0.4 - 3.4%。由于其罕见性以及缺乏既定的治疗策略,原发性小脑GBM的临床病理特征和预后尚不清楚。为了阐明预后因素和播散模式,我们回顾性评估了2003年至2013年间我们治疗的4例小脑GBM病例。所有病例均为男性,年龄范围为53至76岁(中位年龄69.5岁);每位患者均接受了手术切除,并接受了辅助化疗或放疗。每例小脑GBM患者在小脑GBM的各个阶段均发生了鞘内播散。2例患者出现脊髓转移并伴有肿瘤复发,无一例患者有脑干侵犯。3例患者的 突变和MGMT表达均为阴性。小脑GBM患者的中位总生存期为13.8年,中位无进展生存期为5.5年,这与先前报道的结果相似,并且与我们机构同期治疗的幕上GBM的结果相似。总之,小脑GBM的预后似乎与幕上GBM相似;然而,肿瘤进展模式,如鞘内播散,是不同的。对于小脑GBM应仔细考虑进行全脑全脊髓照射,并通过MRI进行全脊柱检查进行频繁随访。 (注:原文中“ mutation”这里有缺失信息)