bluuebunny/crossref_metadata_2025 huggingface dataset:
1422 Pancreatogenic Diabetes (Type 3C) Presenting With Diabetic Ketoacidosis
Authors: Melki Gabriel , Laham Linda , Karim Gres , Komal Fnu , Barham Shaker , Hanna Maggie , Yuridullah Ruhin , Vora Aarohi , Grossman Matthew , Baddoura Walid
Published: October 2019

INTRODUCTION:

Diabetes type 3c (DM3c) accounts for 5% of diabetics in the US. It is due to chronic pancreatitis leading to the destruction of islet cells. Glucagon and insulin levels are both decreased due to destruction of alpha and beta cells, respectively. This makes the development of diabetic ketoacidosis (DKA) a rare occurance in these patients, as glucagon is one of the key hormones in the production of ketone bodies. We report a case of Type 3C diabetes presenting with DKA.

CASE DESCRIPTION/METHODS:

A 50-year-old male with chronic alcoholic pancreatitis s/p pseudocyst drainage presented to the ED complaining of epigastric pain for two months. The patient was on oxycodone for pain control and pancreatic enzymes. V/S: BP 100/92, HR 94, RR 16, and O2 sat 97%. Physical exam was notable for epigastric tenderness. Prior imaging studies revealed diffuse pancreatic calcifications. Cardiac enzymes were normal and lipase level was 156. Blood glucose level was notably elevated at 703. Other lab abnormalities included serum bicarbonate of 16, ketones in the urine and acetone in the blood, anion gap of 27 and HbA1C of 16% (prior values were normal). The patient was admitted to the medical ICU for management of DKA. He was started on IV fluids and IV insulin. An autoimmune workup for diabetes was negative. Once anion gap closed, patient was transitioned to SC insulin and subsequently discharged.

DISCUSSION:

DM3c involves inflammation of the pancreatic islet cells with irreversible fibrotic changes, due to the underlying chronic pancreatitis. Early on, the beta cells and the pancreatic polypeptide cells are affected, but as the disease progresses, glucagon secreting alpha cells are destroyed, making it rare for these patients to present in DKA. However, an upregulation of cortisol, growth hormone and catecholamines make ketone production possible. Due to exocrine pancreatic deficiency, incretin secretory function is lost; thus pancreatic enzyme supplementation can be beneficial for glycemic control. Diagnosing DM3c requires imaging evidence of chronic pancreatitis, pancreatic exocrine insufficiency, glucose intolerance and absence of type 1 DM autoimmune markers. Recognition of DM3c is essential for optimal management of these patients.
1424 Follicular Cholangitis Presenting as Cholangiocarcinoma in a Patient With Serologic Evidence of Autoimmune Disease
Authors: Kleesattel David , Safdar Kamran , Shah Shimul , Wang Jiang , Bari Khurram
Published: October 2019

INTRODUCTION:

Follicular cholangitis is a rare sclerosing cholangitis of the biliary hilum that is difficult to distinguish from primary sclerosing cholangitis, immunoglobulin G4-related sclerosing cholangitis, and most imperatively, hilar cholangiocarcinoma. Pathological findings include fibrotic thickening of bile duct walls containing lymph follicles with germinal center formation. While autoimmune evaluations have previously been negative, we herein report the first case of follicular cholangitis found in the pretense of autoimmune hepatitis.

CASE DESCRIPTION/METHODS:

A 40-year-old female presented following a 2-month history of pruritis, fatigue, and elevation of hepatobiliary enzymes (AST 113 U/L, ALT132 U/L, ALP 499 U/L). Medical history was significant for cholestasis of pregnancy 10 years ago. Laboratory evaluation was positive for an ANA (1:160), an atypical pANCA (1:320), and an elevated F-actin (101 units) while negative for anti-mitochondrial antibody. Serum IgG levels were normal (1250 mg/dl; IgG4 subclass <1). IgM level was elevated at 350 mg/dl. Liver biopsy showed dense portal lymphatic inflammation, including focal germinal center formation as well as paucity of bile ducts without florid ductal lesion or fibrosis. Initial treatment with glucocorticoids, azathioprine and ursodiol did not improve symptoms or liver chemistries. ERCP revealed a tight stricture with direct visualization (spyglass) of a biliary lesion in the distal third of the common bile duct (CBD). Cytology from the stricture returned atypical cells suspicious for adenocarcinoma. Bile duct biopsy revealed severe chronic inflammation and few atypical glands. IgG4 immunostaining was negative. She underwent pancreaticoduodenectomy due to concern for cholangiocarcinoma. Macroscopically, the CBD mucosa was diffusely granular and without mass lesions. Microscopically; bile duct fibrosis, lymphoid hyperplasia with prominent germinal centers, and rare IgG4-positive cells were consistent with severe follicular cholangitis.

DISCUSSION:

Unique to this case is the serological backdrop and histologic findings of autoimmune disease. Whereas previous cases have been strictly ANA negative, there appears to be an unexplored continuum of disease as evidenced by cases of follicular bronchiolitis which have been associated with autoimmune disease and have similar immunohistologic findings. Although the etiology remains unclear, the existence of an autoimmune association raises important considerations for medical therapies.
1426 Type 1 Choledochal Cyst With Anomalous Pancreaticobiliary Malunion Diagnosed on Cholangioscopy
Authors: Ali Mohammed Fahad , El Hage Chehade Nabil , Sowa Piotr , Clary Meredith , Samarasena Jason
Published: October 2019

INTRODUCTION:

Choledochal cysts are cystic dilatations found within the intra and/or extrahepatic bile ducts. Untreated choledochal cysts can lead to increased risk for strictures, pancreatitis, cholangitis, gallbladder cancer, cholangiocarcinoma and pancreatic cancer. Early and accurate identification of choledochal cysts is crucial to determine appropriate medical and surgical planning. We present a case of a patient with a type 1 choledochal cyst with anomalous pancreaticobiliary malunion.

CASE DESCRIPTION/METHODS:

A 42-year-old female with a PMH of a cholecystectomy and known biliary cyst presented with acute onset abdominal pain. MRCP demonstrated a 5.5 cm fusiform dilatation of the common bile duct (CBD). EUS confirmed a CBD dilatation (Figure 1). ERCP with spyglass cholangioscopy showed an abnormal pancreaticobiliary junction with a common channel about 1 cm long leading to two separate openings, one to the CBD/choledochal cyst and another to the pancreatic duct orifice (Figure 2). Fluoroscopy showed contrast filling a dilated cystic structure in the proximal CBD consistent with a choledochal cyst (Figure 3). A 7 French × 7 cm flex stent was deployed into the CBD. Findings were consistent with a type 1 choledochal cyst and an anomalous pancreaticobiliary malunion. Patient underwent ex-lap with resection of the choledochal cyst with roux-en-y hepaticojejunostomy creation, which she tolerated well.

DISCUSSION:

The Todani classification is based on cyst location and bile duct dilatation. There are 5 types with type 1 as the most common which comprises of 80-90% of all choledochal cysts. Type 1 choledochal cysts involve fusiform dilatation of the entire CBD without any intrahepatic dilatation. Cancer is more common in patients who are older and with type I and IV cysts. These are typically treated with complete cyst resection and Roux-en-Y hepaticojejunostomy. Additionally, 50-80% of patients with choledochal cysts have an anomalous pancreaticobiliary malunion. The KOMI classification is used to classify anomalous pancreaticobiliary malunions into 3 types, which is based on the CBD/pancreatic duct angle and presence of CBD dilation. This case was a KOMI type IIB anomalous pancreaticobiliary malunion given the common hepatic and pancreatic ducts join each other at an acute angle creating a single common channel.
1428 Biliary Obstruction Due to Extrahepatic Biliary Mucinous Cystic Neoplasm: An Uncommon Case
Authors: Hawksworth Sara J. , Pluskota Christopher , Confer Bradley , Sarami Iman , Li Jinhong
Published: October 2019

INTRODUCTION:

Biliary mucinous cystic neoplasms (BMCNs) are a rare form of premalignant biliary cystic tumors, much like their better-known counterpart, biliary intraductal papillary mucinous neoplasms (IPMNs). BMCNs most commonly occur in females during the 5th decade of life. They account for approximately 1-5% of all liver cysts. The lack of awareness of these lesions poses a challenge for proper and timely diagnosis. Here, we present an interesting case of extrahepatic BMCN.

CASE DESCRIPTION/METHODS:

A 30-year-old female presented with pruritis, dark urine, and light-colored stools. Labs revealed transaminitis and elevated bilirubin concerning for biliary obstruction. Abdominal ultrasound showed cholelithiasis and dilation of the distal common bile duct (CBD) concerning for choledocholithiasis. MRCP was performed and described a 2.2 × 2.4 cm cystic dilatation of the extrahepatic bile duct involving the distal common hepatic duct (CHD) concerning for either type 1B or type II choledochal cyst. Endoscopic ultrasound revealed a CHD cystic lesion with internal debris concerning for either sludge or biliary IPMN (Figure 1). ERCP with cholangioscopy revealed papillary projection in the CHD (Figure 2). Directed biopsies revealed mucinous epithelium with cytologic atypia. The patient was referred for complete resection of the CBD and CHD, cholecystectomy, and Roux-en-Y hepaticojejunostomy. Surgical pathology revealed a tumor consisting of a cystic structure lined by mucinous epithelium with estrogen receptor (ER) positive ovarian-type stroma, and mild to focal moderate dysplasia, consistent with noninvasive extrahepatic BMCN (Figure 3).

DISCUSSION:

BMCNs are often detected incidentally when investigating other common causes of biliary disease. The overall incidence of BMCNs is approximately 1:20,000 to 1:100,000. They are most commonly found within the liver (90%), are rarely extra-hepatic, and they seldom present with obstructive jaundice. The treatment of choice is surgical resection. Diagnosis is made via histological confirmation of ovarian-type stroma. The location of the lesion, in this case, is more common for IPMNs. Both IPMNs and BMCNs carry malignant potential requiring complete resection for diagnosis and treatment. With the risk of malignant transformation as high as 30%, an awareness of these rare neoplasms is very important.
1431 A Bad Connection: Chronic Pancreatitis Leading to a Pancreticopleural Fistula
Authors: Humes Ross , Sadowski Brett , McCarthy John , Young Patrick
Published: October 2019

INTRODUCTION:

Pancreatic-pleural/mediastinal fistulas are a rare complication of chronic pancreatitis. Fistulous connections occur through fenestrations in the diaphragm. Pancreatic ductal stenting can be valuable, though medical and surgical options may still be required. We describe a case of pancreatic-pleural fistula with mediastinal tracking successfully treated with combined endoscopic and medical management.

CASE DESCRIPTION/METHODS:

The patient is a 53-year-old male with a history of chronic pancreatitis secondary to alcohol abuse, a pancreatic pseudocyst, and compensated heart failure. The patient was admitted with new onset shortness of breath and epigastric abdominal pain. CT showed acute on chronic pancreatitis with a pseudocyst in the uncinate process, lower mediastinal inflammatory changes, and bilateral pleural effusions. Thoracentesis was performed, demonstrating an amylase of >7500. Though MRCP did not show a discrete connection, the clinical presentation was consistent with pancreatic-pleural fistula. ERCP was performed, but the endoscopist was unable to cannulate the pancreatic duct. Patient was placed on octreotide and continued to undergo therapeutic thoracenteses as needed. Patient returned 1 month later with complaints of neck fullness, continued shortness of breath, and abdominal pain. CT was significant for a fluid collection extending from pancreatic head to the mediastinum tracking along the anterior aspect of the esophagus and persistent pleural effusions. MRCP was performed and revealed a fistulous connection between the pseudocyst, pleural space, and mediastinum. Repeat ERCP was done with successful pancreatic stent placement. Patient subsequently had resolution of both mediastinal and pleural fluid collections. The pancreatic stent was removed with durable resolution of fluid collections.

DISCUSSION:

Pancreatic fistulas are reported to resolve with supportive care alone in 80% of external and 50-65% of internal fistulas. In our case, supportive care including octreotide use was insufficient for fistula resolution. The patient was ultimately treated successfully with pancreatic stenting. Had this failed, alternative approaches could have included nasojejunal feeding, endoscopic sealants, or surgical correction. This case highlights a rare, but serious complication of chronic pancreatitis and management options to be considered.
1437 Overlap Syndrome of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis: A Case Report
Authors: Shah Shivani , To Uyen , Silveira Marina
Published: October 2019

INTRODUCTION:

Primary sclerosing cholangitis (PSC) is characterized by inflammatory changes within the extra-and intrahepatic bile ducts and subsequent development of strictures. Primary biliary cholangitis (PBC) is characterized by progressive destruction of intrahepatic bile ducts, typically associated with a positive anti-mitochondrial antibody. Both of these disease processes are rare, chronic cholestatic liver diseases, with possible outcomes of progression to cirrhosis and need for liver transplant.

CASE DESCRIPTION/METHODS:

A 55-year-old gentleman from West Africa with no significant past medical history presented with intractable pruritus, weight loss, polyuria and polydipsia, found to have new onset diabetes. His other laboratory data revealed cholestasis, with alkaline phosphatase 401 U/L, aspartate aminotransferase 100 U/L, alanine aminotransferase 160 U/L, direct bilirubin 0.37 mg/dL on laboratory examination. Magnetic resonance imaging of his abdomen was unremarkable for any biliary dilatation or etiology of his cholestatic liver injury. Liver biopsy was remarkable for non-specific lymphocytic portal inflammation. Other diagnostic work up was remarkable for negative autoimmune diabetes work up. He was subsequently lost to follow up and re-presented five years later with intractable pruritus. Repeat MRI of his abdomen revealed a beaded and irregular appearance of the extra and intrahepatic bile ducts, concerning for PSC (Figure 1). He was started on cholestyramine with minimal improvement in pruritus. Repeat liver biopsy revealed focal bile duct injury. Laboratory examination revealed an elevation of anti-mitochondrial antibody (AMA). These findings were consistent with a diagnosis of overlap syndrome of PBC and PSC. Treatment with ursodeoxycholic acid (UDCA) was started; however, given lack of improvement in biochemical profile, fenofibrate was added. The patient was subsequently hospitalized for rhabdomyolysis with acute kidney injury in setting of fenofibrate use. The patient developed cirrhosis and subsequent decompensation of hepatic encephalopathy and ascites approximately twenty months after his initial diagnosis of overlap syndrome. He is currently undergoing transplant evaluation.

DISCUSSION:

This is a rare case of overlap syndrome of PBC and PSC. At present, there are no consensus guidelines on the diagnosis and management of this syndrome. Given the rarity of this condition, there is currently no available data on long term outcomes in patients with overlap of PBC and PSC.
1440 An Uncommon Cause of Biliary Obstruction in a Patient With Budd-Chiari Syndrome
Authors: Mago Sheena , Mavilia Marianna
Published: October 2019

INTRODUCTION:

Biliary varices (BVs) are an infrequent complication of chronic portal hypertension (PHTN). PHTN causes collateral paracholedochal veins to dilate and protrude into the common bile duct (CBD), forming varices. Most cases of BVs are asymptomatic and are therefore likely underdiagnosed. However, protrusion of BVs into the CBD can lead to clinically significant biliary obstruction.

CASE DESCRIPTION/METHODS:

A 34-year-old female with polycythemia vera and Budd-Chairi syndrome status-post portocaval shunt on chronic warfarin presented with a 1-day history of right upper quadrant pain and non-bloody, non-bilious emesis. She was febrile to 39.9 C and had a soft, but distended abdomen without icterus or asterixis. Labs were notable for total bilirubin 4.1 mg/dL, direct bilirubin 1.2 mg/dL, AST 79 U/L, ALT 69 U/L, INR 1.8, and alkaline phosphatase of 207 U/L. Abdominal ultrasound with Doppler demonstrated a patent portacaval shunt, splenomegaly, and stable prominent portosystemic varices causing limited visualization of the CBD. ERCP for further visualization showed a filling defect of the middle third of the CBD. A biliary stent was deployed, causing hemorrhage within the CBD, raising the possibility of an intraductal varix. Hemostasis was achieved with subsequent metal stent placement. Further investigation with venography displayed multiple infrahepatic IVC collateralization and two regions of significant stenosis along the portal-IVC stent and intrahepatic IVC. This resulted in diversion of blood flow through the azygos system and ultimately giving rise to her CBD varices. Balloons were used for venoplasty resulting in successful reduction of the regions of stenosis and improvement of the pressure gradients.

DISCUSSION:

The presence of BVs can complicate surgical and endoscopic procedures of the biliary tract. In this case, endoscopic intubation of the duct during ERCP caused unexpected hemobilia as BVs were not suspected in this patient. Data on management of biliary varices is limited. In our patient, stent placement was performed to both achieve hemostasis and relieve the biliary obstruction. Venography was done to diagnose and treat IVC stenosis. This ultimately improved pressure gradients in the collateral circulation and should prevent progression of BVs. History of PHTN should increase clinical suspicion for BVs in patients presenting with biliary obstruction. If a biliary tract procedure is required, pre-procedure imaging for variceal screening should be considered to prevent complications.
1443 Hypercalcemia as the First Presentation of an Underlying Rare Variant of Adenosquamous Cholangiocarcinoma
Authors: Ramachandran Preethi , Kaur Amandeep , Yadav Ruchi , Joseph Gardith
Published: October 2019

INTRODUCTION:

Humoral hypercalcemia of malignancy (HHM) is an important paraneoplastic syndrome which is caused by the over secretion of the parathyroid hormone-related peptide (PTHrP) from a variety of cancers. Most commonly HHM is reported in squamous cell carcinomas of the lung, head, neck, skin, esophagus, ovarian and breast cancer. HHM due to intrahepatic cholangiocarcinoma is a rare association and is associated with poor prognosis.

CASE DESCRIPTION/METHODS:

We herein report a case of hypercalcemia presenting as the first presentation of an underlying rare variant of intrahepatic cholangiocarcinoma. Our patient is a 57-year-old male who presented to the emergency room with severe constipation and polyuria. His physical examination was unremarkable except for hepatomegaly. His laboratory values revealed calcium 16.5 mg/dL, creatinine 1.37 mg/dL, ALP of 413 U/L, PTH -2 pg/mL, PTHrP 82 pg/mL. Computed tomography (CT) of the abdomen revealed a 15 cm liver mass with possible satellite lesions. The patient was subsequently treated for symptomatic hypercalcemia with aggressive hydration, zoledronic acid, and calcitonin. Biopsy of the liver lesion revealed poorly differentiated carcinoma with squamous features positive for Immunohistochemical markers CEA, CK19, P63, CK5/6, CK7 which favored pancreaticobiliary malignancy as the primary. Further staging scan with positron emission tomography (PET) revealed extensive hypermetabolic activity in the liver, enlarged upper abdominal lymph nodes, nodularity of the omentum/peritoneum and ascites inferior to the liver. Within a week into the presentation, his bilirubin started to increase, needing emergency percutaneous transhepatic cholangiography (PTC). His repeat CT scans showed progressively increasing tumor size to 19 cm with an associated increase in satellite lesions. This necessitated an urgent inpatient treatment with gemcitabine/cisplatin combination chemotherapy to control the aggressive nature of the malignancy.

DISCUSSION:

Adenosquamous cholangiocarcinoma is a very rare variant accounting for <2% of all liver tumors. It develops due to squamous metaplasia of an underlying cholangiocarcinoma and usually has aggressive clinicopathological features. HMM is a life-threatening yet unrecognized phenomenon of cholangiocarcinoma which represents a poor prognostic marker. Prompt recognition of this complication is important for preventing serious complications associated with hypercalcemia and to improve the quality of life of these patients.
1447 A Rare but Fatal Case of N-methyl-D-aspartate Receptor Antibody Encephalitis Associated With a Pancreatic Neuroendocrine Tumor
Authors: Shim Hong Gi , Lee Raymond , Yacavone Robert F.
Published: October 2019

INTRODUCTION:

N-methyl-D-aspartate receptor antibody (NMDAR-Ab) encephalitis is an autoimmune inflammatory condition of the brain thought to be paraneoplastic given its strong association with malignancy, but such related tumors are rarely detected in older males. To our knowledge, there is only a single case report of NMDAR-Ab encephalitis related to a pancreatic neuroendocrine tumor (NET). Here, we report a case of NMDAR-Ab encephalitis associated with pancreatic NET that was fatal despite early tumor resection.

CASE DESCRIPTION/METHODS:

A 79-year-old man with hypertension presented with acute onset of confusion, agitation, and urinary and bowel incontinence. He was living independently but had progressive short-term memory loss over the year prior. Electroencephalogram showed diffuse slowing without seizure activity. Magnetic resonance imaging of the brain noted trace sulcal FLAIR hyperintensity in perirolandic regions. Cerebrospinal fluid (CSF) analysis revealed elevated protein (96 mg/dL), low nucleated cell count, no oligoclonal bands, and negative infectious serology and cultures. CSF paraneoplastic panel was positive for anti-NR1 antibody, confirming the diagnosis of NMDAR-Ab encephalitis. Malignancy workup revealed a 1.2 cm mass in the pancreatic tail, and endoscopic ultrasound-guided fine-needle biopsy showed positive staining for synaptophysin and chromogranin A consistent with a well-differentiated NET. The patient rapidly declined from worsening encephalopathy and required percutaneous gastrostomy tube placement. He underwent laparoscopic distal pancreatectomy and splenectomy with intravenous immunoglobulin and glucocorticoids, but a repeat CT abdomen next month showed peritoneal metastases with diffuse uptake on somatostatin receptor scintigraphy and rising serum chromogranin A (603 ng/mL). He had no significant neurologic recovery and eventually died on comfort measures without further interventions.

DISCUSSION:

Though rare, prior cases of NMDAR-Ab encephalitis associated with NET described the importance of early surgical removal of the underlying tumor to provide the best chance of neurologic recovery. Metastatic disease portends a poor prognosis as it renders encephalitis impossible to resolve. Worldwide experience with NMDAR-Ab encephalitis is limited and further observations are needed to refine current treatment strategies.
1450 Acute Ischemic Pancreatitis Secondary to Superior Mesenteric Artery Stenosis: A Common Diagnosis Due to an Uncommon Etiology
Authors: Bajwa Maria , Bella Saif , Bank Leslie
Published: October 2019

INTRODUCTION:

Pancreatic ischemia is a rare cause for acute pancreatitis which is poorly defined without specific radiological appearance.

CASE DESCRIPTION/METHODS:

We present a case of 57-year-female with 2-week history of abdominal pain. Her history included CHF, carotid stenosis, hypertension, COPD, and invasive Aspergillosis. She underwent cardiac catheterization 6 years ago which showed heavy atherosclerosis without obstruction. Her post procedure course was complicated by cholesterol embolization to left lower extremity. She was a heavy smoker with 1-2 PPD smoking but denied alcohol use. She was diagnosed with invasive Aspergillosis 2 months ago and initially treated with Voriconazole. It was switched to isavuconazole 1 month ago after developing blurring of vision. Patient reported decreased appetite since then with poor oral intake. She presented with 2 week h/o severe abdominal pain, associated with intense nausea and multiple episodes of vomiting. Her initial work up showed leukocytosis and elevated lipase >4000. CT abdomen showed diffuse, homogenous pancreatic enlargement with prominent aortic atherosclerosis and distal aortic aneurysm measuring 3.1 cm. Initially, the antifungal agent, isavuconazole was thought to be the culprit, causing pancreatitis but literature review was negative. IGg4 levels and lipid profile were unrevealing. Pancreatic ischemia was considered as the likely etiology. An abdominal duplex showed superior mesenteric artery stenosis. However, given h/o cholesterol embolization following cardiac catheterization, she was considered high risk for endo-vascular procedure. High intensity statin therapy was prescribed and she was discharged home with close outpatient follow up. Patient was strongly encouraged to quit smoking, as this would likely decrease her risk of major adverse cardiac event.

DISCUSSION:

Three well-identified causes of acute ischemic pancreatitis include hemorrhage, hypotension, & mesenteric vessel occlusion. Pancreatic ischemia leads to appearance of oxygen-derived free radicals which cause endothelial dysfunction, resulting in increased permeability and localized inflammatory response. Our patient had heavy calcified atherosclerosis. She was an active smoker. Dehydration also played a role in the development of ischemic pancreatitis, as the patient endorsed poor oral intake, confounded by nausea, vomiting and diarrhea. Treatment of acute ischemic pancreatitis consists of supportive care with IV fluids and pain control and restoration of pancreatic perfusion.
1455 Double Trouble: A Rare Case of Colonic Mixed Adenoneuroendocrine Carcinoma (MANEC)
Authors: Averbukh Leon D. , Forouhar Faripour , Vaziri Haleh
Published: October 2019

INTRODUCTION:

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare malignancy involving both epithelial and neuroendocrine components and accounts for fewer than 2% of all colorectal cancers. We present the case of a 34 yo male whose persistent weight loss and cramping abdominal pain led to the discovery of Stage IV MANEC.

CASE DESCRIPTION/METHODS:

34 yo male with no significant past medical history presented with the chief complaint of diffuse abdominal cramps over the preceding 3 days. He had intermittent, non-bloody diarrhea, and endorsed 25-30 lb unintentional weight loss in the preceding 4 months. Labs were significant for a WBC of 17.3 U/L, CRP 94.4 mg/L, and sedimentation rate of 60 mm/hr. CT abdomen and pelvis was concerning for underlying inflammatory bowel disease. Before further work up, the patient left against medical advice. He returned 2 months later due to persistent symptoms. Gastrografin study and colonoscopy showed a circumferential, partially obstructing, ulcerated mass in the sigmoid colon (Figure 1). Biopsy of the lesion showed moderately differentiated adenocarcinoma. He underwent exploratory laparotomy, sigmoidectomy, and mesenteric lymph node dissection with pathology consistent for mixed adenoneuroendocine carcinoma with a high grade aggressive neuroendocrine component of the sigmoid with involvement of small bowel, bladder serosa, and lymph nodes (Figure 2). The metastatic component was exclusively high-grade neuroendocrine tumor. The patient subsequently underwent complete resection of primary and metastatic lesions and was started on Cisplatin and Etoposide for adjuvant chemotherapy.

DISCUSSION:

MANEC is a mixed tumor containing adenocarcinoma and neuroendocrine components each representing at least 30% of the tumor. It is hypothesized that the tumor arises from multi-potent endodermal progenitor cells with bi-directional differentiation into both epithelial and neuroendocrine neoplasms. Outcomes depend on each malignant component and the type of neuroendocrine cells present. Due to its neuroendocrine component, MANEC is more aggressive and advanced at the time of diagnosis with poorer outcomes as compared to adenocarcinoma. While no standardized treatment regimen has been identified for MANEC, currently accepted therapy involves complete resection of the primary and metastatic lesions with individually tailored adjuvant chemotherapy to help reduce its high recurrence rate.
1457 Cocaine-Induced Colonic Ischemia
Authors: Abdo Michael , Kozacek Kyler S. , Oroian Tudor , Mank Victoria M. , Hudspath Caleb , Manibusan Pedro
Published: October 2019

INTRODUCTION:

Cocaine use is associated with arterial vasoconstriction and enhanced thrombus formation. Rarely, these effects on the intestinal blood supply result in intestinal ischemia. Cocaine-induced ischemic colitis is a relatively poorly-defined variant of this phenomenon, but some studies suggest it may be associated with higher morbidity and mortality than other etiologies. Therefore, timing is critical in both diagnosis and initiating treatments to prevent poor outcomes. We present a case of a middle-aged patient who had an unusual presentation for ischemic colitis due to cocaine abuse.

CASE DESCRIPTION/METHODS:

A 49-year-old male with a history remarkable only for alcohol and cocaine use disorders presented with a two day history of hematemesis with syncope and severe abdominal pain. Urine drug screen was positive for cocaine on admission, and initial workup with EGD was grossly unremarkable. An abdominal CT scan showed extensive circumferential submucosal fat but without colonic wall thickening or other signs of acute process, and a mesenteric angiography was similarly unrevealing. However, his clinical condition continued to deteriorate over the next 48 hours with worsening abdominal pain, and he began passing large melanic stools with occasional frank red blood. He subsequently became septic; due to concern for ischemic bowel, a colonoscopy was emergently performed. Diffuse severe ischemic changes with deep cratered ulcers in the descending, transverse, and ascending colon were noted. Emergent laparotomy was performed for a near-total colectomy, after which the patient began to stabilize.

DISCUSSION:

Long-term cocaine abuse is associated with multiple vascular morbidities, including bowel ischemia. Our patient presented in extremis due to bowel ischemia of both SMA and IMA territories requiring intensive care and emergent colectomy. Interestingly, despite the widespread vascular compromise, initial symptoms were nonspecific without blood per rectum and imaging was unremarkable, including CT and angiography. This illustrates the importance of maintaining clinical suspicion and the utility of colonoscopy in diagnosis, as his unusually diffuse disease eventually necessitated a colectomy in this middle-aged man with otherwise unremarkable medical history.
1463 Underwater Cap-Assisted Endoscopic Diagnosis and Treatment of Anal Intraepithelial Neoplasia
Authors: Ali Mohammed Fahad , Brennan Gregory T. , El Hage Chehade Nabil , Samarasena Jason
Published: October 2019

INTRODUCTION:

Anal intraepithelial neoplasia (AIN) is a premalignant lesion which can progress to squamous cell carcinoma of the anus. Although rare, therate of anal canal cancer is rising within the United States and is associated with significant morbidity and mortality. Early detection of premalignant lesions is critical. AIN is frequently missed because detailed examination of the anal canal is difficult. Here we present two cases describing techniques to aid in the diagnosis and resection of AIN including endomicroscopy, distal attachment caps, and underwater endoscopy.

CASE DESCRIPTION/METHODS:

Patient 1 A 78-year-old woman with a history of HPV and AIN presented for follow up. A flexible sigmoidoscopy (FS) with a distal attachment cap was used to examine the anal canal and revealed two verrucous polypoid-appearing lesions just distal to the dentate line. Probe based confocal endomicroscopy of these lesions showed abnormal cellular structure and capillaries. The lesions were completely resected using a combination of biopsy forceps and cold snare. Both lesions were consistent with high-grade squamous AIN II-III. Repeat flexible sigmoidoscopy 6 months later showed no evidence of recurrence. Patient 2 A 63-year-old woman with a history of AIN with high grade dysplasia was referred to our center for resection. FS with a ESD cap attachment and water-immersion revealed a 5 mm villiform lesion (Figure 1). Using water-immersion the lesion appeared to float up off the mucosa. Probe based confocal endomicroscopy showed highly vascularized abnormal cellular architecture. The lesion was removed with cold forceps and pathology was consistent with low grade squamous AIN I. A follow up FS 1 year later showed a lesion consistent with AIN using narrow band imaging and near focus (Figure 2 and 3). The lesion was removed in piecemeal fashion with cold forceps underwater. Pathology again was consistent with low grade squamous AIN 1.

DISCUSSION:

Here we described techniques to improve identification and resection of AIN. Our approach is to use an ESD cap which helps stabilize the scope tip in the anal canal and spread mucosal folds to flatten areas of interest. Confocal probe based endomicroscopy has been described to take “optical biopsies.” Narrow band imaging and near focus of endoscopic images aids in the identification of lesions. Finally, water-immersion technique frequently causes AIN lesions to float up off the mucosa which aids in identification and resection.
1466 Diverticulitis: The Rare and Unanticipated Complication of Colonoscopy
Authors: Khan Zarak H. , Korpole Pranay R. , O'Brien John
Published: October 2019

INTRODUCTION:

Colonoscopy is a common therapeutic and diagnostic tool used for colon cancer screening and surveillance. Although considered low risk, the most common complications include hemorrhage, bowel perforation and infection with rates of 1%, 0.1% and 1.1% respectively. Other uncommon complications include splenic injury and bowel obstruction. Diverticulitis is defined as inflammation of a diverticulum and is the result of a small tears in the diverticular wall. The reported incidence of colonoscopy induced diverticulitis differs between 0.08 and 1.9 per 1000 colonoscopies.

CASE DESCRIPTION/METHODS:

64-year-old male with past history of CAD, HLD and diverticulitis presented with severe abdominal pain. 6 hours before presentation, patient had undergone a screening colonoscopy with polypectomy. Patient's abdominal pain was sharp and non-radiating. He denied any associated fevers, diarrhea, nausea or vomiting. Patient however did complain of chills. Upon presentation, patient had a temperature of 102.3 °F and HR of 109. Physical exam revealed diffuse abdominal tenderness. Labs were significant for lactic acid of 3.7. WBC count was 7,900. CT scan findings suggested diverticulitis. Repeat CBC few hours later showed an up-trending WBC count (10,800). Patient was managed with IV fluids and antibiotics. He was initially kept n.p.o. and then diet was gradually advanced which he tolerated well. A day after admission, patient was tolerating oral intake well and pain had resolved. He was discharged in stable condition.

DISCUSSION:

A retrospective cohort studied 236,377 colonoscopies with development of acute colonic diverticulitis in 68 patients within 30 days after colonoscopy. The rate of colonoscopy induced diverticulitis in the cohort was estimated to be 10 times less than the reported rates of other complication. Past history of diverticulitis is considered to be a risk factor for development of diverticulitis. No evidence of diverticulitis specifically after polypectomy was found on literature review. In our patient, we suspect that the micro and/or macro perforations induced by polypectomy led to introduction of bacteria into the diverticular wall and subsequently to the blood stream resulting in both local and systemic manifestations. Moreover, our patient's past history of diverticulitis also increased his risk. Our case is intended to shed light on this rare but potentially serious complication of colonoscopy in patients who develop severe abdominal pain and/or fever following colonoscopy.
1467 Are Those Really Xanthomas?
Authors: Khan Zarak H. , Korpole Pranay R. , Singh Tejveer , Jarodiya Vijay , Khan Tahir , Rai Raveen
Published: October 2019

INTRODUCTION:

GI xanthomas are benign mucosal neoplasms consisting of foamy histiocytes. Xanthomas most frequently involve the stomach and usually spare other parts of the GI tract. Gastric xanthomas are well known as yellow to white well-demarcated plaques generally noted after gastrectomy. There is varying association of hyperlipidemia with GI tract xanthomas. Colorectal xanthomas are a rare entity when compared with gastric and esophageal xanthomas and their true incidence rates are unknown.

CASE DESCRIPTION/METHODS:

An 89-year-old lady with past medical history of short gut syndrome secondary to partial colectomy presented to the hospital with nausea and vomiting. Upon presentation, her vitals were stable. On examination, her abdomen appeared distended. Lab work was notable for sodium of 120. CT scan of the abdomen revealed marked wall thickening of the rectosigmoid colon consistent with colitis. Gastroenterology was consulted for further evaluation. Patient's electrolyte abnormalities were attributed to fluid loss secondary to vomiting. She underwent an EGD which was normal and a sigmoidoscopy which revealed multiple submucosal masses in the rectum and distal sigmoid. Biopsy specimens taken from the rectosigmoid colon revealed xanthomatous mucosal changes without any evidence of malignancy or inflammation. A fasting lipid panel was within normal limits. Patient's vomiting and electrolyte abnormalities resolved with bowel rest and IV fluids. She was able to tolerate advancement in diet and was therefore discharged home with follow up in as an out patient with Gastroenterology. Review of patient's chart revealed four previous visits in the last 3 years to the hospital for nausea, vomiting and abdominal distension. During each visit, imaging was significant for colitis and colonoscopy revealed sigmoid masses proven to be xanthomas on histopathology.

DISCUSSION:

Majority of the cases of colorectal xanthomas are incidental findings on elective colonoscopies and usually don't present with upper or lower GI symptoms. They typically appear as whitish to yellowish plaques or papules. Our case presentation is unusual in that our patient presented with recurrent symptoms and signs of gastrointestinal tract obstruction such as vomiting and abdominal distension and imaging consistent with colitis without other obvious etiologies. Furthermore, colonoscopy revealed submucosal mass-like xanthomas rather than plaques or papules.
1469 Hematochezia in a Young Female: An Early Sign of Serrated Polyposis Syndrome
Authors: Aamar Ali , Aloreidi Khalil , Tsipotis Evangelos , Reddy Madhavi
Published: October 2019

INTRODUCTION:

Hyperplasic polyposis syndrome is characterized by multiple serrated polyps in the colon. It is also known Serrated Polyposis Syndrome (SPS). Serrated polyps can be divided into hyperplastic polyps, sessile serrated polyps and traditional serrated adenomas. It is associated with up to 50% lifetime risk of colorectal cancer. Serrated polyps can be difficult to diagnose due to their flat and sessile nature making it hard to be visualized by the endoscopist. We report a case of a young Caucasian female who presented with hematochezia and she was found to have SPS.

CASE DESCRIPTION/METHODS:

A 29-year-old female presented to the clinic with hematochezia. She did not have any family history of colon cancer in her first-degree relatives. She underwent a diagnostic colonoscopy and multiple polyps were seen. A 1.5 cm flat polyp (Figure 1) was seen in the ascending colon that was removed with endoscopic mucosal resection (EMR). Biopsy suggested hyperplastic polyp. A 3.5 cm flat polyp (hyperplastic) was seen in the ascending colon (Figure 2) that was removed with EMR and 5 clips were placed to prevent delayed bleeding. A 5 mm flat polyp was seen in the transverse colon and a 1 cm polyp was seen in the descending colon. Patient was rescheduled for colonoscopy in 3 months to remove these polyps. On repeat colonoscopy, a 1 cm polyp (hyperplastic) was seen in the ascending colon at previous EMR site. Repeat EMR was performed to remove the polyp. An 8 mm flat polyp (hyperplastic) was seen in the ascending colon that was removed with cold snare polypectomy. A 6 mm flat polyp (tubular adenoma) was seen at the hepatic flexure that was removed with snare cautery polypectomy. A 6 mm polyp (hyperplastic) was seen in sigmoid colon that was removed with cold biopsy polypectomy.

DISCUSSION:

Sessile polyposis syndrome (SPS) is defined as following: (1) At least 5 serrated lesions proximal to sigmoid colon with at least 2 polyps >1 cm in size; (2) One serrated polyp was family history of SPS; (3) >20 serrated polyps in the entire colon. Based on current studies of SPS, some experts believe that patients should undergo regular endoscopic surveillance to prevent malignant progress of polyps removing only proximally located polyps, while others believe all polyps greater than 5 mm should be removed. First-degree relatives of patients with SPS should be screened starting at age 40 or 10 years earlier than age of presentation of SPS in the family member.
1471 Normal Polypoid Tissue After Endoscopic Full Thickness Resection of Colonic Lesions
Authors: Al-Taee Ahmad M. , Alkaade Samer
Published: October 2019

INTRODUCTION:

Endoscopic full thickness resection (EFTR) is a novel endoscopic procedure for management of challenging colorectal lesions. Resection site growths after EFTR are usually concerning for incomplete resection or recurrence of the initial lesion. Here we report two patients who underwent EFTR and had polypoid growth of normal tissue at the site of EFTR on surveillance colonoscopy.

CASE DESCRIPTION/METHODS:

Case 1 A 51-year-old Caucasian male was referred for evaluation of residual sigmoid colon tubulovillous adenoma with focal high grade dysplasia. He has a family history of late onset colorectal cancer in his mother and grandmother. He had no abdominal symptoms and examination was unremarkable. Colonoscopy revealed residual polypoid tissue at the site of a prior attempted polypectomy for which he underwent EFTR. Surveillance colonoscopy 6 months later showed a 15-mm polyp-like growth at the site of prior EFTR that was lifted and resected with a hot snare (Figure 1). Pathology showed benign mesenchymal proliferation. Case 2 A 70 year-old Caucasian female was referred to our clinic for evaluation of an incompletely resected 20-mm transverse colon tubular adenoma. Her father developed colon cancer after the age of 60. She had no abdominal symptoms and examination was unremarkable. Colonoscopy showed a 20-mm transverse colon polyp with overlying scarring. She underwent EFTR and pathology showed tubular adenoma with clear margins. Repeat colonoscopy 6 months later showed a 10-mm polyp-like lesion at the site of previous EFTR that was lifted and resected with a hot snare (Figure 2). Pathology showed normal colonic mucosa.

DISCUSSION:

EFTR is a novel procedure for management of lesions that are not amenable to conventional endoscopic resection methods such as non-lifting lesions, lesions with high probability of malignancy, and lesions located in difficult anatomic locations. It is an attractive alternative to surgical resection or endoscopic submucosal dissection for management of such lesions. Resection site growths after EFTR are usually concerning for recurrence. However, polyp-like growths of normal tissue after EFTR were noted in 2 out of 8 patients who underwent EFTR at our institution. Therefore, EFTR site growths may not always represent recurrence of the initial lesion. Awareness of this possibility can alleviate patient anxiety and reduce cost associated with further investigations and resections.
1473 A Vanishing Cecal Mass
Authors: Newman Krista M. , Shmidt Eugenia , Vercellotti Gregory
Published: October 2019

INTRODUCTION:

Systemic mastocytosis (SM) is a rare disease characterized by infiltration of mast cells in extracutaneous organs. Although gastrointestinal (GI) manifestations such as nausea, vomiting, abdominal pain are observed in up to 60-80% of patients, colon lesions are rarely identified. Here we describe an unusual case of SM that manifested as a colon mass on routine screening colonoscopy.

CASE DESCRIPTION/METHODS:

A 71-year-old, asymptomatic, female with a long history of urticaria pigmentosa was referred to a gastroenterology clinic for evaluation of an eight by twelve centimeter, polypoid mass involving the cecum and ileocecal valve, found on screening colonoscopy. Subsequently, a CT scan noted mucosal hyperenhancement with slight vascular engorgement and colonic wall thickening involving the cecum and proximal ascending colon. Pathology from the initial biopsies revealed dense (>100 per high powered field) infiltration of mast cells that stained positive for CD117 and CD25 cell surface proteins. The initial, large, polypoid, cecal mass was not present on repeat colonoscopy with upper endoscopy. Instead, there was patchy nodular- appearing mucosa in the right colon. Follow-up biopsies of the upper and lower GI tract revealed dense infiltration of mast cells in the duodenum, ileum, ascending colon and transverse colon as well as elevated serum tryptase level (38 ug/L), all findings consistent with GI involvement of SM. Histamine-blocking therapy with ranitidine and cetirizine was commenced.

DISCUSSION:

Systemic mastocytosis results from a sporadic somatic mutation in the KIT gene that encodes a protein implicated in cell growth, division & survival that results in overproduction of mast cells that accumulate in organs. There are four subtypes of SM that range in severity from slow and indolent to rapidly progressive towards organ failure. It is important to recognize and categorize systemic involvement of mastocytosis to aid in prognosis and treatment. Frequently, GI symptoms are mild but can include more serious effects of mast cell infiltration including peptic ulcer disease, malabsorption and non-cirrhotic portal hypertension. Specifically important to gastroenterologists is the recognition of GI involvement of mastocytosis disorders and understanding of appropriate evaluation, including upper and lower endoscopy with random biopsies to identify mast cell infiltration. Although GI symptoms are frequent and result in endoscopic evaluation, there are only rare reports of colon masses.