Published online Mar 24, 2025. doi: 10.5306/wjco.v16.i3.101236
Revised: December 7, 2024
Accepted: December 27, 2024
Published online: March 24, 2025
Processing time: 134 Days and 20.1 Hours
Primary hepatic neuroendocrine tumors (PHNETs) are extremely rare tumors originating from neuroendocrine cells. Due to lack of neuroendocrine symptoms and specific radiographic characteristics, PHNETs are challenging to differentiate from other liver tumors.
This case involved a 67-year-old male who was admitted with a discovered hepatic mass and a suspicious lung lesion. Primary hepatic carcinoma was initially speculated based on the characteristic magnetic resonance imaging findings. The patient underwent a laparoscopic right partial hepatectomy, and subsequent immunohistochemical examination revealed a HNET. To exclude other potential origins, a positron emission tomography-computed tomography scan and gastrointestinal endoscopy were performed, leading to a final diagnosis of PHNETs. Then we conducted a literature review using the PubMed database, identifying 99 articles and 317 cases related to PHNETs. The characteristics, diagnostic methods, and treatment of PHNETs have been described. Finally, we elaborate on the presumed origins, pathological grades, clinical features, diagnosed methods, and treatments associated with PHNETs.
The diagnosis of PHNETs was primarily an exclusionary process. A definitive diagnosis of PHNETs relied mainly on immunohistochemical markers (chromogranin A, synaptophysin, and cluster of differentiation 56) and exclusion of primary foci in other organs. Radical surgery was the preferred treatment for early-stage tumors.
Core Tip: This paper reports a case of a rare primary hepatic neuroendocrine tumor (PHNET). Subsequently, we conducted a literature review using the PubMed database to describe the characteristics, diagnostic methods, and treatment of PHNETs. The diagnosis of PHNETs relies on definitive pathological findings, with the primary objective of preoperative examinations being the exclusion of extrahepatic primary sources. Radical surgery is the preferred treatment for early-stage tumors, and overall outcomes of PHNETs are generally better than those of gastrointestinal NETs with liver metastasis.