背景:髓母细胞瘤,有四个分子亚组,通常是快速生长的肿瘤,具有显着的对比增强和明确的边缘。然而,每个亚组的临床特征,包括疾病时程和影像学特征,没有很好的定义。
方法:作者描述了一例15岁女性,她有7个月的左手运动受损病史,发现第四脑室背侧有病变。患者首次表现的T2加权磁共振成像(MRI)显示弥漫性高信号,无明显肿块,钆增强T1加权成像显示非常轻微的对比增强。在1个月内,她的症状进展了,随访MRI显示病变的大小增加,显示更大的扩散限制和对比度增强。她接受了全切除,病理与经典髓母细胞瘤一致。肿瘤的遗传分析证实了无翼(WNT)分子亚群。进行辅助化疗和质子束治疗。在18个月的随访中,MRI显示疾病无复发。
结论:生长缓慢的髓母细胞瘤非常罕见,不知道与特定的分子亚群相关。这里,作者报告了一例生长缓慢的WNT髓母细胞瘤,表明缓慢的增长可能是这个亚组的一个特征。
BACKGROUND: Medulloblastomas, with four molecular subgroups, are generally rapid-growing tumors with significant contrast enhancement and well-defined margins. However, each subgroup\'s clinical features, including disease time course and imaging characteristics, are not well defined.
METHODS: The authors describe the
case of a 15-year-old female who presented with a 7-month history of impaired left-hand movement and was found to have a lesion on the dorsal side of the fourth ventricle. T2-weighted magnetic resonance imaging (MRI) at the patient\'s first presentation showed diffuse hyperintense signal without apparent mass, and gadolinium-enhanced T1-weighted imaging showed very slight contrast enhancement. In 1 month, her symptoms progressed, and follow-up MRI revealed an increase in the size of the lesion, showing greater diffusion restriction and contrast enhancement. She underwent gross-total resection, and pathology was consistent with classic medulloblastoma. Genetic analysis of the tumor confirmed the wingless (WNT) molecular subgroup. Adjuvant chemotherapy and proton beam therapy were performed. At the 18-month follow-up, MRI showed no recurrence of disease.
CONCLUSIONS: Slow-growing medulloblastoma is very rare and not known to be associated with a specific molecular subgroup. Here, the authors report a
case of slow-growing WNT medulloblastoma, indicating that slow growth may be a feature of this subgroup.