The guidelines recently recognized the intra-ampullary papillary tubular neoplasm (IAPN) as a distinct tumor entity. However, the data on IAPN and its distinction from other ampullary tumors remain limited. A detailed clinicopathologic analysis of 72 previously unpublished IAPNs was performed. The patients were: male/female=1.8; mean age=67 years (range: 42 to 86 y); mean size=2.3 cm. Gross-microscopic correlation was crucial. From the duodenal perspective, the ampulla was typically raised symmetrically, with a patulous orifice, and was otherwise covered by stretched normal duodenal mucosa. However, in 6 cases, the protrusion of the intra-ampullary tumor to the duodenal surface gave the impression of an "ampullary-duodenal tumor," with the accurate diagnosis of IAPN established only by microscopic correlation illustrating the abrupt ending of the lesion at the edge of the ampulla. Microscopically, the preinvasive component often revealed mixed phenotypes (44.4% predominantly nonintestinal). The invasion was common (94%), typically small (mean=1.2 cm), primarily pancreatobiliary-type (75%), and showed aggressive features (lymphovascular invasion in 66%, perineural invasion in 41%, high budding in 30%). In 6 cases, the preinvasive component was pure intestinal, but the invasive component was pancreatobiliary. LN metastasis was identified in 42% (32% in ≤1 cm IAPNs). The prognosis was significantly better than ampullary-ductal carcinomas (median: 69 vs. 41 months; 3-year: 68% vs. 55%; and 5-year: 51% vs. 35%, P=0.047). Unlike ampullary-duodenal carcinomas, IAPNs are often (44.4%) predominantly nonintestinal and commonly (94%) invasive, displaying aggressive features and LN metastasis even when minimally invasive, all of which render them less amenable to ampullectomy. However, their prognosis is still better than that of the "ampullary-ductal" carcinomas, with which IAPNs are currently grouped in CAP protocols (while IAPNs are kindreds of intraductal tumors of the pancreatobiliary tract, the latter represents the ampullary counterpart of pancreatic adenocarcinoma/cholangiocarcinoma).

中文翻译：

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壶腹内乳头状管状肿瘤 (IAPN)：72 例临床病理学分析凸显了一种难以识别的实体的独特特征。


该指南最近将壶腹内乳头状管状肿瘤（IAPN）视为一种独特的肿瘤实体。然而，关于 IAPN 及其与其他壶腹肿瘤的区别的数据仍然有限。对 72 个先前未发表的 IAPN 进行了详细的临床病理学分析。患者为：男/女=1.8；平均年龄 = 67 岁（范围：42 至 86 岁）；平均尺寸=2.3 厘米。宏观-微观相关性至关重要。从十二指肠的角度来看，壶腹部通常对称地凸起，具有扩张的开口，并且被拉伸的正常十二指肠粘膜覆盖。然而，在6例中，壶腹内肿瘤突出到十二指肠表面，给人一种“壶腹-十二指肠肿瘤”的印象，仅通过微观相关性（说明病变在十二指肠表面突然结束）才能准确诊断IAPN。壶腹边缘。显微镜下，侵袭前成分通常显示出混合表型（44.4% 主要是非肠道）。侵袭很常见（94%），通常较小（平均=1.2 cm），主要为胰胆型（75%），并表现出侵袭性特征（66%为淋巴管侵犯，41%为神经周围侵犯，30%为高出芽） 。 6例中，侵袭前成分为纯肠道成分，但侵袭成分为胰胆成分。 42% 的患者发现淋巴结转移（32% 的 IAPN ≤1 cm）。预后明显好于壶腹导管癌（中位：69 个月 vs. 41 个月；3 年：68% vs. 55%；5 年：51% vs. 35%，P=0.047）。与壶腹部十二指肠癌不同，IAPN 通常（44.4%）主要是非肠癌，通常（94%）具有侵袭性，即使是微创性的，也表现出侵袭性特征和淋巴结转移，所有这些都使得它们不太适合壶腹切除术。 然而，它们的预后仍然优于“壶腹导管”癌，IAPN 目前在 CAP 方案中被分组（虽然 IAPN 是胰胆管内肿瘤的同类，后者代表胰腺腺癌的壶腹对应物/胆管癌）。