There are few large-scale epidemiological and prognostic studies on blastic plasmacytoid dendritic cell neoplasm (BPDCN) due to its rarity. We used the Surveillance, Epidemiology, and End Results database to investigate the incidence, clinical characteristics, prognostic factors, and survival trends of BPDCN. The age-adjusted incidence of BPDCN had a bimodal pattern with peaks in those under 20 and 60 years and older. Of 697 patients, the median age at diagnosis was 31 years. The most common primary sites were lymph nodes (59.4%), followed by bone marrow (17.1%) and skin (11.6%). Extranodal involvement (59.7%) was more common in patients aged 60 years and older, while lymph node involvement was predominant in other age groups. The 1-year, 3-year, and 5-year overall survival (OS) rates were 90.7%, 83.7%, and 82.3% in patients aged under 20, but dropped to 53.1%, 27.7%, and 20.0% in patients aged 60 and older. Multivariate Cox regression analysis revealed that age, sex, and first malignancy were independent prognostic factors for OS. Based on this regression model, a nomogram was built with high discrimination and calibration. The incidence, clinical characteristics, and prognosis of BPDCN patients vary by age group. Moreover, using the nomogram to predict OS can help guide individualized evaluations and clinical decisions.

中文翻译：

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母细胞浆细胞样树突状细胞肿瘤的发病率、预后因素和生存结果：监测、流行病学和最终结果数据库中的一项回顾性研究

由于母细胞浆细胞样树突状细胞肿瘤 (BPDCN) 的罕见性，很少有大规模的流行病学和预后研究。我们使用监测、流行病学和最终结果数据库来调查 BPDCN 的发病率、临床特征、预后因素和生存趋势。BPDCN 的年龄调整发病率呈双峰模式，在 20 岁以下和 60 岁及以上人群中达到峰值。在 697 名患者中，诊断时的中位年龄为 31 岁。最常见的原发部位是淋巴结 (59.4%)，其次是骨髓 (17.1%) 和皮肤 (11.6%)。结外受累 (59.7%) 在 60 岁及以上的患者中更为常见，而淋巴结受累在其他年龄组中占主导地位。20 岁以下患者的 1 年、3 年和 5 年总生存率 (OS) 分别为 90.7%、83.7% 和 82.3%，但在 60 岁及以上的患者中下降到 53.1%、27.7% 和 20.0%。多变量 Cox 回归分析显示年龄、性别和首发恶性肿瘤是 OS 的独立预后因素。基于该回归模型，构建了具有高辨别力和校准度的列线图。BPDCN 患者的发病率、临床特征和预后因年龄组而异。此外，使用列线图预测 OS 有助于指导个体化评估和临床决策。BPDCN 患者的预后因年龄组而异。此外，使用列线图预测 OS 有助于指导个体化评估和临床决策。BPDCN 患者的预后因年龄组而异。此外，使用列线图预测 OS 有助于指导个体化评估和临床决策。