Mamelak A N, Prados M D, Obana W G, Cogen P H, Edwards M S
Department of Neurological Surgery, School of Medicine, University of California, San Francisco.
J Neurosurg. 1994 Jul;81(1):24-30. doi: 10.3171/jns.1994.81.1.0024.
Little is known about the risk of developing multicentric disease in patients with juvenile pilocytic astrocytoma (JPA), and even less about its prognosis. Only five cases have been reported. Between 1986 and 1992, the authors treated 90 patients with either primary or recurrent JPA, 11 of whom developed multicentric spread. Ten patients had primary tumors in the hypothalamic region, eight were under 4 years of age at initial diagnosis, all had initially undergone a subtotal resection or biopsy, and 10 received postoperative multiagent chemotherapy or irradiation for residual disease. Multicentric spread was discovered immediately to 108 months after initial diagnosis; nine patients were asymptomatic at the time. Most patients received chemotherapy for the multicentric disease, which was found throughout the craniospinal axis. During 21 to 148 months of follow-up monitoring, seven patients had stabilization or regression of multicentric disease and four died. Patients with hypothalamic region tumors were 23 times more likely to develop multicentric spread than were those with primary tumors located elsewhere (p < 0.001). Based on this review, it is concluded that multicentric spread of JPA occurs more frequently than was previously recognized. In patients with subtotally resected JPA and several years of follow-up review via magnetic resonance imaging, the incidence of recurrence in a site different from the original was 12%. Patients with subtotally resected JPA in the hypothalamic region should be considered to be at high risk for developing multicentric spread. Chemotherapy appears useful in stabilizing multicentric disease. Earlier detection and intervention may result in longer disease-free survival in patients with multicentric spread of JPA.
关于青少年毛细胞型星形细胞瘤（JPA）患者发生多中心性疾病的风险所知甚少，对其预后的了解更少。仅报告了5例。1986年至1992年间，作者治疗了90例原发性或复发性JPA患者，其中11例发生了多中心扩散。10例患者的原发性肿瘤位于下丘脑区域，8例初诊时年龄在4岁以下，均最初接受了次全切除或活检，10例因残留病灶接受了术后多药化疗或放疗。多中心扩散在初次诊断后立即至108个月被发现；9例患者当时无症状。大多数患者因多中心性疾病接受了化疗，该疾病遍布整个颅脊柱轴。在21至148个月的随访监测期间，7例患者的多中心性疾病病情稳定或有所缓解，4例死亡。下丘脑区域肿瘤患者发生多中心扩散的可能性是其他部位原发性肿瘤患者的23倍（p<0.001）。基于本综述，得出结论：JPA的多中心扩散比之前认为的更常见。在接受次全切除的JPA患者中，通过磁共振成像进行数年的随访复查，在与原发部位不同的部位复发的发生率为12%。下丘脑区域接受次全切除的JPA患者应被视为发生多中心扩散的高危人群。化疗似乎有助于稳定多中心性疾病。早期检测和干预可能会使JPA多中心扩散患者获得更长的无病生存期。
