Ann Hepatobiliary Pancreat Surg.  2023 Nov;27(4):380-387. 10.14701/ahbps.23-045.

Clinicopathological characteristics of extrahepatic biliary neuroendocrine neoplasms in the gallbladder, extrahepatic biliary tract, and ampulla of Vater: A single-center cross-sectional study

• Park YM
• Seo HI
• Noh BG
• Kim S
• Hong SB
• Lee NK
• Kim DU
• Han SY

• KMID: 2548504
• DOI: http://doi.org/10.14701/ahbps.23-045

Abstract

Backgrounds/Aims
In 2019, the grading and staging system for neuroendocrine neoplasms (NENs) was significantly changed. In this study, we report the clinicopathological characteristics and surgical outcomes of patients with extrahepatic biliary NENs who underwent curative resection with or without adjuvant treatment.
Methods
We retrospectively reviewed a database of 16 patients who developed NENs, neuroendocrine carcinoma (NEC), and mixed endocrine non-endocrine neoplasms (MiNENs) after curative resection. Among them, eight patients had ampulla of Vater (AoV) tumors, and eight patients had non-AoV tumors.
Results
G1 and G2 were more frequently observed in the AoV group than in the non-AoV group (12.5% and 62.5%, respectively). In contrast, NEC and MiNEN were more common in the non-AoV group (50.0%). High Ki-67 index (> 20%) and perineural invasion (PNI) were more frequently observed in the non-AoV group. Advanced age (> 65 years), mitotic count > 20 per 2 mm² , and Ki-67 index > 20% were strongly correlated with patient survival (p = 0.018, 0.009, and 0.044, respectively). Advanced age (> 65 years) and mitotic count > 20 per 2 mm² were significantly correlated with disease recurrence (p = 0.033 and 0.010, respectively).
Conclusions
AoV and non-AoV tumors had significant differences in the histologic grade, Ki67, and PNI. Patients with non-AoV tumors had an increased risk for survival and recurrence than those in the AoV group. For extrahepatic biliary NENs, early detection of tumors, adequate surgery, and aggressive adjuvant treatment for high-risk patients are important to achieve long-term survival and prevent disease recurrence.

Keyword

Neuroendocrine neoplasm; Neuroendocrine carcinoma; Mixed endocrine non-endocrine neoplasm; Extrahepatic biliary tract; Ampulla of Vater

Figure

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  Fig. 1 Overall and disease-free survival rates of patients with neuroendocrine tumors. (A) The 1-, 3-, and 5-year overall survival rates were 87.5%, 81.3%, and 72.2%, respectively. (B) The 1-, 3-, and 5-year disease-free survival rates were 68.8%, 62.5%, and 62.5%, respectively.

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  Fig. 2 The survival and DFS comparison of the AoV and non-AoV groups. (A) The 1-, 3-, and 5-year postoperative OS rates: 87.5%, 87.5%, and 72.9%; and 87.5%, 75.0%, and 75.0%, p = 0.782, respectively. (B) The 1-, 3-, and 5-year postoperative DFS rates: 75.0%, 62.5%, and 62.5%; and 62.5%, 62.5%, and 62.5%, p = 0.869, respectively. DFS, disease-free survival; AoV, ampulla of Vater; OS, overall survival.

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