原发性肝神经内分泌瘤（PHNETs）是罕见的恶性肝肿瘤，由于其罕见性和无特异性临床特征，使其诊断具有挑战性。本研究旨在调查这种罕见肝肿瘤的特征，以增进我们对该疾病的理解，提高诊断准确性，并探索标准化的诊断和治疗方法。在体检中，发现两名年龄分别为64和74岁的老年妇女有肝脏肿块。两位患者的18F-FDG正电子发射计算机断层扫描（18F-FDG PET-CT）和Ga68-DOTATATE PET-CT扫描显示多发性肝脏肿块，最初被怀疑为肝神经内分泌瘤。随后的穿刺病理证实了神经内分泌瘤的诊断。此外，在1号病例中，18F-FDG PET-CT还检测出肺部的肿瘤，提示转移性肿瘤，并与肝脏免疫组化和影像学结果相结合。实验室检查显示肝功能和自身免疫性肝病指标无明显异常，无乙型肝炎感染的证据。然而，对于有远处转移或多个占位病变的病例并不适宜行部分切除术。对于这种情况，已经制定了个体化的治疗方案。肿瘤的大部分通过经动脉栓塞（TAE）治疗，并根据病理结果进行靶向化疗或内分泌治疗。在定期随访的13和12个月期间，肿瘤保持稳定。患者生活质量良好，心理健康状况良好。他们过着积极的生活方式，对自己的疾病及其进展有透彻的了解，并在随访过程中积极配合。我们的研究结果表明，血清学、影像学和免疫组化检查的综合应用有助于PHNET的诊断。此外，我们确定TAE联合药物治疗可能是控制PHNET进展的有效方法。定期术后随访对于监测PHNET患者的预后和肿瘤进展状态非常重要。Copyright © 2023 Tang, Chen, Lu, Yuan, Yang, Qiu and Li.

Primary hepatic neuroendocrine tumors (PHNETs) are rare malignant liver tumors that present diagnostic challenges owing to their rarity and absence of specific clinical features. This study aimed to investigate the characteristics of this rare liver tumor to enhance our understanding of the disease, improve diagnostic accuracy, and explore standardized diagnostic and treatment approaches.During physical examination, two elderly women, aged 64 and 74 years, were found to have liver masses. 18F-FDG Positron Emission Tomography-Computed Tomography (18F-FDG PET-CT) and Ga68-DOTATATE PET-CT scans of both individuals revealed multiple liver masses that were initially suspected to be hepatic neuroendocrine tumors. Subsequent puncture pathology confirmed the diagnosis of neuroendocrine tumors. Furthermore, in Case 1, the tumor was also detected by 18F-FDG PET-CT in the lung, suggesting a metastatic tumor, in conjunction with liver immunohistochemistry and imaging findings. Laboratory tests revealed no significant abnormalities in liver function or autoimmune liver disease indicators, and there was no evidence of viral hepatitis infection. However, partial hepatectomy was not indicated for cases with distant metastasis or multiple space-occupying lesions. Individualized treatment approaches have been developed for such situations. A large portion of the tumor underwent Transarterial Embolization (TAE), and targeted combination chemotherapy or endocrine therapy was administered based on the pathological results. During regular follow-ups a 13 and 12 months, the tumor remained stable. The patients' quality of life was good, and their psychological well-being was healthy. They led active lifestyles, demonstrated a thorough understanding of their disease and its progression, and actively cooperated during the follow-up process.Our findings suggest that a combination of serological, radiological, and immunohistochemical examinations can aid in the diagnosis of PHNET. In addition, we determined that TAE combined with drug therapy could be an effective method for controlling PHNET progression. Regular postoperative follow-ups are important for monitoring the prognosis and tumor progression status of patients with PHNET.Copyright © 2023 Tang, Chen, Lu, Yuan, Yang, Qiu and Li.