Tumor lysis syndrome (TLS) is a life-threatening oncologic emergency. It is characterized by massive tumor cell death leading to metabolic derangements and multiple organ failure. It is a rare complication of hepatocellular carcinoma (HCC) with only a few cases have been reported in the literature to date. We collected and summarized published case reports of tumor lysis syndrome in patients with HCC. We also reported one additional case who developed TLS after sorafenib therapy and wrote a clinical vignette. A comprehensive and current search for relevant articles was conducted in Medline and EMbase through May 2018. A systematic review was performed following the guideline of Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). A total of 28 cases of TLS associated with HCC were enrolled in our review. The median age of included cases was 55.5 years with a male to female ratio of 25:3. The two most common attributed factors of TLS were transcatheter arterial chemoembolization (TACE) (12 cases, 42.9 %) and sorafenib (nine cases, 32.1%). Among enrolled cases, the diameter of the largest tumor was 12 cm. Regarding Barcelona Clinic Liver Cancer (BCLC) staging, seven cases were at least stage A (22.6%), 11 cases were at least stage B (35.5%), and 10 cases were at least stage C (32.3%). The median time of onset of TLS was three days. As for uric acid-lowering agents, nine cases (32.1%) used allopurinol and four cases (14.3%) used rasburicase. Ten cases (35.7%) did not specify the medication prescribed. The overall mortality rate of this cohort was 67.9%. Compared with patients developing TLS following TACE, patients who had TLS following sorafenib therapy had a later onset of TLS (two days versus seven days, p < 0.001) and a more advanced stage of HCC (p = 0.002). There was a trend toward increased mortality of patients in the sorafenib group in comparison with those in the TACE group (77.8% versus 41.7%, p = 0.18). The results of this current review suggest that TLS rarely occurs in HCC but carries significantly higher mortality compared to TLS occurring in hematologic malignancies. It may occur shortly after TACE or with a delayed onset following sorafenib therapy. Considering the kaleidoscope of novel therapies and diverse pathogenesis of HCC, it is crucial for clinicians to recognize the clinicolaboratory derangements suggestive of TLS and initiate appropriate management. The present review highlights the need for clinicians to consider TLS within differentials when caring for patients with HCC.

Introduction: Symptomatic pancreatic fluid collections (PFCs) commonly require surgical, percutaneous, endoscopic, or combination therapies for drainage. Endoscopic transluminal drainage is becoming an increasingly favorable first-line therapy at available centers. One of the challenges to endoscopic management of PFCs is the transluminal accessibility of the collection. We present a challenging case of a EUS-guided tandem transluminal stenting to achieve drainage of a large, lobulated pseudocyst extending into the mediastinum causing dysphagia. Case Description/Methods: A 75 year old male with metastatic pancreatic adenocarcinoma presented with progressive dysphagia. He was found to have an obstructing mid-body pancreatic mass with a leak identified at the tail end of the pancreas on ERCP. A resultant, large, multi-lobulated pseudocyst had formed tracking from the perigastric region into the mediastinum compressing the distal esophagus. EUS identified a large pseudocyst divided into two cavities by a septum; the only site of access was the distal esophagus. A 10Fr x 10cm hot lumen apposing metal stent (LAMS) was deployed with good drainage of the proximal cavity. Upon re-examination, the space entered by the LAMS was collapsed with healthy granulation tissue. The echoendoscope tip was passed through the LAMS with good sonographic visualization of the adjacent fluid cavity. Under EUS-guidance a needle was passed into this well-defined cystic space and a tract was created. A 10mm x 60mm fully covered wallstent was placed in tandem through the existing LAMS into the cystic cavity. The wallstent was clipped to the LAMS to prevent migration. After two weeks, imaging and fluoroscopy confirmed reduced cavity sizes and the combined LAMS-Wallstent were removed. Patient’s dysphagia improved and he was able to tolerate oral intake. Discussion: Dysphagia secondary to a mediastinal pancreatic pseudocyst is very uncommon. Endoscopic management of difficult to access collections can be very challenging. This serves as an example of combining the utility of a Wallstent with that of a larger diameter, wider lumen conduit (LAMS) to gain access to a challenging paraesophageal pancreatic fluid collection.Figure 1.: A fully covered wallstent placed in tandem through an existing LAMS with clips for the drainage of a paraesophageal pancreatic fluid collection.

INTRODUCTION: Jaundice remains a common reason for GI consultation in the adult hospitalized patient. Here we present the case of a patient admitted for a rare cause of jaundice illustrating the importance to keep a broad differential in mind when working up a patient with a high bilirubin level. CASE DESCRIPTION/METHODS: 24 y/o M who presented to the hospital with 2-3 days of lethargy and jaundice. He has a known history of cannabis use and binge use of alcohol in the past. Last drink was 1 month prior. He recently started to consume large amounts of raw egg whites. His friends have noted his eye "turn yellow" in the past. Physical Exam: General: 24 y/o well developed male who appeared stated age HEENT: Scleral icterus. CV: no murmurs. Abdominal: Soft, non tender to palpation, no hepatosplenomegaly. Labs: Hb 15.2 AST 62, ALT 33, Alk Phos 64Total bilirubin 32.5, Direct Bili 0.8 Acute hepatitis panel negativeHaptoglobin < 8, Reticulocyte count 2.4% Normal G6PD enzyme activity Urine urobilinogen < 2. US: Normal liver, there is no gallstones or biliary duct dilation He was started on phenobarbital for a probable diagnosis of Crigler-Najjar Type 2. His bilirubin declined from 27.1 to 21.8 to 12.6. He was discharged on day 4 after an uneventful hospital stay and followed up in our office with repeat lab work showing a bilirubin of 1.5 while on maintenance phenobarbital. DISCUSSION: Type 2 Crigler Najjar Syndrome (CNS) is an inherited disorder of bilirubin metabolism due to a mutation in the gene encoding for Uridine Diphosphoglucoronate Glucoronosyltransferase (UGT1A1) resulting in reduced activity of this enzyme. Patients with Type 2 CNS may have bilirubin levels as high as 40 mg/dL during their exacerbations. Phenobarbital can be used in patients with Type 2 CNS to induce activity of the UGT1A1 enzyme. In our patient, hemolysis was ruled out before a diagnosis of Type 2 CNS was made. While the patient did have a low serum haptoglobin, he was not anemic and there was no reticulocytosis to point towards a hemolytic process. He tested homozygous for the UGT1A1*28 allele indicating an additional TA sequence in the promoter region of the UGT1A1 gene affecting transcriptional activity. While homozygosity for this allele is typically seen in patient’s with Gilbert’s, his degree of unconjugated hyperbilirubinemia would not be explained by a Gilbert’s syndrome. A UGT1A1 activity level is still pending. Currently the patient has recovered and is maintained on phenobarbital with a now normal bilirubin level.

INTRODUCTION: Ampullary neoplasias are uncommon, with most tumors being of pancreaticobiliary or intestinal origins. SRCC of the Ampulla of Vater is an extremely rare variant of adenocarcinoma with only 38 previously cases reported in the literature. Little is known about the pathogenesis, treatment, and outcomes of this uncommon subtype. We report a case of ampullary SRCC in a patient who presented with a TIA and choledocholithiasis. CASE DESCRIPTION/METHODS: An 83-year-old lady was brought to the hospital with one day of slurred speech and right-sided weakness. Stroke workup was negative, her symptoms resolved rapidly, and she was diagnosed with a TIA. GI service was consulted for elevated liver enzymes. Patient reported decreased appetite for a few weeks with some weight loss. Denied fevers, chills, nausea, vomiting, jaundice, pruritis, dark urine, pale stools, new medications, alcohol abuse, or Tylenol use. She was a 15-pack year active smoker. On exam, she was afebrile with stable vitals and mild epigastric tenderness. Lab work up showed WBC 15.9 with a left shift. CMP noted TBili 3.6 (Conjugated 1.4), AST 442, ALT 260, ALP 645, Lipase 57. RUQ US showed cholelithiasis and a dilated CBD (7.3 mm). MRCP revealed mild diffuse intrahepatic and extrahepatic biliary dilatation, multiple small stones within a 1.6cm CBD with appropriate tapering at the ampulla. ERCP revealed 2-3 cm nodular cratered ulceration with irregular heaped up margins adjacent to the Ampulla of Vater. Patient underwent papillotomy, stone extraction, and placement of a fully covered wall stent. Tumor markers noted CA19-9 of 50. Pathology showed infiltrating poorly-differentiated adenocarcinoma with signet-ring cell features of the Ampulla of Vater. Tumor cells were positive for CK7, CK19, CK20, CDX2, pan keratin, MUC-1, MUC-2; and negative for MUC-6 by immunohistochemistry (IHC). After extensive discussion of findings, treatments options with multidisciplinary team including surgical oncology and palliative care services, patient declined surgical intervention and decided to spend her remaining days at home. DISCUSSION: A signet-ring variant of the adenocarcinoma of the Ampulla of Vater is an extremely rare neoplasm. This is a unique case of a lady with concomitant choledocholithiasis incidentally found to have SRCC of the Ampulla of Vater. Given the rarity of these cases, special staining and IHC will add to our knowledge of the origins of these neoplastic cells and help tailor future therapeutics.Figure 1.: Histological examination of biopsied specimen in high power view showing atypical infiltrating cells with eccentric nuclei and intra-cytoplasmic mucin.Figure 2.: Histological examination showing adenocarcinoma with characteristic signet ring cells.Figure 3.: Histological examination at lower power with signet ring cell adenocarcinoma tumor infiltration.

Intravenous sedation during colonoscopy has become the standard practice in the United States given its higher patient satisfaction and procedural quality. This practice is not free of side effects as a significant proportion of patients undergoing this procedure tend to have respiratory depression and desaturation events. Obesity, as it relates to higher levels of body mass index (BMI) has a positive correlation with the incidence of hypoxemia. During colonoscopy High flow nasal cannula (HFNC) may potentially improve oxygen performance in patients receiving colonoscopy under intravenous sedation. Here we present 3 cases of patients undergoing adjunctive oxygen therapy with HFNC during colonoscopy with intravenous sedation. We found patients to have lower number of desaturation events and were satisfied with their experience.

Lee, Chi Chan; Perez, Osman; Farooqi, Faryal; Akella, Trupti; Danckers, Mauricio; Shaharyar, Sameer Author Information

Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, is a rare condition which causes chronic recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament with resultant ischemia. Clinical presentation involves the triad of upper abdominal pain, weight loss and epigastric bruit in examination. The diagnosis is one of exclusion. The purpose of this case report is to describe a common presentation of an infrequently described rare disorder in literature. A 28-year-old healthy female presented to the emergency department (ED) with recurrent progressively worsening post-prandial sharp right upper quadrant abdominal pain for the past four months. Her pain has been associated with a 10-pound weight loss and intermittent episodes of nausea and vomiting without any fever, chills, diarrhea, or loss of appetite. She reported to no improvement with use of antacids, antiemetics and analgesics. She previously underwent outpatient ultrasonography revealing gallstones and esophagogastroduodenoscopy (EGD) revealing mild gastritis. Examination, laboratory studies, repeat ultrasound and computerized tomography (CT) scan revealed no acute abnormalities, including no gallstones. Due to nature of her chronic pain to be postprandial, a duplex ultrasound was ordered, which revealed elevated systolic velocities of the celiac artery, suggesting celiac artery stenosis and subsequently confirmed with angiography. Patient underwent a laparoscopic celiac artery decompression with a median arcuate ligament resection release. Patient was discharged following an uneventful postoperative period during her hospitalization. CACS is a rare syndrome. The pathophysiology, while not completely understood, is believed to be celiac arterial compression by variations in median arcuate ligament position. Patients are typically asymptomatic as the incidence of attributable clinical symptoms is much lower than the imaging finding. However, we present a case of a patient with chronic post-prandial pain and weight loss, in the absence of any obvious etiology, found to have a rare diagnosis of median arcuate ligament compressing on the celiac artery. We encourage clinicians to consider celiac artery compression syndrome in patients with chronic abdominal pain without a clearly established etiology.