Abstract
BACKGROUND: Primary hepatic Neuroendocrine carcinoma (PHNEC) is exceptionally rare, and when it cannot be surgically removed locally, systemic combination therapy is the preferred treatment. However, current treatments have shown limited effectiveness, and more effective approach remains a matter of debate. CASE PRESENTATION: We present a case involving a female patient diagnosed with non-surgically suitable PHNEC, confirmed through pathology. Following four cycles of standard first-line systemic chemotherapy, this intervention was prompted by imaging indicating suboptimal local lesion control, the patient underwent localized interventional and microwave ablation therapy. Subsequently, an evaluation based on mRECIST criteria revealed complete remission post-procedure. The disease sustained this remission status throughout the 14-month follow-up, with the administration of 14 cycles of immunocheckpoint inhibitor maintenance therapy, showing no signs of local recurrence or distant metastasis and devoid of any associated complications. CONCLUSION: This case introduces a novel therapeutic avenue for individuals who are ineligible for surgery and have not responded to systemic chemotherapy. The diagnosis and management of PHNEC are deliberated within the framework of this particular case.