Elshihabi Said, Husain Muhammad, Linskey Mark
Department of Neurological Surgery, Little Rock, Arkansas 72205, USA.
Surg Neurol. 2003 Dec;60(6):566-70. doi: 10.1016/s0090-3019(03)00192-7.
Lipomatous medulloblastoma is a rare but apparently distinct variant of medulloblastoma. There have been only 14 prior published cases. We report an additional case of an adult who presented with a multicentric form of this unique lesion.
A 45-year-old woman underwent magnetic resonance imaging for evaluation of progressive headaches, nausea/vomiting, vertigo, and ataxia. Multiple bilateral mass lesions in the posterior fossa were noted, along with obstructive hydrocephalus and significant mass effect.
Via a right suboccipital and retrosigmoid craniotomy the largest cerebellar lesion was resected and a second lesion biopsied. The third mass on the contralateral side was not disturbed. Postoperatively the patient received fractionated radiotherapy to a dose of 54 Gy and chemotherapy with cisplatin, PCNU, and vincristine for 1 year. She is alive without deficit, with a Karnofsky Performance Status of 100, and with no evidence of disease on neuroimaging 3 years after diagnosis.
This distinct variant of medulloblastoma appears to occur in adults only and has a uniquely favorable prognosis, even with incomplete resection with institution of appropriate adjuvant therapies. Multicentricity does not imply a less favorable prognosis.
脂肪型髓母细胞瘤是髓母细胞瘤中一种罕见但明显不同的变异型。此前仅有14例已发表的病例。我们报告了另外一例成年患者，其表现为这种独特病变的多中心形式。
一名45岁女性接受磁共振成像检查，以评估进行性头痛、恶心/呕吐、眩晕和共济失调。发现后颅窝有多个双侧肿块病变，伴有梗阻性脑积水和明显的占位效应。
通过右枕下乙状窦后开颅手术，切除了最大的小脑病变，并对第二个病变进行了活检。对侧的第三个肿块未予处理。术后患者接受了剂量为54 Gy的分次放疗，并使用顺铂、卡氮芥和长春新碱进行了1年的化疗。诊断3年后，她存活且无功能缺损，卡氏功能状态评分为100，神经影像学检查未发现疾病迹象。
这种独特的髓母细胞瘤变异型似乎仅发生于成年人，即使手术未完全切除且采用了适当的辅助治疗，其预后也特别良好。多中心性并不意味着预后较差。
