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We report the case of a 72-year-old man who was initially diagnosed with far advanced intrahepatic cholangiocarcinoma, associated with bulky lymph node metastasis involving the common hepatic artery and moderate amount of ascites around the liver. After 10 cycles of systemic chemotherapy combining gemcitabine and S-1 with well-tolerated toxicities, a CT scan showed a marked shrinkage of the liver mass and lymph nodes (clinical partial response) with disappearance of ascites, which could permit a radical resection of the tumor. He underwent left lobectomy of the liver with lymph node dissection, and histopathological examination revealed pathologic complete response. Seven years after surgery, he is in a good overall condition.Keywords Intrahepatic cholangiocarcinoma (ICC) • Pathologic complete response (pCR) • Gemcitabine (GEM) • S-1

Background Mixed neuroendocrine–non-neuroendocrine neoplasms of the ampulla of Vater are rare and heterogenous, making it difficult to achieve a definitive preoperative diagnosis. Herein, we describe a patient in whom a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater was made preoperatively. Case presentation Computed tomography revealed an enhancing periampullary tumor in a 69-year-old man with obstructive jaundice. Subsequent duodenoscopy revealed an ulcerated lesion in the swollen ampulla of Vater, from which six biopsies were collected. Pathological examination revealed adenocarcinoma in five of them. The remaining one was a neuroendocrine neoplasm according to immunohistochemical analysis. With a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater, the patient underwent subtotal stomach-preserving pancreaticoduodenectomy with modified Child’s reconstruction and was discharged without complications. Pathological examination revealed both adenocarcinoma and neuroendocrine carcinomas, each accounting for ≥ 30% of the tumor, resulting in a definitive diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater. Lymph node metastases with neuroendocrine components were also observed. Adjuvant chemotherapy was not administered because of the patient’s renal dysfunction. Liver and lymph node metastases were detected 2 months after surgery, the neuroendocrine component being considered responsible for that relapse. The patient underwent platinum-based chemotherapy at 50% dosage, which initially resulted in significant tumor shrinkage; however, he died 6 months after surgery. Conclusions While these tumors’ heterogeneity make definitive preoperative diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater difficult, the possibility of this disease can be considered by careful examination. Further study is needed to establish the optimal diagnostic criteria and treatment strategy.

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