Institute of Pathology, Department of Medical Services, Ministry of Public Health, Bangkok, Thailand.
Pediatr Blood Cancer. 2012 May;58(5):690-4. doi: 10.1002/pbc.23325. Epub 2011 Sep 15.
Neuroblastoma is characterized by heterogeneity of histology, biology, and clinical behavior. Most epidemiology studies are based on Western and Japanese populations; there are very few studies on neuroblastoma from Southeast Asia.
Cases of Thai children with neuroblastoma were retrospectively reviewed to determine if the epidemiology of the disease differs from Western populations. Sixty-two cases were assembled from two pathology centers in Bangkok. Histologic prognostic category and MYCN copy number were determined.
The median age at diagnosis was 2.9 years. Only 11% of cases presented at less than 1 year of age and 12% cases had low stage disease (1, 2, and 4S). The majority of tumors had unfavorable histology (48/62); this was at least partly due to the higher age at diagnosis for most patients. MYCN amplification was detected in 18/52 (35%) tumors, all in stage 3 or 4 tumors. We assigned patients to high, intermediate and low risk categories using the Children's Oncology Group risk stratification criteria. In contrast to Western studies, the majority of cases (50/59 or 85%) in our series had high risk disease.
Since there is no evidence to date that the biology of neuroblastoma varies by geographic region, the paucity of low risk cases in our study may reflect spontaneous resolution/differentiation of tumors that are not clinically detected. Moreover, a delay in diagnosis of intermediate risk cases could result in higher tumor burden at the time of diagnosis, increasing the proportion of high risk cases observed.
神经母细胞瘤的组织学、生物学和临床行为具有异质性。大多数流行病学研究基于西方和日本人群；来自东南亚的神经母细胞瘤研究非常少。
回顾性分析泰国儿童神经母细胞瘤病例，以确定疾病的流行病学是否与西方人群不同。从曼谷的两个病理中心收集了 62 例病例。确定了组织学预后类别和 MYCN 拷贝数。
诊断时的中位年龄为 2.9 岁。只有 11%的病例在 1 岁以下发病，12%的病例为低分期疾病（1、2 和 4S）。大多数肿瘤具有不良的组织学特征（48/62）；这至少部分是由于大多数患者的诊断年龄较高。52 例（35%）肿瘤中检测到 MYCN 扩增，均为 3 期或 4 期肿瘤。我们使用儿童肿瘤学组风险分层标准将患者分为高危、中危和低危组。与西方研究不同，我们研究中大多数病例（50/59 或 85%）为高危疾病。
由于迄今为止没有证据表明神经母细胞瘤的生物学因地理位置而异，我们研究中低危病例较少可能反映了未被临床检测到的肿瘤自发消退/分化。此外，中间风险病例的诊断延迟可能导致诊断时肿瘤负荷更高，从而增加观察到的高危病例比例。
