Perry Arie
Division of Neuropathology, Box 8118, Washington University School of Medicine, 660 South Euclid Avenue, St. Louis, MO 63110-1093, USA.
Adv Anat Pathol. 2002 Nov;9(6):345-50. doi: 10.1097/00125480-200211000-00003.
Prognostically favorable and unfavorable variants of medulloblastoma have recently been identified, corresponding to medulloblastoma with extensive nodularity and large cell/anaplastic medulloblastoma, respectively. In an effort to identify clinically relevant grading criteria for medulloblastoma in general, 330 Pediatric Oncology Group (POG) cases were carefully reviewed for presence and extent of histologic anaplasia, nodularity, and desmoplasia. The resulting data was statistically analyzed using event-free and overall patient survival as endpoints. Significant anaplasia (moderate to severe) was identified in 24% of cases and was strongly associated with decreased survival times. Additionally, those with diffuse or extensive anaplasia fared worse than those with only focal anaplasia. Although the study confirmed the favorable prognosis for the rare cases of medulloblastoma with extensive nodularity, lesser degrees of nodularity or desmoplasia were not associated with a statistically significant survival advantage. Medulloblastoma grading based on anaplasia demonstrated a statistically stronger association with patient outcome than clinical staging. Therefore, histologic grading of medulloblastomas seems warranted as a routine diagnostic aid.
髓母细胞瘤预后良好和不良的亚型最近已被确定，分别对应广泛结节性髓母细胞瘤和大细胞/间变性髓母细胞瘤。为了确定一般髓母细胞瘤的临床相关分级标准，对330例儿童肿瘤学组（POG）病例进行了仔细审查，以确定组织学间变、结节性和促纤维增生的存在及程度。以无事件生存期和患者总生存期作为终点，对所得数据进行了统计分析。24%的病例存在显著间变（中度至重度），且与生存期缩短密切相关。此外，弥漫性或广泛性间变的患者比仅局灶性间变的患者预后更差。尽管该研究证实了罕见的广泛结节性髓母细胞瘤预后良好，但较低程度的结节性或促纤维增生与统计学上显著的生存优势无关。基于间变的髓母细胞瘤分级与患者预后的统计学关联比临床分期更强。因此，髓母细胞瘤的组织学分级似乎有必要作为一种常规诊断辅助手段。
