Tang Yongsheng, Chen Xianyu, Lu Xu, Yuan Zenan, Yang Yang, Qiu Chunhui, Li Hua
Department of Hepatic Surgery, Liver Transplantation Center, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Department of Hepatobiliary Surgery, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Front Oncol. 2023 Aug 4;13:1225583. doi: 10.3389/fonc.2023.1225583. eCollection 2023.
BACKGROUND & AIMS: 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.
原发性肝神经内分泌肿瘤(PHNETs)是一种罕见的肝脏恶性肿瘤,因其罕见性和缺乏特异性临床特征而在诊断上面临挑战。本研究旨在调查这种罕见肝脏肿瘤的特征,以增进我们对该疾病的了解,提高诊断准确性,并探索标准化的诊断和治疗方法。
在体检过程中,发现两名老年女性,年龄分别为64岁和74岁,肝脏有肿块。对两人进行的18F-氟代脱氧葡萄糖正电子发射断层扫描-计算机断层扫描(18F-FDG PET-CT)和镓68-奥曲肽PET-CT扫描显示有多个肝脏肿块,最初怀疑为肝神经内分泌肿瘤。随后穿刺病理确诊为神经内分泌肿瘤。此外,在病例1中,18F-FDG PET-CT在肺部也检测到肿瘤,提示为转移性肿瘤,结合肝脏免疫组化和影像学检查结果。实验室检查显示肝功能或自身免疫性肝病指标无明显异常,也没有病毒性肝炎感染的证据。然而,对于有远处转移或多个占位性病变的病例,不建议进行部分肝切除术。针对此类情况制定了个体化的治疗方法。大部分肿瘤接受了经动脉栓塞术(TAE),并根据病理结果给予靶向联合化疗或内分泌治疗。在术后13个月和12个月的定期随访中,肿瘤保持稳定。患者生活质量良好,心理健康。他们生活积极,对自己的疾病及其进展有充分了解,并在随访过程中积极配合。
我们的研究结果表明,血清学、放射学和免疫组化检查相结合有助于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况很重要。