Bräutigam Konstantin, Chouchane Aziz, Konukiewitz Björn, Perren Aurel
Institut für Gewebemedizin und Pathologie, Universität Bern, Murtenstr. 31, 3008, Bern, Schweiz.
Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Christian-Albrechts-Universität zu Kiel, Kiel, Deutschland.
Pathologie (Heidelb). 2024 Feb;45(1):35-41. doi: 10.1007/s00292-023-01276-4. Epub 2024 Jan 4.
Pancreatic neuroendocrine neoplasms (PanNEN) are rather rare entities. Morphology, combined with immunohistochemistry, allows typing and grading, thereby leading therapeutic decisions. Depending on tumor stage and differential diagnosis, a broad diagnostic panel may be required. The present work summarizes the minimal diagnostic, prognostic, and predictive markers in PanNEN.Markers of choice for defining a neuroendocrine phenotype are synaptophysin, chromogranin A, and INSM1. The proliferation fraction Ki67 is indispensable for grading, while p53 and Rb1 can help in the differentiation from neuroendocrine carcinoma (NEC). Transcription factors, such as cdx2, TTF‑1, and Islet‑1, can indicate the site of a primary tumor in the setting of a cancer of unknown primary (CUP). DAXX/ATRX immunohistochemistry has mainly prognostic value. Molecular pathology studies currently have little practical value in the diagnosis of PanNEN.An important pitfall in routine diagnostics is the wide spectrum of differential diagnoses mimicking neuroendocrine neoplasms. An expanded immunohistochemical panel is strongly recommended in case of doubt.
胰腺神经内分泌肿瘤（PanNEN）是相当罕见的疾病。形态学结合免疫组织化学可进行分型和分级，从而指导治疗决策。根据肿瘤分期和鉴别诊断，可能需要一套广泛的诊断指标。本研究总结了PanNEN中最低限度的诊断、预后和预测标志物。用于定义神经内分泌表型的首选标志物是突触素、嗜铬粒蛋白A和胰岛素瘤相关蛋白1（INSM1）。增殖分数Ki67对于分级必不可少，而p53和视网膜母细胞瘤蛋白1（Rb1）有助于与神经内分泌癌（NEC）相鉴别。转录因子，如尾型同源盒转录因子2（cdx2）、甲状腺转录因子1（TTF-1）和胰岛1（Islet-1），可在原发灶不明的癌症（CUP）情况下指示原发肿瘤的部位。死亡结构域相关蛋白（DAXX）/α地中海贫血伴智力低下综合征X连锁基因（ATRX）免疫组织化学主要具有预后价值。分子病理学研究目前在PanNEN的诊断中实用价值不大。常规诊断中的一个重要陷阱是存在一系列模仿神经内分泌肿瘤的鉴别诊断。如有疑问，强烈建议使用扩展的免疫组织化学指标。
