OBJECTIVE To describe the long-term natural history of Branch duct intraductal papillary mucinous neoplasm (BD-IPMN). BACKGROUND The BD-IPMN is a known precursor of pancreatic cancer, yet its long-term natural history is largely unknown. METHODS We retrospectively reviewed patients with BD-IPMN who were followed at the Massachusetts General Hospital for at least ten years without surgical intervention. Patient and cyst characteristics, development of worrisome features (WF), need for surgery, and malignancy were recorded. The risk of pancreatic cancer in this cohort was compared with the general population by determining the Standardized Incidence Ratio (SIR). RESULTS 316 patients with BD-IPMN who were followed for at least ten years without intervention were identified. The median age was 63 years, and the median follow-up was 13.5 years (range 10 - 28.8 years). Median cyst size at diagnosis was 1.2 cm (IQR 0.8 - 1.7), was 1.8 cm (IQR 1.2-2.6) at ten years, and increased to 2.0 cm (IQR 1.3 - 3.0) by the end of surveillance. At the 10-year mark, 24% of patients had WF, and by the end of surveillance, an additional 20% had developed WF or high-risk stigmata. 8.2% of patients developed pancreatic malignancy (high-grade dysplasia or invasive cancer). The SIR for pancreatic cancer was 9.28 (95%CI of 5.82 - 14.06), with almost two-thirds of invasive cancers occurring within the pancreatic cyst. CONCLUSIONS After ten years of surveillance for BD-IPMN without intervention, the disease continues to progress and one of every 12 patients will develop malignancy. The risk of pancreatic cancer appears to be nine times higher than in the comparable age-matched population.

中文翻译：

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揭示分支管导管内乳头状粘液性肿瘤的长期自然史：超过 10 年。


目的 描述分支管导管内乳头状粘液性肿瘤（BD-IPMN）的长期自然史。背景 BD-IPMN 是已知的胰腺癌前兆，但其长期自然史很大程度上未知。方法 我们回顾性分析了 BD-IPMN 患者，这些患者在马萨诸塞州总医院随访了至少十年，没有进行手术干预。记录患者和囊肿的特征、令人担忧的特征（WF）的发展、手术的需要以及恶性肿瘤。通过确定标准化发病率（SIR），将该队列中的胰腺癌风险与一般人群进行比较。结果 确定了 316 名 BD-IPMN 患者，这些患者在没有干预的情况下被随访了至少十年。中位年龄为 63 岁，中位随访时间为 13.5 年（范围 10 - 28.8 年）。诊断时囊肿大小中位数为 1.2 cm (IQR 0.8 - 1.7)，十年时为 1.8 cm (IQR 1.2-2.6)，监测结束时增加至 2.0 cm (IQR 1.3 - 3.0)。 10 年时，24% 的患者患有 WF，到监测结束时，另有 20% 的患者出现了 WF 或高风险耻辱。 8.2% 的患者发展为胰腺恶性肿瘤（高度不典型增生或浸润性癌症）。胰腺癌的 SIR 为 9.28（95%CI 为 5.82 - 14.06），几乎三分之二的浸润性癌症发生在胰腺囊肿内。结论 经过 10 年的 BD-IPMN 监测且无干预后，该疾病继续进展，每 12 名患者中就有 1 名会发展为恶性肿瘤。胰腺癌的风险似乎比同龄人群高九倍。