胰腺导管内乳头状黏液肿瘤（IPMN）是一种罕见的胰腺囊性肿瘤，上世纪70年代和80年代第一次报道了该病例[1]。20世纪90年，IPMN正式被命名，并成为胰腺囊性肿瘤一个特殊疾病[2]。IPMN占所有胰腺肿瘤的不足10%，及所有胰腺囊性肿瘤的25%左右[3]。大多数IPMN患者年位于60岁至70岁之间。男性患病率略高于女人[4]。近年来，随着大量检查仪器和外科器械的发明以及许多创新手术技术的不断涌现， IPMN在诊断、 治疗以及预后等各个方面均取得了巨大进展。本文针对IPMN在诊断及外科治疗及其预后等方面做一综述。

1：定义及其分型
1.1 胰腺导管内乳头状黏液肿瘤（IPMN）起源于胰腺导管上皮，呈乳头状增生，分泌过多的黏液，其特征是囊性或囊性扩张的分支胰管（Branch Ductal-IPMN，BD-IPMN和/或主胰管（Main Ductal-IPMN，MD-IPMN）。IPMN是一个可见的实体，不同于胰腺上皮内肿瘤（PanIN），它被定义为一个微观病变[5]。IPMN肿瘤大多位于胰腺头（70%），约20%位于胰体及胰尾，约10% - 5%呈腺体弥漫性受累。大多数是单发病灶但20% - 40%为多发病灶[ 6.7]。
1.2 IPMN根据它们的结构来源分为：胰腺主导管（16%至36%），胰腺支导管型（BD）（40%到65%）和混合或组合式（15%至23%）[ 8.9 10.11]，这种分类反映了重要的生物学差异及预后[ 12.13]。主胰管可轻度扩张，也可同时伴有胰腺萎缩。混合型表现为胰腺钩突分支胰管扩张合并主胰管扩张，也可表现为体尾部分支胰管和主胰管扩张的组合。然而，并不是所有IPMN病例都被认为存在潜在的恶性[14.15]，如肿瘤内出现>10 mm的实性结节、主胰管扩张>10mm、弥漫性或多中心起源、壁内钙化及糖尿病临床症状，应高度警惕，提示为恶性IPMN。大多数MDIPMN和混合型IPMN，临床表现出恶性肿瘤的特征（即重度异型增生或癌），其中45%与浸润性癌相关[ 16.17.18]。相反，大多数BD型显示低度发育不良，只有约15%是与浸润性癌。
2 诊断及鉴别诊断：
2.1鉴别诊断：根据患者年龄，临床表现和放射学结果。[ 16.19.20.21.22.23.24.25.26]，胰腺的几个病变可以与IPMN相鉴别，其中最可能被误诊为IPMN的胰腺疾病包括慢性胰腺炎、粘液性囊腺瘤，单纯性囊肿，浆液性囊腺瘤，和假性囊肿[27.28] 。
2.2诊断:胰腺囊性肿瘤的术前诊断准确性仍需优化。目前临床挑战是建立一个可根据临床随访和影像学确定是否需要手术。多层螺旋CT3D重建作为一种非侵入性的，方便和广泛应用的成像方法，可以提供详细的资料，对胰腺囊性病变[29]。“较大”的大小对非侵入性的成像可以做出诊断，而EUS-FNA可以帮助识别“小”囊肿，高级别/浸润性肿瘤[30]。超声内镜（EUS）和细针穿刺（FNA）联合，该项技术被广泛用于诊断实施,。据报道增强超声内镜（CE-EUS）可将BD-IPMN合并壁结节的诊断率提高到98%[31]，Iannicelli等人提到胰泌素刺激与MRCP能够更好地显示肿瘤和导管系统之间的关系，从而确定诊断[32 ]。Chen等人发现,超声造影将BD-IPMN与浆液性囊腺瘤分辨出来是有帮助的[33]。
3. 治疗：
因MD-IPMN有较大的恶变可能性，国际胰腺学会（IAP）推荐手术切除MD-IPMN，但是对于BD-IPMN的治疗取决于肿瘤是否有浸润倾向[34]；同样仙台共识指南也制定了对于IPMN的治疗手段；他们于2006年出台了相关指南并于2012年进行了修正，2012仙台标准建议对于MD-IPMN患者推荐手术切除，而对于分支胰管内乳头状黏液性肿瘤（BD-IPMN）内径达3厘米或更大的没有任何额外的“高风险”的因素存在，可密切观察。对于BD-IPMN的治疗疗更加倾向于保守，治疗手段取决于肿瘤的大小、胰管直径、存在胰腺炎与否等方面[16]，但这些指导方针基于专家意见，而不是存在强大的临床数据，仍需大量临床数据的支持。对于IPMN浸润型行配套辅助放化疗，可根据患者的术中及术后病理结果，包括所有阳性淋巴结和淋巴结阴性的患者，制定方案，霍普金斯大学一个团队的研究表明，辅助化疗将减少57%的死亡率，对于淋巴结转移阳性的患者，辅助化疗效果更加明显[35]。
5. 总结：
虽然IPMN占胰腺肿瘤的比例不足10%。然而，值得关注的是，如果未经恰当的处理的，他们中的一些可能会跟随不典型增生的癌序列，从而进展为浸润性癌。因为IPMN的发病率较低，我们对IPMN这种疾病的认识仍是不完整的。尽管放射学科及各种检查技术的进步，它仍然有时很难从其他良性病变区分。但就目前来说,对于MD-IPMN患者，适合手术切除。对于具有侵袭性IPMN治疗，是将胰体尾切除术并周围淋巴结清扫，同时限制切除无创性病变。对于IPMN的诊疗指南，仍需临床大数据的支持。
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