Clinicopathological Characteristics of Extrahepatic Biliary Neuroendocrine Neoplasms in the Gallbladder, Extrahepatic Biliary Tract, and Ampulla of Vater: A Single-Center Cross-Sectional Study

BackgroundSignificant changes were made in the grading and staging systems for neuroendocrine neoplasms (NENs) in 2017. Therefore, a clinical report to comprehensively update the clinicopathological characteristics, therapeutic approaches, and prognosis of these patients should be drafted.MethodsWe retrospectively reviewed a database of 16 patients who developed NENs or mixed endocrine non-endocrine neoplasms (MiNENs) after curative resection. Among them, eight had ampulla of Vater (AoV) tumors, and eight had non-AoV tumors. One patient had NEN Grade 1, five had NEN Grade 2, and five had NEN Grade 3 (G3); all had poorly differentiated neuroendocrine carcinoma. Five patients had MiNEN or combined carcinoma.ResultsThe mean age was 57.88 years. The overall survival rates after curative surgery at 1, 3, and 5 years were 87.1, 80.4, and 71.4%, respectively. The 1-, 3-, and 5-year disease-free survival rates were 87.1%, 79.8%, and 71.8%, respectively. In univariate analysis, age >65 years, mitotic count (>20/10 high-power fields), Ki-67 index >20%, and presence of stones were significant prognosticators, whereas only the mitotic count was statistically significant in the multivariate analysis. Age >65 years (p=0.039), a Ki-67 index >20% (p=0.03), presence of stones (p=0.021), G3 stage disease, MiNEN (p=0.002), or perineural invasion (p<0.001) were more frequently observed in the non-AoV group than in the AoV group.ConclusionHigh mitosis count had a greater effect on extrahepatic biliary NEN than the tumor-node-metastasis staging system. Patients with AoV tumors had better prognostic predictor factors compared to those with non-AoV tumors.

Surgical Outcomes and Comparative Analysis of Transduodenal Ampullectomy and Pancreaticoduodenectomy: A Single-Center Study

Background Transduodenal ampullectomy (TDA) is performed for adenoma or early cancer of the ampulla of Vater (AoV). This study aimed to analyze the short- and long-term outcomes of TDA (TDA group) when compared with conventional pancreaticoduodenectomy (PD) or pylorus-preserving pancreaticoduodenectomy (PD group). Methods Patients who underwent TDA between January 2006 and December 2019, and PD cases performed for AoV malignancy with carcinoma in-situ (Tis) (high-grade dysplasia, HGD) and T1 and T2 stage from January 2010 to December 2019 were reviewed. Results Forty-six patients underwent TDA; 21 had a benign tumor, and 25 cases with malignant tumors were compared with PD cases (n = 133). Operation time (p < 0.001), estimated blood loss (p < 0.001), length of hospital stays (p = 0.003), and overall complication rate (p < 0.001) were lower in the TDA group than in the PD group. Lymph node metastasis rates were 14.6% in pT1 and 28.9% in pT2 patients. The 5-year disease-free survival and 5-year overall survival rates for HGD/Tis and T1 tumor between the two groups were similar (TDA group vs PD group, 72.2% vs 77.7%, p = 0.550; 85.6% vs 79.2%, p = 0.816, respectively). Conclusion TDA accompanied with lymph node dissection is advisable in HGD/Tis and T1 AoV cancers in view of superior perioperative outcomes and similar long-term survival rates compared with PD.

Assessment of Telomere Length and Molecular Characterization of TERT Gene Promoter in Periampullary Carcinomas

Genetic and epigenetic alterations of the telomere maintenance machinery like telomere length and telomerase reverse transcriptase (encoded by TERT gene) are reported in several human malignancies. However, there is limited knowledge on the status of the telomere machinery in periampullary carcinomas (PAC) that are rare and heterogeneous groups of cancers arising from different anatomic sites around the ampulla of Vater. In the current study, we investigated the relative telomere length (RTL) and the most frequent genetic and epigenetic alterations in the TERT promoter in PAC (n=20) and compared with tumor-adjacent nonpathological duodenum (NDu; n=16). We found shorter RTLs (1.27 vs 1.33, P=0.01) and lower TERT protein expression (p=0.04) in PAC tissues as compared to the NDu. Although we did not find any mutation at two reactivating hotspot mutation sites of the TERT promoter, we detected polymorphism in 55% (11/20) of the cases at rs2853669 (T>C). Also, we found a hypermethylated region in the TERT promoter of PACs consisting of four CpGs (cg10896616 with ∆β 7%; cg02545192 with ∆β 9%; cg03323598 with ∆β 19%; and cg07285213 with ∆β 15%). In conclusion, we identified shorter telomeres with DNA hypermethylation in the TERT promoter region and lower TERT protein expression in PAC tissues. Further studies with a larger sample size are necessary to substantiate these results.

Acute obstructive pancreatitis secondary to migration of a gastrostomy tube into duodenum

Percutaneous endoscopic gastrostomy (PEG) tube is a common procedure. This discusses the rare complication of acute pancreatitis, due to tube migration, causing obstruction of the ampulla of Vater. Radiological confirmation of tubes prior to usage may aid in preventing this reversible complication.

Rare Orbital Metastasis Originating from Ampullary Adenocarcinoma

Background: Orbital metastasis from ampullary carcinoma is rare, with no previously reported cases. Case presentation: We report the case of a 60-year-old man who complained of a right-sided headache, blurred vision, progressive proptosis, ptosis, and right eye pain for 3 months. His past medical history included an ampullary adenocarcinoma stage IIIA treated via the Whipple procedure and adjuvant chemoradiotherapy 1 year ago. However, he was lost to follow-up. Computed tomography of the orbit showed a soft tissue lesion in the right orbital fossa measuring 3.3 × 2 × 2 cm. An orbital mass biopsy demonstrated an intestinal-type adenocarcinoma that tested positive for cytokeratins 7 and 20 and CDX2 on immunohistochemical staining. The pathologic diagnosis was metastatic adenocarcinoma from the ampulla of Vater. Despite oncological treatment, the patient’s illness progressed. He received palliative treatment and died 1 month later. Conclusions: We presented a rare case of orbital metastasis from ampullary adenocarcinoma. This should be considered in the differential diagnosis of patients with a history of ampullary adenocarcinoma who present with symptoms referring to the relevant locations.