Department of Surgery, Policlinico "Umberto I", "Sapienza" University of Rome, 00185 Rome, Italy.
Department of Radiological, Oncological and Anatomo-Pathological Sciences, Policlinico "Umberto I", "Sapienza" University of Rome, 00185 Rome, Italy.
Ann Ital Chir. 2024;95(5):783-787. doi: 10.62713/aic.3810.
Primary hepatic neuroendocrine carcinomas (PHNECs) are rare tumors with limited understanding and management. Diagnosing and treating these tumors pose significant challenges due to the lack of clinical experience. Surgery is often considered an effective therapeutic strategy for early-stage PHNECs. This case report describes an advanced metastatic PHNEC managed primarily with chemotherapy.
The medical records of the patient and diagnostic imaging findings were reviewed. A multidisciplinary team of specialists discussed the case and determined the treatment plan. The patient received a chemotherapy regimen tailored to PHNEC, which included specific neuroendocrine tumor-targeting agents.
Despite the advanced stage of the disease, the patient responded well to chemotherapy, with a notable reduction in tumor size and improvement in symptoms. The treatment was well-tolerated, and the patient showed a favorable overall response. The survival duration from the time of diagnosis was 18 months.
In this case of advanced metastatic PHNEC, a primarily chemotherapy-based approach yielded positive outcomes. Although surgery is typically preferred for early-stage cases, this case highlights the potential efficacy of chemotherapy in managing advanced PHNECs. Further research and clinical experience are warranted to better understand the optimal treatment strategies for these rare tumors.
原发性肝神经内分泌癌（PHNEC）是一种罕见的肿瘤，其发病机制和治疗方法尚不完全清楚。由于临床经验有限，诊断和治疗这些肿瘤具有挑战性。手术通常被认为是早期 PHNEC 的有效治疗策略。本病例报告描述了一例主要采用化疗治疗的晚期转移性 PHNEC。
我们回顾了患者的病历和诊断影像学结果。一个多学科专家小组对该病例进行了讨论，并制定了治疗计划。患者接受了针对 PHNEC 的化疗方案，其中包括特定的神经内分泌肿瘤靶向药物。
尽管疾病处于晚期，但患者对化疗反应良好，肿瘤大小明显缩小，症状改善。治疗耐受性良好，患者总体反应良好。从诊断到死亡的生存时间为 18 个月。
在这例晚期转移性 PHNEC 中，以化疗为主的治疗方法取得了良好的效果。尽管手术通常是早期病例的首选治疗方法，但该病例突出了化疗在治疗晚期 PHNEC 方面的潜在疗效。需要进一步的研究和临床经验来更好地了解这些罕见肿瘤的最佳治疗策略。
