Departments of Pathology Radiation Oncology Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Neuropathology. 2012 Oct;32(5):534-42. doi: 10.1111/j.1440-1789.2012.01297.x. Epub 2012 Mar 1.
Gliosarcoma is a rare variant of glioblastoma multiforme (GBM) with similar clinical presentation and prognosis but a distinct genetic profile. The clinicopathological features of 22 cases of gliosarcoma were analyzed with respect to age, sex, KPS score, operative diagnosis, extent of resection and histopathological subtype (predominantly sarcomatous [PS], predominantly gliomatous [PG] or mixed). Twelve cases were PS, six were PG and four were mixed. The histological subtype did not correlate with the operative diagnosis; however, it did significantly correlate with the extent of resection (P=0.014). In 14 cases with available survival data it was found that none of the clinicopathological parameters significantly correlated with survival (P>0.05). Methyl guanine DNA methyl transferase promoter methylation studies were performed using methylation-specific PCR in 16 cases which showed a methylation rate of 31.25% (5/16). The promoter methylation status did not correlate with the histological subtype and did not significantly affect survival (P>0.05). Although gliosarcomas continue to be treated in the same way as GBM, the role of chemotherapy with temozolomide is not clear. This cohort is the largest to date to uniformly receive the Stupp's protocol which is currently "standard of care" for GBM. A median overall survival of 18.5 months is substantially higher than previous studies, suggesting that temozolomide should be included in gliosarcoma therapy.
胶质肉瘤是多形性胶质母细胞瘤(GBM)的一种罕见变异型,具有相似的临床表现和预后,但遗传特征明显不同。分析了 22 例胶质肉瘤的临床病理特征,包括年龄、性别、KPS 评分、手术诊断、切除范围和组织病理学亚型(主要为肉瘤型[PS]、主要为神经胶质瘤型[PG]或混合性)。12 例为 PS,6 例为 PG,4 例为混合性。组织病理学亚型与手术诊断无关,但与切除范围显著相关(P=0.014)。在 14 例有生存数据的病例中,发现没有任何临床病理参数与生存显著相关(P>0.05)。在 16 例可进行甲基鸟嘌呤 DNA 甲基转移酶启动子甲基化研究的病例中,使用甲基化特异性 PCR 进行了研究,结果显示甲基化率为 31.25%(5/16)。启动子甲基化状态与组织病理学亚型无关,也不会显著影响生存(P>0.05)。尽管胶质肉瘤仍按照与 GBM 相同的方式进行治疗,但替莫唑胺化疗的作用尚不清楚。这是迄今为止接受 Stupp 方案治疗的最大胶质肉瘤队列,目前该方案是 GBM 的“标准治疗方案”。中位总生存期为 18.5 个月,显著高于以往的研究,表明替莫唑胺应包含在胶质肉瘤的治疗中。