bluuebunny/crossref_metadata_2025_split huggingface dataset:
1310 A Rare Case of Biliary Pancreatitis Caused by Ampullary Somatostatinoma in a 46-Year-Old Female
Authors: Kloska Michal , Vaze Reema , Buckshaw Ricky , Shah Hiral 
Published: October 2019

INTRODUCTION:
Somatostatinoma is a rare neuroendocrine tumor predominantly found in the pancreas and the duodenum and only in 3% of cases within ampulla of Vater. Affected people usually present with abdominal pain, GI bleed, obstructive jaundice or when metabolically active with somatostatin syndrome (DM, diarrhea, cholelithiasis). Somatostatinoma is typically a large malignant tumor usually with metastatic disease on presentation.

CASE DESCRIPTION/METHODS:
This is a case of a 46-year-old female with a medical history of cholecystectomy, fatty liver and diabetes mellitus who presented with a two-day history of scleral icterus, clay-colored stools and dark urine. On initial evaluation, she was afebrile and normotensive. Her lab work was consistent with acute pancreatitis (lipase 2498 U/L) as well as an obstructive biliary disease (ALP 1210 U/L; bilirubin 6.3 mg/dl; AST 157 U/L; ALT 153 U/L). CT scan of the abdomen showed a 1.5 cm bulbous irregular ampullary lesion and moderate intra-and extrahepatic as well as pancreatic duct dilation. Further evaluation with EUS revealed a 12 mm × 12.7 mm hypoechoic mass of the ampulla and FNA was obtained. ERCP revealed dilated bile ducts with the possibility of subtle stricture in the distal most portion of the CBD. Brushings were performed and 10 mm × 60 mm fully covered biliary metal stent was deployed with resolution of patient's symptoms and improvement of her liver enzymes. Cytology results from the stricture were negative for malignancy. EUS FNA was positive for neoplastic cells immunoreactive with synaptophysin and somatostatin. Unfortunately, the patient did not follow up until she developed abdominal pain 2 years later. Repeated CT of the abdomen showed 0.9 × 0.6 cm mass in periampullary region decreased in size from previous CT. ERCP revealed epithelization and tissue regrowth in the stent. Two 7 Fr, 7 cm Boston Scientific double-pigtail stents were placed through the previous metal stent with successful decompression. Pancreaticoduodenectomy is scheduled with surgical oncology.

DISCUSSION:
Somatostatinomas, especially in the ampullary region, may present as painless jaundice leading to acute biliary pancreatitis. Interestingly, even after patient was lost to follow up for 2 years, she did not develop metastatic disease which is usually found with first presentation of the disease. Nevertheless, due to high metastatic potential every case when possible should be treated radically and pancreaticoduodenectomy is the treatment of choice.
1312 A Prognosis Worse Than Pancreatic Adenocarcinoma—Metastatic Pancreatic Adenosquamous Carcinoma
Authors: Dharmavaram Gourisree , Shah Raj , Katz Jeffry 
Published: October 2019

INTRODUCTION:
Adenosquamous carcinoma of the pancreas (ASCAP) is a rare subtype of pancreatic cancer with estimated incidence of 1% to 4% among all pancreatic cancers. We report a case of this infrequently encountered tumor.

CASE DESCRIPTION/METHODS:
An 80-year-old man with hypertension, hyperlipidemia, social drinking (2 drinks/week), and tobacco abuse (24 pack years) presented with epigastric pain radiating to the back. Abdominal CT scan showed inflammatory pancreatic changes that were consistent with acute pancreatitis. CA 19-9 at this time was 222. MRCP revealed hypointense calcifications at the pancreatic head and uncinate process with numerous peripancreatic pseudocysts (largest 2 cm), favoring a diagnosis of acute on chronic pancreatitis. He was treated conservatively, improved, and was discharged home. He presented two months later with worsening epigastric pain and a 17-pound weight loss. CA 19-9 was now increased to 725. Repeat MRCP demonstrated persistence of pseudocysts and new 0.9 mm stricture at the mid-CBD with dilation of proximal CBD to 11 mm. EUS with FNA showed a 13 × 20 mm solid lesion with cystic component in the pancreatic head, showing squamous cell carcinoma. EGD showed gastric stenosis at the pylorus, and prepyloric gastric biopsy had tumor cells positive for CK7, CDX2, D2-40 and ERG, consistent with invasive adenocarcinoma with lymphovascular invasion. Further review of pathology with mucicarmine stain showed that the pancreatic tumor had a small adenocarcinoma component with predominant squamous differentiation, concluding metastatic ASCAP as the final diagnosis. Given his age and diagnosis, the patient received hospice. He survived 37 days from the time of initial presentation.

DISCUSSION:
ASCAP is thought to develop from squamous metaplasia related to inflammation from chronic pancreatitis. Our patient's presentation was atypical showing radiologic changes of chronic pancreatitis despite only one prior episode of acute pancreatitis. The prognosis of ASCAP is worse compared to pancreatic adenocarcinoma (PDAC) with reported median survival of 7 months. Additionally, aside from the risk factors of smoking, older age, and male gender, also associated with the more common PDAC, no other specific risk factors exist for development of ASCAP. Given the rarity of this diagnosis and the rapid deterioration of our patient, further information on pathogenesis and serum markers of ASCAP is necessary for improved risk stratification and better treatments for this poorly understood entity.
1315 Groove Pancreatitis as a Rare Complication of TACE
Authors: Mirza Zeyn , Estremera Rodolfo , Martin-Ortiz Jose 
Published: October 2019

INTRODUCTION:
Transarterial chemoembolization (TACE) is often performed for hepatocellular carcinoma. Side effects can occur due to arterial ischemia and cytotoxicity from chemotherapy delivery. TACE can be complicated via inadvertent embolization of arteries supplying the liver, gallbladder, stomach and pancreas. The association of acute pancreatitis with TACE is rare. The proposed mechanism involves regurgitation of embolic beads from the hepatic artery into the arterial supply of the pancreas. Acute pancreatitis can develop up to 15 days after TACE. Herein we present a case of groove pancreatitis occurring after TACE. Groove pancreatitis is a rare variant of focal chronic pancreatitis affecting the area between the head of the pancreas, duodenum, and the common bile duct. Functional and anatomical obstruction of the minor papilla from viscous pancreatic secretions leads to impaired pancreatic enzyme outflow and proliferation of Brunner's glands causing pancreatitis. Imaging may show a submucosal mass which may confound the diagnosis since pathologies including leiomyoma, gastrointestinal stromal tumor and ampullary carcinoma may have similar imaging findings. Groove pancreatitis remains largely unfamiliar to most physicians thus making its true incidence a challenge.

CASE DESCRIPTION/METHODS:
This is the case of a 69-year-old male patient with multicentric hepatoma secondary to chronic Hepatitis C, status-post TACE who presented to ER with a nine-day history of epigastric abdominal pain and nausea. The patient had received TACE nine days prior and was admitted with the diagnostic impression of acute pancreatitis. The physical exam was remarkable for flank pain, negative murphy's sign. Labs showed stable hemoglobin and platelet levels. Liver chemistry showed a mixed pattern with AST minimally elevated and ALT at 102, Alk Phos at 225, Amylase slightly elevated at 135 and lipase elevated at 161. Imaging studies showed acute pancreatitis with possible necrotic collection of 2.1 cm at the pancreatic head and uncinate process, duodenitis, mild gallbladder wall thickening and no ductal dilation. Likely etiology was pancreatitis secondary to recent TACE.

DISCUSSION:
Pancreatitis is a rare complication of TACE. Although elevated pancreatic enzymes may be seen in up to 40% of TACE cases, the incidence of symptomatic pancreatitis after TACE is low at 2%-4%. Despite this low incidence, it is a complication that one should be aware of in patients undergoing TACE procedure given its high morbidity and mortality.
1324 Recurrent Pancreatitis Associated With Pancreas Divisum and Rare Solid Pseudopapillary Neoplasm of the Pancreas
Authors: Ratra Atul , Block Geoffrey 
Published: October 2019

INTRODUCTION:
Pancreas Divisum (PD) is a benign congenital anomaly found in approximately 4-10% of general population. Rare cases of acute pancreatitis associated with PD and pancreatic tumors have been described. While most common causes of pancreatitis are gallstones and alcohol use, uncommon causes such as anatomic/genetic variants and malignancies of the pancreaticobiliary system should be investigated especially in case of recurrent cryptogenic pancreatitis. We present a case of recurrent pancreatitis in a patient with pancreas divisum and rare solid pseudopapillary neoplasm of the pancreas.

CASE DESCRIPTION/METHODS:
A 65-year old woman with past medical history of psoriatic arthritis on methotrexate therapy and no family history of pancreatitis was evaluated for recurrent pancreatitis. Patient had at least eight episodes of acute pancreatitis from 2011 to 2019. Patient denied any alcohol/tobacco history. Serum triglycerides/calcium were not elevated. Genetic causes were excluded. Imaging ruled out gallstones but showed presence of pancreas divisum. Endoscopic ultrasound (EUS)/Endoscopic retrograde cholangiopancreatography (ERCP) in 2016 was negative for stricture, focal mass or lesions. In April 2017, patient underwent repeat EUS/ERCP with stent placement into the minor papilla for presumed stenosis. However, that resulted in an exacerbation of her symptoms and the stent was subsequently removed. Patient continued to have recurrent acute pancreatitis and underwent repeat EUS in August 2018 which showed an 8 × 8 mm pancreatic genu mass adjacent to the Santorini duct. Biopsy was consistent with rare, slow growing solid pseudopapillary neoplasm (SPN) of the pancreas. Patient underwent pancreaticoduodenectomy in 2019. Pathology confirmed SPN. Patient has had no further episodes of pancreatitis.

DISCUSSION:
Pancreas Divisum (PD) results from failure of fusion of ventral and dorsal pancreatic ducts during embryogenesis. It has been controversial whether PD by itself causes recurrent pancreatitis. Clearly, in our patient, addressing the PD alone did not resolve her symptoms of recurrent pancreatitis. Sporadic hemorrhage within the SPN and intermittent compression of the Santorini duct was the likely cause of recurrent pancreatitis in our patient. Patients with cryptogenic recurrent pancreatitis should be evaluated periodically for the presence of slow growing tumors that may not be evident on initial imaging and may be the cause of recurrent pancreatitis in such patients.
1354 It's Not Always PSC: An Intriguing Case of IgG-4 Sclerosing Cholangitis
Authors: Ali Sara , Hammad Nour , Hakim Christopher M. , Iskendarian Ziyad 
Published: October 2019

INTRODUCTION:
IgG4-related disease has a broad spectrum of clinical manifestations, when it involves the biliary tract it is termed as IgG4-associated cholangitis. It is the most frequent extra-pancreatic manifestation of type 1 autoimmune pancreatitis and shares clinical and radiographic features with Primary Sclerosing Cholangitis however differentiation is important.

CASE DESCRIPTION/METHODS:
71-year-old female with a known history of recurrent pancreatitis and cholangitis status post laproscopic cholecystectomy presented to the hospital with abdominal pain and obstructive pattern of LFTs. Previous ERCPs had revealed common bile duct stricture and a stent had been placed. Prior CT, ultrasound and MRCP all were consistent with findings of pancreatitis with pancreatic duct dilation along with dilation of the intra-hepatic ducts. Endosonographic imaging of the pancreas demonstrated a markedly abnormal pancreas However CA19-9 was normal. She underwent repeat ERCP with biliary dilatation revealing multiple biliary strictures and underwent stent placement in the common bile duct and right hepatic duct. Cytology did not reveal malignant cells and core liver biopsy showed preserved hepatic architecture with no evidence of fibrosis. Features of autoimmune hepatitis are not identified. She presented in a couple of months with similar abdominal pain and ERCP revealed a new 2 cm hepatic duct stricture which was stented. Due to recurrent strictures in the biliary system with failed response to recurrent endoscopic stenting a multi disciplinary decision was made to repeat liver biopsy which revealed very dense lymphoplasmacytic inflammatory infiltrates, numerous lymphoid follicles and obliterative phelebitis similar to findings noted in type I autoimmune pancreatitis. IgG-4 liver stains performed highlighted multiple positive plasma cells establishing the diagnosis of IgG4-sclerosing cholangitis. She was started on steroids and noted to have improvement in her liver enzymes.

DISCUSSION:
The differentiation of IgG4-related sclerosing cholangitis from primary sclerosing cholangitis and bile duct carcinoma is often difficult however necessary as the former is steroid responsive where as the latter are not. Serum IgG-4 levels are highly predictive for IgG-RD. Tissue acquisition is absolutely essential for the diagnosis. We present a challenging case where due to non-conclusive histological evidence the diagnosis was delayed and later a portal-based inflammatory nodule was seen which is a characteristic feature of this disease.
1367 A Rare Feat: Successful ERCP Treatment of Mirrizi's Syndrome After Failed Surgery
Authors: Shiralkar Malan , Voaklander Rebecca , Walker Courtney , McVay Tyler , Sossenheimer Michael 
Published: October 2019

INTRODUCTION:
Mirizzi's syndrome (MS) is a rare complication, occurring in around 1% of all cholecystectomies and affecting around 0.1% of all patients with gallstones (GS). MS occurs when an impacted GS in the neck of the gallbladder (GB) or cystic duct (CD) causes compression of the common bile duct (CBD) or common hepatic duct (CHD), resulting in obstruction and potential progression to a cholecystocholedochal fistula. MS is usually managed by surgery. We report a rare case of a critically ill patient who was successfully cleared of obstructing stones via ERCP after failed surgery, allowing the patient to undergo subsequent successful heart transplantation.

CASE DESCRIPTION/METHODS:
A 42-year-old male with a history of coronary artery disease and ischemic cardiomyopathy presented with cholangitis due to CBD obstruction. Despite successful ERCP duct clearance, he was deemed not to be a surgical candidate due to severe cardiac morbidities. Months later he represented with cholangitis and was found on ERCP to have a CBD/CHD obstruction due to a large stone, originating from the CD, which was filled with many large stones. He underwent an attempt of GB resection. However, safe GB resection was precluded due to profound adhesions, complete tissue plane loss, and extensive fibrosis. GB wall debridement and removal of multiple large gallstones was surgically attempted to alleviate the CBD/CHD obstruction. A follow-up ERCP for stent removal showed persistent MS, thereby precluding the patient from heart transplant listing due to persistent cholangitis risk. Three subsequent ERCPs with aggressive SpyScope exploration of the CBD/CHD and CD, electrohydraulic shockwave as well as mechanical basket lithotripsy within the CD, and basket and balloon stone fragment removal resulted in complete clearance of all CD and CBD/CHD stones. Shortly afterwards, the patient presented with decompensated heart failure and underwent successful heart transplantation.

DISCUSSION:
MS presents a significant challenge to clinicians. The treatment is surgical, although inflammation, adhesions, and associated tissue plane distortion pose a challenge to even the best surgeon with an increased risk of bile duct injury. ERCP allows for diagnostic confirmation and biliary drainage via stent placement to alleviate the obstruction and cholangitis. We describe the successful implementation of ERCP as definitive therapy by utilizing a multitude of tools to clear the CD, CBD/CHD stones after failed surgery.
1368 Hilar Inflammation Mimicking a Klatskin Tumor
Authors: Shiralkar Malan , McKenzie Patrick , Lynch Keisa , O'Hara Ryan , Cizman Ziga , Sossenheimer Michael 
Published: October 2019

INTRODUCTION:
Hilar cholangiocarcinomas or Klatskin tumors (KT) pose significant diagnostic challenges; 15% of resections for KT prove to be non-malignant. Inflammatory, infectious, and other etiologies mimic radiological features of KT. A high index of suspicion is needed to avoid hepatic resection and associated morbidities while aggressively excluding KT.

CASE DESCRIPTION/METHODS:
A 64-year-old male who presented with jaundice, unintentional 50 lb weight loss, and fatigue was found to have bile duct dilation on ultrasound. A CT confirmed severe right and left hepatic duct dilatation with abrupt cutoff due to a 4 cm infiltrative hilar mass involving the right and left hepatic ducts, and extending into the common hepatic duct. Work-up for causes of jaundice (autoimmune hepatitis, hemochromatosis, alpha-1 antitrypsin deficiency, viral hepatitis, celiac sprue, autoimmune cholangiopathy) via ANA, AMA, F-actin, IgG, IgG4, HFE mutation, TIBC, ferritin, A1AT, tTG, hepatitis serologies was negative. Over 12 weeks, 3 ERCPs failed to confirm KT via biopsies, cytology, and fluorescence in situ hybridization (FISH). A severe proximal common hepatic duct stricture was found on initial ERCP, which was dilated and stented. ERCP with Spyscope showed hepatic bifurcation stricture improvement. This stricture was biopsied and again brushed, dilated and stented. A third ERCP showed further improvement in stricture and otherwise normal intra- and extra-hepatic ducts. Dilation to 8 mm was performed without further stenting. Cytology and FISH were negative for KT. Two CT guided biopsies of the mass were also negative for KT and failed to show biliary ductal thickening or clear evidence of the prior noted hilar mass. Two MRCPs, 7 months apart, showed significant improvement of right and left bile duct confluence stricturing, felt to represent fibrous tissue and scarring.

DISCUSSION:
Despite improvement in cross sectional imaging, advances in interventional radiologic and endoscopic modalities, differentiation between benign and malignant hilar biliary stenoses remains a major challenge. Up to 20% of hilar resection show chronic fibrosing inflammation without evidence of KT. We aggressively pursued this concern with 3 ERCPs (cytology, FISH, biopsy with Spyscope), and 2 CT guided biopsies. CT and MR showed radiologic improvement of the mass and lack of evidence of KT. Exclusion of autoimmune disease or other etiologies allowed a diagnosis of idiopathic inflammation, thus avoiding surgery.
1375 Osteoclast-Like Giant Cell Carcinoma of the Pancreas: Case Series
Authors: Pallante Angeline , Hansel Stephanie 
Published: October 2019

INTRODUCTION:
Osteoclast-like giant cell carcinoma of the pancreas (OGCP) is an extremely rare type of pancreatic neoplasm representing less than 1% of all pancreatic cancers. Due to its rarity, sufficient knowledge is lacking regarding the natural history of the tumor, its prognosis, and treatment recommendations.

CASE DESCRIPTION/METHODS:
We were respectively able to identify four confirmed cases of OGCP at our institution. Table 1 provides details of each case. Case 1: A 69 year-old male presented with left lower quadrant pain similar to prior pain associated with diverticulitis. The pain did not resolve with antibiotics. Imaging revealed a 1.2-cm non-enhancing mass in the pancreatic tail. Case 2: A 77 year-old male presented with a 7-month history of epigastric discomfort and weight loss. Imaging revealed a 9-cm pancreatic head mass. Case 3: A 65 year-old male had a PET scan for diagnosis of sarcoidosis. A new 1-cm pancreatic tail nodule was revealed. He denied GI symptoms. Case 4: A 65 year-old male reported 8-weeks of abdominal cramps, loss of appetite, and weight loss. CT imaging revealed a 13-cm pancreatic mass with extension into his liver.

DISCUSSION:
OGCs resemble giant cell tumors of the bone. OGCs are non-neoplastic cells that are reactive in nature and are massively recruited to the neoplasm which is a hallmark of this tumor. Patients typically present in their sixth to eight decades of life. The clinical presentation is like that of other large pancreatic masses. These masses typically occur in the body and tail of the pancreas; they are well vascularized and frequently hemorrhagic. They can be diagnosed by image-guided FNA or biopsy. Surgery remains first-line therapy for OGCP's. Unfortunately, once the tumor invades surrounding organs, such as the liver, the mortality rate significantly increases. Treatment is difficult in these patients as they are typically not healthy enough to begin chemotherapy. Gemcitabine remains the chemotherapy agent of choice, as most physicians treat OGCPs similarly to adenocarcinomas given the lack of literature guidance.
1377 Gastric Necrosis Associated With Necrotizing Pancreatitis
Authors: Mulki Ramzi , Shah Rushikesh , Qayed Emad 
Published: October 2019

INTRODUCTION:
Acute pancreatitis may lead to local complications and systemic toxicity. Gastric necrosis is a rare complication with only few cases reported in the literature. Herein we present a case of severe acute necrotizing pancreatitis acute complicated by gastric necrosis.

CASE DESCRIPTION/METHODS:
Our patient is a 42-year-old male with a history of idiopathic recurrent necrotizing pancreatitis who presented with severe epigastric abdominal pain, nausea and vomiting. Previous imaging revealed pancreatic necrosis in the body and head of the pancreas and extensive splanchnic thrombosis. Labs showed normal blood count, lipase and complete metabolic panel. CT of the abdomen during this admission revealed large volume ascites and total pancreatic necrosis and multiple areas of low attenuation in the expected location of the pancreatic head and body (Figure 1). Despite supportive care and antibiotics, he was unable to tolerate oral intake. On day 14 he developed hematemesis and hypotension. His hemoglobin dropped to 9.3 g/dL from 13.3 g/dL. An abdominal x-ray revealed a curvilinear lucency overlying the left lateral upper stomach suggestive of gastric pneumatosis. Upper endoscopy showed erythema, edema, and severe ulcerations with black necrotic mucosa in the inferior and posterior walls of the stomach. The anterior wall was relatively spared with clear demarcation of ischemic and viable mucosa (Figure 2). Gastric biopsies confirmed gastric necrosis. The patient's hemodynamic status worsened, and he required vasopressor support. Further interventions were deferred given his comorbidities and worsening hemodynamic status, and he was discharged to hospice care.

DISCUSSION:
Gastric necrosis is a rare complication of acute pancreatitis and occurs due to severe reduction in gastric mucosal blood flow. This can be secondary to thrombosis of large vessels such as the celiac trunk or attributed to microcirculatory vasospasm due to the release of thrombogenic and inflammatory mediators. The development of gastric necrosis leads to further clinical deterioration in a clinically ill patient. Despite its rarity, gastric necrosis should be suspected in the setting of severe pancreatitis in patients with signs of gastrointestinal bleeding and clinical deterioration.