Primary hepatic neuroendocrine tumor with multiple intrahepatic metastases and concurrent hepatic angiomyolipoma: a case report and review of the literature.

backgroundPrimary hepatic neuroendocrine tumor is extremely rare, and hepatic angiomyolipoma is also a rare benign tumor of the liver with malignant biological behavior. The combination of primary hepatic neuroendocrine tumor and hepatic angiomyolipoma is exceptionally rare, and to date, there have been no reports in the literature. CASE PRESENTATION: In this report, we present the case of a 70-year-old elderly Han man living in Mainland China with multiple intrahepatic metastases from a primary hepatic neuroendocrine tumor complicated with hepatic angiomyolipoma. He was admitted to our hospital with complaints of "recurrent diarrhea and progressively worsening abdominal pain." After admission, further imaging studies disclosed lesions with mixed signal intensity in the S2 segment of the liver, highly suggestive of hepatic angiomyolipoma, in addition to a well-defined mass adjacent to the gallbladder in the S4 segment of the left hepatic lobe, the nature of which remains undetermined, along with multiple other foci of abnormal signal intensity. Percutaneous biopsy of the mass adjacent to the gallbladder in the S4 segment of the liver confirmed a diagnosis of neuroendocrine tumor, and the symptoms of diarrhea were considered to be related to the tumor. With subsequent positron emission tomography-computed tomography imaging ruling out metastatic sources, the patient subsequently underwent open abdominal surgery for resection of multiple liver masses and left hepatic lobectomy. The pathological results revealed a larger nodule in the left hepatic lobe consistent with hepatic vascular smooth muscle lipoma (S2 segment). In addition, smaller nodules in the left hepatic lobe, adjacent to the gallbladder in the S4 segment, and in the S6 segment were consistent with neuroendocrine tumors (grade 1). The symptoms of diarrhea improved after operation. Postoperatively, abdominal contrast-enhanced computed tomography examination showed no residual masses, and adjuvant therapy was not administered. Follow-up at 6 months with liver contrast-enhanced magnetic resonance imaging revealed no tumor recurrence.

conclusionIn the extremely rare case reported herein, concurrent primary hepatic neuroendocrine tumor and hepatic angiomyolipoma was confirmed by imaging and pathology, and diarrhea was confirmed to be related to primary hepatic neuroendocrine tumor after surgery. This case not only highlights the association between neuroendocrine tumors and metabolic symptoms but also sheds light on the diagnosis and treatment of unexplained metabolic symptoms.