神经内分泌肿瘤（NET）是由神经内分泌细胞发展而来的罕见恶性肿瘤，最常见于胃肠道、肺和胰腺。这些肿瘤的治疗过程很大程度上取决于原发部位，这可能会给原发部位未知的 NET 的诊断和治疗带来挑战。在此，我们介绍一例原发性不明且具有明显肝转移的 NET 病例。我们的目的是强调原发性不明的 NET 的检查的关键组成部分，并详细介绍当前文献推荐的生化、组织病理学和成像模式。我们强调多学科方法对这些患者的诊断和治疗以及治疗选择的重要性。版权所有 © 2024，Patresan 等人。

Neuroendocrine tumors (NETs) are uncommon malignancies that develop from neuroendocrine cells which most commonly occur in the GI tract, lung, and pancreas. Treatment courses for these tumors are largely dictated by the primary origin site, which can present diagnostic and therapeutic challenges in NETs of unknown primary origin. Herein, we present a case of an NET of unknown primary origin with significant liver metastases. Our aim is to highlight the key components of the workup of NETs of unknown primary origin and detail the biochemical, histopathological, and imaging modalities as recommended by current literature. We highlight the importance of a multidisciplinary approach to both diagnosis and treatment of these patients as well as touch upon therapeutic options.Copyright © 2024, Patresan et al.