Mixed adenoneuroendocrine carcinoma (MANEC) of the ampulla of Vater with sarcomatosis: a case report.

INTRODUCTION

Biliary mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are rare, accounting for only 4-24% of all intestinal MiNENs. Diagnosing them preoperatively remains challenging due to the limitations of biopsy and imaging. As a result, the optimal treatment strategy, particularly for postoperative management, is still poorly defined.

CASE PRESENTATION: A 61-year-old woman presented to the Surgical Department of Sfax General Hospital on 23 December 2021, with a 1-month history of jaundice, yellow discoloration of the eyes and urine, and clay-colored stools. Contrast-enhanced CT revealed dilated intra- and extrahepatic biliary ducts without evidence of stones or masses in the common bile duct. A positron emission tomography scan confirmed peritoneal and thoracic adenomegaly.

INTERVENTION: The patient underwent a cephalic pancreatoduodenectomy (Whipple procedure).

OUTCOMES HISTOPATHOLOGY: Histopathological examination of the surgical specimen confirmed a MiNEN. This case is particularly noteworthy for its rare association of a biliary MiNEN with a sarcomatoid component.

CONCLUSION

This case highlights the diagnostic and therapeutic complexities of biliary MiNENs. Given their rarity, sharing evidence-based clinical experiences is vital to standardize practices and improve patient outcomes. This report emphasizes the need for a multidisciplinary approach and thorough histopathological analysis to ensure accurate diagnosis and tailored management.