The ampulla of Vater is a unique, highly vascularized pouch. Its anatomic transition is halfway along the second part of the duodenum from the foregut to the midgut. According to the World Health Organization’s (WHO) latest nomenclature, carcinoid tumors are now called neuroendocrine tumors (NETs). Knowledge of NETs is important because of their rarity, reclassification, prognosis, and management. NETs involving the ampulla of Vater are extremely rare, constituting <0.05% of gastrointestinal NETs, and involving the pancreas are rare to our knowledge. There are only a few reports of ampullary NETs. We report two rare NET cases involving the ampulla and pancreas and review the relevant literature. A 71-year-old patient with neurofibromatosis and multiple comorbidities presented with chronic intermittent abdominal pain. Abdominal imaging studies showed a suspicious mass at the level of the ampulla. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound revealed bulging papillae. Histopathology of the biopsied mass revealed a low-grade NET. Given his multiple comorbidities, the patient was scheduled for endoscopic resection. Our second patient was an 83-year-old lady presenting with nausea and vomiting. Abdominal imaging studies revealed a lobular mass over the body of the pancreas. Histopathological examination of fine-needle aspiration of the mass confirmed a well-differentiated low-grade NET. Octreoscan and dotatate scans showed pancreatic, multiple hepatic and metastatic lesions in the left lung and left shoulder. The patient is currently stable after completing peptide receptor radioligand therapy at a tertiary oncology center. Because of the patients’ comorbidities and staging, their management has taken different approaches. More data and more research are needed for accurate assessment of prognosis; however, a review of the latest literature recommends Whipple resection with lymphadenectomy for all ampullary NETs provided patients can tolerate the procedure. Endoscopic resection or surgical ampullary resection should be performed on contraindicated patients. The majority of ampullary and pancreatic NETs would have metastasized by the time patients sought treatment. Because of their rarity and ill-defined and highly variable presentation, NET diagnosis is always delayed and sometimes incidental; therefore, we emphasize the importance of early diagnosis and management to reduce mortality and morbidity.

Introduction: Vascular complications in pancreatitis generally occur in the form of hemorrhage or thrombosis. Pancreatitis resulting in splanchnic thrombosis has been well studied, but the cause of this correlation has not been studied in the current era of increasing anticoagulant use for deep venous thrombosis (DVT) prophylaxis. Hemorrhagic pancreatitis and peri-pancreatic bleeding are also known phenomena encountered in relation to pancreatitis, but these risks are not well established in the setting of chemical prophylaxis for DVT. Objectives: Our objective was to identify whether chemical DVT prophylaxis in pancreatitis harms the patient by increasing the risk of hemorrhagic conversion of pancreatitis or peri-pancreatic hemorrhage or if it is beneficial by preventing splanchnic venous thrombosis in the abdominal vasculature that surrounds the pancreas. Methods: We undertook a retrospective chart review with approval from the Institutional Review Board on patients who were hospitalized for or developed pancreatitis during their hospital stay from April 2014 to July 2015. We reviewed the charts for imaging suggestive of venous thrombosis or the development of intra-abdominal hemorrhage at admission during hospitalization and within 30 days after hospitalization. We also reviewed the methods of DVT prophylaxis to identify any correlation with the risk of hemorrhage or thrombosis. A bedside index of severity in acute pancreatitis score was used within 24 hours of admission to calculate the severity of the patients’ pancreatitis. The data collected were analyzed for descriptive statistics, correlation using Pearson’s coefficient, and multivariate regression analysis using Microsoft Excel and SPSS Inc. Released 2009. PASW Statistics for Windows, Version 18.0. Chicago: SPSS Inc. Results: This study included 389 patients who met the inclusion criteria. Of these, 74.6% of patients received chemical prophylaxis, mostly low molecular weight heparin, and 18.5% of patients were not on chemical or mechanical means of DVT prophylaxis. Only 12 patients (3%) had complications related to thrombosis and hemorrhage. Seven patients had splanchnic venous thrombosis, one had a hemorrhagic conversion of pancreatitis, three had a peri-pancreatic hemorrhage, and one had both the hemorrhagic conversion of pancreatitis and peri-pancreatic hemorrhage. Ten patients out of 12 patients had complications before admission, and nine of the 12 patients were on chemical prophylaxis. Pearson’s coefficient showed no statistically significant correlation between the incidence of complications and the use of chemical DVT prophylaxis. Multivariate analysis showed no specific variable that increased the risk of complications. Conclusions: Our study showed that chemoprophylaxis for DVT in patients hospitalized for acute pancreatitis is neither harmful by causing hemorrhagic conversion of pancreatitis, peri-pancreatic hemorrhage nor beneficial by preventing splanchnic venous thrombosis.

Introduction: Anti-mitochondrial antibody-positive primary biliary cholangitis (AMA-pos PBC) is an autoimmune disorder in which monoclonal antibodies are produced against epitopes with in the mitochondrial membranes of biliary epithelial cells, resulting in progressive non-suppurative biliary cholangitis. Up to 5 % of PBC patients lack these auto antibodies, termed as anti-mitochondrial antibody negative (AMA-neg) PBC. This is a somewhat new entity and a variant of AMA-pos PBC but not an overlap syndrome. There have not been good studies describing this phenomenon or associated terminology in the literature. Case Description/Methods: An 87-year-old woman was referred to our clinic after her medical oncologist found elevated isolated levels of serum alkaline phosphatase (714 units/L). She reported fatigue but denied any other symptoms. The physical examination was benign, except for bilateral lower extremity swelling secondary to lymphedema. Her serum alkaline phosphatase level decreased to 413 units/L after an initial dose of prednisone 40 mg daily, and she was maintained on 10 mg daily. Her antinuclear antibody titer was greater than 1:2560 in a centromere pattern. Anti-mitochondrial antibody was not detected. Total IgG level was 871 mg/dL (normal, , 1600 mg/dL), serum anti-smooth muscle antibody was negative, and the hepatitis panel was normal. Computed tomography of the abdomen and pelvis without contrast showed normal liver parenchyma and no acute intra-abdominal pathology. Histopathological examination indicated florid duct lesions. Background parenchyma showed no significant steatosis, and the inflammatory changes were limited primarily to the portal areas. Periodic acid-Schiff staining highlighted the intact hepatic parenchyma and architecture. The patient was diagnosed with AMA-neg PBC and responded well to Urosdeoxycholic acid therapy. (Figure) (Table). Discussion: This case highlights the importance of recognizing AMA-neg PBC as a variant of AMA-pos PBC and being able to differentiate them. Autoimmune cholangitis is a vague and imprecise term that cannot be used in this context. All AMA-negative PBC patients should be tested for other PBC-specific autoantibodies. Although prognosis and bile duct damage and loss are worse in AMA-neg PBC for unknown reasons, treatment remains the same for both.

Antimitochondrial antibody-positive primary biliary cholangitis (AMA-pos PBC) is an autoimmune disorder in which monoclonal antibodies are produced against epitopes in the mitochondrial membranes of biliary epithelial cells, resulting in progressive nonsuppurative biliary cholangitis. Up to 5% of patients lack these autoantibodies, termed antimitochondrial antibody-negative (AMA-neg) PBC. Although a somewhat new variant of AMA-pos PBC, it is not an overlapping syndrome. Few studies to date have described this phenomenon. An 87-year-old woman was referred to our clinic with elevated serum alkaline phosphatase (714 U/L). She reported fatigue but no other symptoms. A physical examination revealed a benign lesion and bilateral lower extremity swelling secondary to lymphedema. The serological profile was significant for a high antinuclear antibody titer (&gt;1:2,560) with a centromere pattern and negative for antimitochondrial antibody (AMA). The hepatitis panel was negative for viruses A, B, and C. Her serum immunoglobulin G level was 871 mg/dL (normal, &lt;1,600 mg/dL). The rest of the serological tests, including anti-smooth muscle antibodies (ASMA) and anti-liver/kidney microsomal antibodies, were negative. Computed tomography of the abdomen and pelvis without contrast showed normal liver parenchyma and no acute intra-abdominal pathology. Histopathology indicated florid duct lesions. The background parenchyma showed no significant steatosis, and inflammatory changes were limited to the portal areas. Periodic acid-Schiff staining revealed intact hepatic parenchyma and architecture. The patient was diagnosed with AMA-neg PBC and responded well to ursodeoxycholic acid therapy. This case highlights the importance of recognizing AMA-neg PBC as a variant of AMA-pos PBC and differentiating between them. Autoimmune cholangitis is a vague and imprecise condition. All patients with AMA-negative PBC should be tested for other PBC-specific autoantibodies. Although the prognosis and bile duct damage and loss are worse in AMA-neg PBC for unknown reasons, treatment remains the same for both.

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