bluuebunny/crossref_metadata_2025_split huggingface dataset:
1333 Not so Small Biliary Obstruction: A Case of Small Cell Carcinoma of the Ampulla of Vater
Authors: Gomez Paul , Naim Alan , Zucker Kelly , Kiafar Camron , Gomez Adam 
Published: October 2019

INTRODUCTION:
Small cell carcinomas commonly arise in the lung, but can also originate in extrapulmonary sites. Extrapulmonary small cell carcinomas are rare and are most frequently found in the female genital tract, gastrointestinal tract and genitourinary tract. Small cell carcinoma of the ampulla of Vater is exceedingly rare, and there are currently less than 15 documented case reports. The neuroendocrine features of small cell carcinoma of the lung and extra pulmonary sites appear to be similar.

CASE DESCRIPTION/METHODS:
64-year-old male with past medical history of hypertension and coronary artery disease presented to our hospital with weight loss and jaundice. At time of admission, labs demonstrated AST 114 U/L, ALT 168 U/L, alkaline phosphatase 715 U/L, total bilirubin 5.8 mg/dL (Dbili 4.5), INR 1.1, platelet 271 k/mm3, and albumin 3.5 g/dL. Notable findings on physical exam included jaundice and scleral icterus. Ultrasound of the biliary tree showed no visible stone. ERCP showed an ulcerated, bleeding ampullary mass. Subsequent cholangiogram revealed a distal bile duct stricture measuring approximately 1.5 cm with shelf effect from extension of ampullary mass into the bile duct. Sphincterotomy was performed, followed by distal bile duct biopsy and stent placement. Pathology report of ampullary mass showed findings consistent with small cell neuroendocrine carcinoma. Immunohistochemical staining showed malignant cells were positive for synaptophysin and chromogranin and negative for cytokeratin AE1/3 and CD45. Patient underwent a Whipple procedure with specimen demonstrating a 4.2 cm × 2.8 cm exophytic mass at the ampulla with invasion into the pancreas. Five of twenty peripancreatic lymph nodes were positive for metastatic small cell carcinoma. Following resection our patient was started on adjuvant chemotherapy with cisplatin and etoposide.

DISCUSSION:
The most common tumor involving the ampulla of Vater is adenocarcinoma. Small cell neuroendocrine carcinoma of the ampulla of Vater accounts for no more than 2% of ampullary malignancies. This case illustrates that although biliary obstruction is commonly attributed to choledocholithiasis and other intrinsic or extrinsic tumors, rarer forms of malignancy should be considered in the differential. Careful assessment of the ampulla during ERCP can aid with diagnosis. Final diagnosis is challenging and can only be confirmed with histopathological examination. Treatment involves a multidisciplinary approach involving surgical resection and chemotherapy.
1336 A Rare Case of a Non-Functioning Pancreatic Neuroendocrine Tumor
Authors: Dasu Neethi R. , Khalid Yaser , Gordon Richard 
Published: October 2019

INTRODUCTION:
Pancreatic neuroendocrine tumors (PNETs) are very rare, representing 1–2% of all pancreatic tumors and 7% of all NETs. 85% of PNETs are non-functional PNETs (NF-PNETs). Most are found incidentally at an advanced stage because patients are asymptomatic and the tumors do not secrete hormones that would manifest with symptoms. We report a case of a 32-yr-old female diagnosed with widely metastatic intermediate grade NF-PNET. For pancreatic neuroendocrine tumors that have metastasized, prognosis is poor, with a survival of only 1–3 years.

CASE DESCRIPTION/METHODS:
A 32-yr-old female presented with increasing abdominal girth and lower extremity swelling. A CT of abdomen and pelvis showed a massively enlarged liver with diffuse metastases, pancreatic tail mass, vaginal and peritoneal masses, sigmoid colon mass, and gallbladder masses. Core biopsy of her liver showed a metastatic NF-PNET. Following 6 cycles of chemotherapy with capecitabine and temozolomide, repeat CT showed a significant decrease in her pancreatic mass and metastases. Her chromogranin A level decreased from 5800 to 35 ng/dL. Then she had an exploratory laparotomy, lysis of adhesions, and bilateral oopherectomy. Postoperatively she was changed to octeotide and everolimus until MRI showed a progression of her liver metastases. She was switched to 4 cycles of treatment with doxorubicin, 5-fluorouracil (5-FU), and streptozocin as well as Y90 to her liver. Repeat MRI showed stable disease in her liver.

DISCUSSION:
Based on histology and pathology Ki 67 of 40% and 10 mitoses/10 HPF our patient's tumor was classified as G2 intermediate grade. Our patient had none of the potential risk factors for PNETs. Imaging includes an Octreoscan, negative for our patient, and CT scans in 3 phases. Our patient tested negative for increased levels of all hormones and biomarkers seen with newly diagnosed NETs. Serum levels of CgA are elevated in the 80-90% of patients with advanced NETs and can be used to monitor disease progression and treatment as seen in our case. Surgery is the only treatment proven to prolong survival as seen with our patient. Medical treatment with somatostatin analog octreotide plus chemotherapy regimen of doxorubicin, 5-FU and streptozocin are used for locally advanced and metastatic pancreatic endocrine carcinomas like with our case. This case is important because it illustrates an atypical presentation in a younger patient and it is important for practitioners to be vigilant in diagnosing rare diseases.
1342 A Rare Presentation of Lactobacillus Paracasei Endocarditis With Involvement of Aortic Prosthetic Valve in a Patient With Septic Shock Due to Acute Cholecystitis
Authors: Gueorguieva Iana , Kosti Jorgena , Makandura Mahesha , Ismail Rana 
Published: October 2019

INTRODUCTION:
Endocarditis caused by Lactobacilli species is rare and usually manifests in patients with severe comorbidities, congenital heart disease, or prosthetic valves. Lactobacilli species are gram-positive coccobacilli mostly found in the gastrointestinal tract flora. Current literature shows an association of this bacterium with the use of probiotics. We hypothesize a possible association between acute cholecystitis and the occurrence of Lactobacillus endocarditis in a patient with no history of use of probiotics.

CASE DESCRIPTION/METHODS:
A 75-year-old female with a history of Birt-Hogg-Dube Syndrome with multiple spontaneous pneumothoraxes, COPD, aortic stenosis status post prosthetic aortic valve (AV) replacement, presented with acute respiratory failure and septic shock. She was diagnosed with acute cholecystitis, severe aortic regurgitation, and found to have lactobacillus bacteremia. We started her on empiric antibiotic coverage and later switched to penicillin G and gentamicin following three positive blood cultures for Lactobacillus paracasei. A transesophageal echocardiogram showed vegetation with emerging severe aortic regurgitation, degeneration, and perforation of AV leaflet when compared with echocardiogram one year ago. A CT-thorax demonstrated pan lobular emphysema with large cysts and pneumatoceles at the lung bases. Bacteremia persisted for two weeks after initiation of antibiotic therapy. Treatment with penicillin G and gentamicin was deemed necessary for up to 4 weeks after definitive surgical treatment. However, the patient's severe lung condition complicated preoperative clearance, and she was transferred to a specialized center for open-heart AV replacement.

DISCUSSION:
Very few cases of Lactobacillus endocarditis are reported in the literature. A handful of studies describe some implicated pathogenesis of the Lactobacillus in the blood as induced by cholecystitis. Other studies suggest that the increased presence of Lactobacillus in the respiratory tract in cases of severe lung pathology can lead to hematogenous dissemination to the heart valves. However, the association between Lactobacillus species and prosthetic valve material (and implication with endocarditis) is currently well-established. All things considered, and despite the lack of histopathology, we suggest that the source of Lactobacillus was likely the acute cholecystitis, and the presence of lung comorbidities and prosthetic AV further exacerbated the patient's condition.
1347 From the Outside Looking in: Psoriasiform Dermatitis Presenting as a Paraneoplastic Syndrome for Pancreatic Adenocarcinoma
Authors: Narasimha Shruthi , Drame Awa , Shah Jalak 
Published: October 2019

INTRODUCTION:
Cancer is a devastating finding but diagnosis can be difficult due to the vague symptoms and often indolent course. Sometimes, medical professionals have clues to possible malignancy precursors but it can easily be overlooked when they are presented in different clinical pictures. Paraneoplastic syndromes range from almost ever organ system but the case discussed here deals with a rare cutaneous manifestation that can be seen with a number of malignancies known as bazex syndrome.

CASE DESCRIPTION/METHODS:
This case involved a 64-year-old female with history of hypertension and paroxysmal atrial fibrillation who came to the hospital with complaints of yellow discoloration of her skin, intermittent fevers and a recent history of right upper quadrant pain with nausea. She was seen by her primary care physician who did basic labs that showed a total bilirubin of 14.3 and she was sent to the emergency room for further evaluation. Of note, patient was previously seen in the hospital 6 months prior for a skin rash on her extremities and abdomen that was biopsied and later described as psoriasiform dermatitis. It was thought to be a drug reaction from a medication but the rash still persisted after discontinuation. On this admission, a CT abdomen showed masses in the pancreatic head and neck with confluent disease encasing the periportal structures that was concerning for malignancy. Biopsy after endoscopic ultrasound showed adenocarcinoma and the patient was evaluated by oncology with gastroenterology and she had biliary sphincterotomy with stenting and was started on modified FOLFIRINOX therapy.

DISCUSSION:
This case highlights a rare finding with the psoriasiform dermatitis rash that erupted a few months prior to formal diagnosis of pancreatic adenocarcinoma, which is concerning for an uncommon paraneoplastic process known as bazex syndrome. What makes this case even more uncommon is that bazex is usually seen in head, neck and lung cancers and is less commonly seen in gastrointestinal malignancies. Clinicians should be made aware of these cutaneous changes, so that they can improve on early evaluation, diagnosis and possible treatment.
1349 A Rare Presentation of Cholecystogastric Fistula With Associated Mirizzi Syndrome Leading to the Diagnosis of Gallbladder Cancer
Authors: Bournique Marissa , Lopez Tania , Kudelko Marc , Thomas Miriam 
Published: October 2019

INTRODUCTION:
Biliary fistulas are a rare complication of gallstone disease. Technological advances have allowed for successful management & diagnosis. Mirizzi syndrome is a condition seen in less than 1% of patients with cholelithiasis & malignancy in 5 to 28% of cases. This is a rare presentation of coexistent Mirizzi syndrome and a cholecystogastric fistula leading to the diagnosis of gallbladder adenocarcinoma.

CASE DESCRIPTION/METHODS:
A 68 y/o female presented to the ER with complaints of jaundice & pruritis. Records demonstrated prior CTA&P with cholelithiasis with stranding & nonspecific stranding around the distal stomach. MRI w/wo contrast demonstrated loss of fat plane between gallbladder & stomach suggestive of fistulizing disease & a filling defect within the extrahepatic common hepatic duct related to external compression suggestive of Mirizzi Syndrome, no pneumobilia was seen. EGD/EUS was without direct visualization a fistulous tract, but revealed ulceration of gastric mucosa suggesting extrinsic pressure from a gallstone. ERCP demonstrated a stricture within the proximal CBD. Brushings were negative for malignancy. Lap chole revealed adherence of the stomach to the gallbladder fundus & takedown of cholecystogastric fistula was also performed. Histology from gallbladder specimen returned positive for invasive poorly-differentiated adenocarcinoma with transmural and lymphatic invasion.

DISCUSSION:
The presentation of cholecystogastric fistula generally includes pneumobilia & lacks pruritis suggestive of obstructive jaundice due to drainage of the biliary system via tract formation. Yet during the development of the fistulous tract the presentation varies quite drastically. Surgery is guided by the sub-classification of fistula formation/erosion as noted in the table figure. ERCP allows better characterization of the anatomy, in addition to offering the potential for therapeutic intervention with biliary decompression. There is an important known relationship between Mirizzi syndrome & gallbladder cancer likely related to the recurrent irritation occurring in the region along with chronic biliary stasis therefore frozen sections should be performed in all cases of Mirizzi syndrome to conclusively evaluate for gallbladder cancer. Our case highlights the malignancy potential within the presentation of fistulizing gallbladder disease. With a heightened index of suspicion and advancements in endoscopic and surgical techniques management of these patients will hopefully become less challenging.
1351 The Peculiar Presentation of Extramedullary Plasmacytomas as Acute Pancreatitis
Authors: Jiang Yi , Di Vitantonio Thomas A. , Rotundo Laura , Kutner Matthew , Galan Mark A. , Mirani Neena , Ahlawat Sushil 
Published: October 2019

INTRODUCTION:
Extramedullary plasmacytomas (EMP) are a subcategory of plasma cell neoplasms that involve organs outside the bone marrow. Less than 5% of the cases are seen in the gastrointestinal tract. Pancreatic plasmacytomas are an extremely rare condition, with only 50-60 cases previously described in the literature. We report a unique case of concurrent gastric and pancreatic plasmacytomas presenting as acute pancreatitis.

CASE DESCRIPTION/METHODS:
A 62-year-old woman presented to the Emergency Department (ED) for epigastric pain radiating to back for one day. She had a history of multiple myeloma, which had relapsed after autologous stem cell transplant and progressed despite chemotherapy. On exam, she was jaundiced and had an elevated lipase to 1,112 U/L. A computed tomography scan of abdomen showed two masses involving the pancreatic head and body, with biliary and pancreatic duct dilatation. A subsequent endoscopic ultrasound revealed multiple nodules in the stomach. Biopsy of the gastric nodules and fine needle aspiration of the pancreatic lesions revealed atypical plasma cells with immunoreaction to kappa light chain, CD138, and CD56. These findings confirmed the diagnosis of extramedullary plasmacytoma. Acute pancreatitis and obstructive jaundice were diagnosed and an endoscopic retrograde cholangiopancreatography (ERCP) was performed. During the ERCP, a distal bile duct stricture was found and one metal stent was placed. Following the procedure, the patient reported gradual resolution of her abdominal pain and serum bilirubin returned to the normal range within one week.

DISCUSSION:
In previous reports, the majority of pancreatic plasmacytoma cases involved a single lesion located in pancreatic head which presented as painless, obstructive jaundice. We report a case of concurrent gastric and pancreatic plasmacytomas presenting with acute pancreatitis. It is important to recognize the extramedullary manifestations of multiple myeloma in patients presenting with acute pancreatitis and gastrointestinal tract lesions. This observation of aggressive tumor behavior in treatment-resistant multiple myeloma also suggests a potential modulation effect on the tumor and its microenvironment by chemotherapy and stem cell transplant. Further investigation into the underlying mechanism of metastasis in multiple myeloma may shed light on new therapeutic strategies to improve outcomes for future patients.
1357 Pseudocirrhosis in Subacute/Chronic Budd-Chiari Syndrome With JAK 2 Mutation
Authors: Lakshmanan Seetha L. , Toubia Nabil 
Published: October 2019

INTRODUCTION:
Budd Chiari Syndrome (BCS) is a rare disorder often characterized by hepatic venous outflow obstruction. Myeloproliferative disorders especially V617F-JAK2 mutations account for almost 40% of these patients. BCS may present differently depending on location, extent, severity of obstruction and existence of collaterals. The course of disease may be fulminant, acute, subacute or chronic, ranging from incidental findings to fatal acute hepatic failure.

CASE DESCRIPTION/METHODS:
A 74-year-old female with remote history of alcoholism, presented to our facility with bleeding per rectum for 2 days. Labs on admission revealed WBC 18.4, HGB 16.8, PLT 588, AST 570, ALT 804 and TB 4.6. Ultrasound Abdomen with Doppler revealed ascites and portal vein thrombosis. Follow-up MRI Abdomen confirmed thrombus in main portal vein and non-opacification of major intrahepatic veins suggesting Budd Chiari Syndrome. Hypercoagulability workup showed positive JAK2 mutation. She was started on IV Heparin immediately. Diagnostic paracentesis revealed transudative fluid and liver elastography confirmed cirrhosis. Grade 2 esophageal varices were seen on EGD with a negative colonoscopy. Other possible etiology of congestive hepatopathy/cirrhosis were excluded. Patient was discharged on daily oral Rivaroxaban with intermittent therapeutic paracentesis. Over next 3 months, she had recurrent admissions for decompensated liver disease. During her last visit however, we noted preserved synthetic liver function and inconsistencies in her presentation thus a liver biopsy was obtained which revealed centrilobular hepatocellular necrosis, sinusoidal dilatation and mild portal fibrosis consistent with hepatic venous outflow obstruction. However, there was no evidence of cirrhosis. She was transferred to an advanced facility for portal vein reconstruction to relieve the obstruction from splanchnic venous thrombosis and possibly reverse the “pseudocirrhosis”.

DISCUSSION:
Pseudocirrhosis is often associated with metastatic cancers in response to chemotherapy induced ischemic injury. Although it radiologically and clinically mimics liver cirrhosis, it can be prevented with early detection. To our knowledge, this is the first reported case of pseudocirrhosis in BCS. Subacute/chronic BCS may present with cirrhotic complications like portal hypertension, ascites and variceal bleeding. Apart from anticoagulation, these patients may require other interventions to bypass the obstruction like Direct Intrahepatic Portosystemic Shunt (DIPS) placement.
1360 Cholecystohepatic Fistula With Liver Abscess From Gallbladder Perforation in a Patient With Ascending Cholangitis
Authors: Lu Ray , Smock Justin W. 
Published: October 2019

INTRODUCTION:
Ascending cholangitis can lead to liver abscess (LA). Intrahepatic gallbladder (GB) perforation with fistula formation is an uncommon cause of LA. The co-occurrence of all 3 phenomena is rare.

CASE DESCRIPTION/METHODS:
An 88-year-old man with a history of idiopathic fibrosing pancreatitis diagnosed 3 months prior that required stenting of the common bile duct (CBD) presented with 4 days of malaise, nausea, vomiting, and fever. Endoscopic retrograde cholangiopancreatography (ERCP) showed occluded CBD stent coated with stone material. Successful biliary sphincterotomy and stone extraction was performed. The patient's symptoms initially resolved but recurred later that day. On exam he was tachycardic, tachypneic, and febrile to 103.1 F, with pain in the right upper quadrant (RUQ) of the abdomen without guarding nor rebound. Labs showed WBC 33.4 k/mm3, total bilirubin 2.2 mg/dL, ALP 488 U/L, AST 43 U/L, ALT 39 U/L, and lipase 44 U/L. RUQ ultrasound (US) showed a heterogeneously hypoechoic right liver lesion 6.3 × 7.4 × 4.3 cm in size, consistent with LA and noted to be contiguous with the GB, which had a thickened wall, an irregular sloughing appearance concerning for gangrenous cholecystitis, and contained sludge, gallstones, and air. A percutaneous cholecystostomy drain was placed by interventional radiology (IR). Intra-procedure sinogram and US showed the LA to be connected to the GB. A second percutaneous drain was placed into the LA. Abscess cultures were polymicrobial. The patient was treated with 4 weeks of piperacillin-tazobactam, and then transitioned to amoxicillin-clavulanic acid with plans to continue antibiotic therapy for a total of 6 months. One month after discharge, computed tomography (CT) showed resolution of LA, and both IR drains were removed. Given clinical improvement, cholecystectomy has not been pursued due to advanced age.

DISCUSSION:
When ascending cholangitis and choledocholithiasis are successfully treated through ERCP but the patient does not improve clinically, LA should be considered. Intrahepatic GB perforation leading to fistula formation is a rare cause of LA that most commonly occurs in the elderly, particularly those with a history of chronic biliary disease. Exam and labs can be non-specific, while US and CT are useful diagnostically. Successful treatment involves ERCP, drainage of LA, an extended course of antibiotics, and, while cholecystectomy is preferred, percutaneous cholecystostomy appears to be a viable first option in high-risk patients.
1364 Metastatic Pancreatic Adenocarcinoma Presenting as a Colonic Obstruction
Authors: King Connor , Nehme Fredy , Tawfik Ossama 
Published: October 2019

INTRODUCTION:
Pancreatic cancer is the fourth most common cause of cancer-related mortality in the United States largely due to a delayed diagnosis at an advanced stage. Patients with pancreatic tumors commonly present with abdominal pain, anorexia and jaundice. Nearly 50% of patients with pancreatic cancer present with metastatic disease, most commonly to the liver. Rarely it can metastasize to the colon and present with an obstruction.

CASE DESCRIPTION/METHODS:
A 68-year-old male presented with a 2 week history of abdominal pain and constipation. On physical exam, the abdomen was distended with generalized tenderness. Initial computed tomography (CT) scan of the abdomen and pelvis showed abnormal colonic and small bowel distention with induration surrounding the proximal sigmoid colon and distal decompression. There was also evidence of a bulky mass-like appearance of the pancreatic tail and prominent pericolonic lymph nodes surrounding the sigmoid colon. Despite conservative measures, the patient had progressively worsening colonic distention on serial abdominal X-rays and a repeat CT scan was concerning for perforated viscus. The patient underwent emergent exploratory laparotomy and a large inflammatory mass was noted in the sigmoid colon. A subtotal colectomy with end ileostomy was performed. Surgical pathology revealed metastatic mucinous adenocarcinoma to the colonic wall (Figure 1). Several days later, an endoscopic ultrasound with fine needle aspiration was obtained and showing a 31 × 20 mm hypoechoic mass involving the body and tail of the pancreas (Figure 2). Pathology was positive for malignant cells consistent with adenocarcinoma. Histopathological patterns of both tumors were compared and had almost identical features.

DISCUSSION:
Differentiating a primary colonic cancer from metastasis can be challenging and the absence of mucosal involvement favor metastatic disease. In cases where metastasis are favored, immunohistochemistry is helpful to determine the primary origin of the tumor. It is not uncommon to encounter an extrapancreatic adenocarcinoma suspected to originate from the pancreas, with most common locations being liver, lymph nodes, lungs, and adrenal glands. Metastatic pancreatic cancer to the colon is extremely rare and no clear mechanism has been identified. Despite its rarity, pancreatic cancer should be considered in patients presenting with colonic obstruction and a pancreatic mass on imaging.