Multidisciplinary treatment with durvalumab-containing regimens for mixed neuroendocrine-non-neuroendocrine neoplasm of the extrahepatic bile duct.
Yuto Kitano, Kento Terasaki, Toru Kurata et al.
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Case in brief
A 63-year-old man presented with jaundice and a bile duct tumor involving the perihilar-to-distal extrahepatic duct, with bulky lymph node metastases. Initial biopsy showed adenocarcinoma. After biliary drainage and four cycles of gemcitabine, cisplatin, and durvalumab, the tumor and nodes decreased in size, allowing curative pancreatoduodenectomy. Resection pathology showed adenocarcinoma with a small-cell neuroendocrine component, confirming MiNEN. Para-aortic nodal recurrence developed four months after surgery; chemotherapy was resumed, but the patient died two years after treatment began.
What made this case unusual
The initial biopsy identified adenocarcinoma, whereas the resection specimen revealed an additional small-cell neuroendocrine component, establishing MiNEN.
Diagnostic / clinical pearl
A biopsy showing adenocarcinoma may not capture both components of a MiNEN; examination of the resection specimen established the mixed histology in this case.
Why it matters
Tumor shrinkage and curative resection were followed by early nodal recurrence, illustrating the aggressive course reported for MiNEN with a neuroendocrine carcinoma component.