Alipour Zahra, Sweeney Jacob R, Zhang Qingzhao, Yang Zhaohai
Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman, School of Medicine, Philadelphia, PA.
Department of Pathology, Cleveland Clinic, Cleveland, OH.
Adv Anat Pathol. 2023 Jan 1;30(1):58-68. doi: 10.1097/PAP.0000000000000369. Epub 2022 Sep 22.
Most pancreatic neuroendocrine neoplasms are slow-growing, and the patients may survive for many years, even after distant metastasis. The tumors usually display characteristic organoid growth patterns with typical neuroendocrine morphology. A smaller portion of the tumors follows a more precipitous clinical course. The classification has evolved from morphologic patterns to the current World Health Organization classification, with better-defined grading and prognostic criteria. Recent advances in molecular pathology have further improved our understanding of the pathogenesis of these tumors. Various issues and challenges remain, including the correct recognition of a neuroendocrine neoplasm, accurate classification and grading of the tumor, and differentiation from mimickers. This review focuses on the practical aspects during the workup of pancreatic neuroendocrine neoplasms and attempts to provide a general framework to help achieve an accurate diagnosis, classification, and grading.
大多数胰腺神经内分泌肿瘤生长缓慢，即使发生远处转移，患者也可能存活多年。这些肿瘤通常呈现出具有典型神经内分泌形态的特征性类器官生长模式。一小部分肿瘤的临床病程更为凶险。其分类已从形态学模式发展到目前的世界卫生组织分类，分级和预后标准更加明确。分子病理学的最新进展进一步加深了我们对这些肿瘤发病机制的理解。仍然存在各种问题和挑战，包括神经内分泌肿瘤的正确识别、肿瘤的准确分类和分级以及与相似病变的鉴别。本综述重点关注胰腺神经内分泌肿瘤检查过程中的实际问题，并试图提供一个通用框架，以帮助实现准确的诊断、分类和分级。
