Background
Mixed Neuroendocrine-Nonneuroendocrine Neoplasms (MiNENs) are rare tumors comprising of both adenocarcinoma and neuroendocrine component, at least 30% of each in the lesion. We report 4 biliary MiNENs, with a brief literature review.
Case 1: 22 year old male with jaundice had distal bile duct MiNEN, and a single DOTA positive liver lesion. He underwent neoadjuvant 4 cycles FOLFOX followed by pancreaticoduodenectomy (PD).
Case 2: 65 year old male came post-laparoscopic cholecystectomy with biopsy suspicious of adenocarcinoma. Patient underwent completion radical cholecystectomy which revealed an infiltrating GB MiNEN. Patient is on Cisplatin + Etoposide.
Case 3: 51 year old male presented with endoscopic ampullectomy for periampullary lesion - MiNEN arising from distal bile duct. Patient was taken for PD, abandoned due to dense adhesions to major vessels. He was given 3 cycles of FOLFIRINOX followed by surgery followed by 2 cycles of Cisplatin + Etoposide, later changed to 4 cycles of Gemcitabine + Capecitabine due to poor response. He responded well, and is disease free at 14 months.
Case 4: 35-year old lady with painless jaundice had a mid-bile duct stricture. Endoscopic Ultrasound guided Fine Needle Aspiration Cytology (EUS-FNA) revealed neuroendocrine differentiation. She underwent margin-negative extrahepatic bile duct resection that was a MiNEN. She was given 6 cycles of capecitabine + temozolomide, and is disease free at 2 years.
Conclusion
Biliary MiNENs don’t have a dedicated treatment approach as of now. Treatment for resectable tumours is upfront surgery, but advanced tumours can be downstaged with chemotherapy and then resected. Survival in MiNENs is generally poor, and response to chemotherapy is modest.