Objective:To summarize clinical manifestations, imaging finding and pathological characteristics, diagnosis, treatment and survival of primary hepatic neuroendocrine neoplasm (PHNEN).Methods:Clinical data of 12 patients with PHNEN admitted to the Second Affiliated Hospital of Kunming Medical University from January 2020 to January 2022 were retrospectively analyzed. Among them, 5 patients were male and 7 female, aged from 24 to 77 years, with a median age of 66 years. The informed consents of all patients were obtained and the local ethical committee approval was received. According to the WHO classification of digestive system tumors in 2019, clinical manifestations, imaging finding and pathological characteristics, diagnosis and treatment, and survival of PHNEN patients were analyzed.Results:Abdominal pain was the main clinical manifestation, including 8 cases of abdominal pain and 2 cases of abdominal mass. 4 patients were diagnosed with single PHNEN and 8 cases of multiple PHNEN.7 cases were diagnosed with neuroendocrine tumor (NET), including 6 cases of grade G2 and 1 case of grade G3. 5 cases developed neuroendocrine carcinoma (NEC), including 3 cases of large cell type and2 cases of unknown type. Contrast-enhanced ultrasound showed that the incidence of hyper-enhancement in the arterial phase was 3/5, and the incidence of circumferential enhancement in enhanced CT scan was 6/12. Enhanced MRI indicated that the incidence of enhancement in the arterial phase was 9/12. The positive rates of neuron-specific enolase, ferritin and CA19-9 were 6/10, 5/9 and 5/12, respectively. Immunohistochemical staining showed that the positive rates of Syn, CD56, CgA, CD34 and CK-19 were 11/12, 9/12, 2/12, 10/10 and 7/9, respectively. 5 patients underwent surgery, 1 case of radiofrequency ablation +chemotherapy, 1 case of TACE, 1 case of chemotherapy + radiotherapy, 1 case of everolimus + octreotide, and 3 cases rejected further treatment after puncture biopsy. The follow-up time was ranged from 1.0 to22.0 months, with a median of 7.5 months. During subsequent follow-up, 7 patients survived, and most of them underwent surgery, radiofrequency ablation and TACE. 5 patients died, and most of them rejected treatment.Conclusions:PHNEN is manifested with non-specific clinical symptoms and certain imaging features. The diagnosis of PHNEN depends on pathological examination. Active surgical resection combined with TACE, chemotherapy and targeted therapy can improve the clinical prognosis of PHNEN patients.
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