Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
Koc University School of Medicine.
Am J Surg Pathol. 2024 Sep 1;48(9):1093-1107. doi: 10.1097/PAS.0000000000002275. Epub 2024 Jul 18.
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 A detailed clinicopathologic analysis of 72 previously unpublished IAPNs was performed.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 those with ≤1 cm invasion). 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). In conclusion, 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).
该指南最近将壶腹内乳头状管状肿瘤（IAPN）确认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 例以前未发表的 IAPN 进行了详细的临床病理分析。患者的男女比例为 1.8；平均年龄为 67 岁（范围：42 至 86 岁）；平均大小为 2.3 厘米。巨微镜相关性至关重要。从十二指肠的角度来看，壶腹通常对称升高，孔口张开，壶腹被拉伸的正常十二指肠黏膜覆盖。然而，在 6 例中，IAPN 向十二指肠表面的腔内肿瘤突起给人一种“壶腹-十二指肠肿瘤”的印象，只有通过微观相关性来准确诊断 IAPN，才能说明病变在壶腹边缘的突然结束。显微镜下，侵袭前成分通常显示混合表型（44.4% 主要为非肠型）。侵袭常见（94%），通常较小（平均 1.2 厘米），主要为胰胆管型（75%），具有侵袭性特征（66%有淋巴血管侵犯，41%有神经周围侵犯，30%有高芽生）。在 6 例中，侵袭前成分是纯肠型，但侵袭性成分是胰胆管型。淋巴结转移在 42%（≤1 厘米侵袭者为 32%）中发现。与胰胆管癌相比，预后明显更好（中位：69 与 41 个月；3 年：68%与 55%；5 年：51%与 35%，P=0.047）。总之，与壶腹十二指肠癌不同，IAPN 通常（44.4%）主要是非肠型，常见（94%）侵袭性，表现出侵袭性特征和淋巴结转移，即使是微小侵袭性，所有这些都使它们不太适合壶腹切除术。然而，与“胰胆管”癌相比，它们的预后仍然更好，目前在 CAP 方案中将 IAPN 与胰胆管肿瘤归为一类（虽然 IAPN 是胰胆管肿瘤的同类肿瘤，但后者是胰腺腺癌/胆管癌的壶腹对应物）。
