Lupus Pancreatitis Masquerading as Pancreatic Cancer: A Rare Clinical Presentation.

Systemic lupus erythematosus (SLE) is an autoimmune multisystemic inflammatory disease. SLE-associated pancreatitis is uncommon, and pancreatic cancer in SLE is very rare. Imaging findings in SLE with pancreatitis can mimic malignancy. Endoscopic ultrasound with fine-needle aspiration/biopsy can guide in the accurate diagnosis and management of SLE-associated pancreatitis.

A decade's experience of managing suspected pancreatic adenocarcinoma at a tertiary cancer center.

Background: We present our experience and established management strategy for endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA) in diagnosing suspected pancreatic neoplasms at a tertiary referral cancer hospital. Method: Relevant data were extracted from our database for patients who underwent EUS-FNA for suspected pancreatic neoplasms at our institution between 2007 and 2016. Results: Among the 309 patients, the median age was 67 years and 56% were men. The most common presenting symptoms were abdominal pain (37%) and jaundice (29%). Concordance between radiographic diagnosis and final pathology was 89%. The mean lesion size was 34.9 mm on computed tomography and 31.5 mm on EUS. There were 197 patients (64%) with localized disease, of whom 115 (58%) had resectable lesions, 61 (31%) had borderline resectable, and 21 (11%) had unresectable lesions (mean CA 19-9 levels 1705 U/mL, 2490 U/mL, and 479 U/mL, respectively). A median of 3 FNA passes were performed to establish a pathologic diagnosis. Two patients (1%) had postprocedural adverse events. Median overall survival was 47 months in those who underwent surgery after EUS and 12 months in those who did not (P<0.001). Conclusions: A multidisciplinary approach is employed for management of suspected pancreatic neoplasm at our tertiary cancer center. A combination of cross-sectional imaging and EUS-FNA serves as a highly effective duo in establishing a tissue diagnosis and staging with a low adverse event rate. Counterintuitively, CA 19-9 is not necessarily higher with resectable lesions than with unresectable lesions, indicating the limitation of CA 19-9 as a pancreatic tumor marker.

SpyGlass DS system-assisted retrieval of proximally migrated pancreatic duct stent in a patient with chronic pancreatitis.

Pancreatic duct (PD) stent migration is among the recognized complications of endoscopic retrograde cholangiopancreatography (ERCP) with PD stent placement. Proximal stent migration poses a challenge for removal due to risks of PD damage, smaller caliber, and possible stricture. Here, we present a case of SpyGlass DS system-assisted PD stent repositioning after failure with traditional tools.

Liver transplantation in patients with acute-on-chronic liver failure.

Acute-on-chronic liver failure (ACLF) is a dynamic syndrome associated with a very high short-term mortality. Hence, the ongoing assessment of treatment response, an expedited liver transplant evaluation and listing, and the determination of futility of treatment are critical for optimal outcomes. In this review, we appraise our current understanding of the timing and futility of liver transplantation, and the short- and long-term outcomes including the quality of life after deceased or live donor liver transplantation in those with ACLF.

Pancreatic paraganglioma diagnosed by endoscopic ultrasound-guided fine needle aspiration: A case report and review of literature.

BACKGROUND: Pancreatic paragangliomas (PPGL) are rare benign neuroendocrine neoplasms but malignancy can occur. PPGL are often misdiagnosed as pancreatic neuroendocrine tumor or pancreatic adenocarcinoma. CASE SUMMARY: We reviewed 47 case reports of PPGL published in PubMed to date. Fifteen patients (15/47) with PPGL underwent endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). Only six (6/15) were correctly diagnosed as PPGL. All patients with PPGL underwent surgical resection except three (one patient surgery was aborted because of hypertensive crisis, two patients had metastasis or involvement of major vessels). Our patient remained on close surveillance as she was asymptomatic. CONCLUSION: Accurate preoperative diagnosis of PPGL can be safely achieved by EUS-FNA with immunohistochemistry. Multidisciplinary team approach should be considered to bring the optimal results in the management of PPGL.

Early- and late-onset pancreatic adenocarcinoma: A population-based comparative study.

BACKGROUND: Pancreatic cancer is projected to become the second leading cause of cancer related death in the US. We aim to investigate the demographics, clinical outcomes and survival outcomes of patients diagnosed with early-onset (<40 years) and late-onset (>40 years) pancreatic adenocarcinoma (PAC). METHODS: Data on PAC between 1975 and 2016 were extracted from the Surveillance, Epidemiology, and End Results Registry. RESULTS: Within the study period, 136,100 patients were identified which included 1181 patients with early-onset PAC and 134,919 patients with late-onset PAC. Both cohorts tend to present with distant metastasis (70.3% vs 57.9%). Both groups also showed an exponential rise in incidence (early-onset 3.69% annual change vs late-onset 6.25% annual change). When stratified by anatomical location, there was a trend of increasing cancer in the head of the pancreas for patients <40 years (3.63% annual change). While late PAC showed increasing cancer in all anatomical locations, the largest increase was observed in the tail of the pancreas (8.62% annual change). Overall, there was a mild difference in survival for early- and late-onset PAC (7 months vs 6 months, respectively, log rank p = 0.004). Both age groups showed the worse prognosis when cancer occurred in the tail of the pancreas (6 months vs 4 months, respectively). On cox proportion analysis, patients with late-onset PAC had twice the risk of mortality compared to early-onset PAC (HR 2.06, CI: 1.788-2.370, P = 0.001). CONCLUSIONS: Our study showed that both early- and late-onset PAC are increasing and while prognosis remains poor. Tumor anatomy showed a growing incidence of early-onset PAC in the head of the pancreas while late-onset PAC showed a rising incidence in the body and tail of the pancreas.

Performing Endoscopic Retrograde Cholagiopancreatpgraphy and Endoscopic Ultrasound for Management of Malignant Bile Duct Obstruction in a Patient With a Situs Inversus Totalis.

Situs inversus totalis (SIT) is a rare anomaly characterized by the transposition of organs. We present a case of a 67-year-old White woman with a history of SIT, who presented with fatigue, jaundice, and abnormal liver enzymes. Endoscopic ultrasound demonstrated a solid lesion at the distal common bile duct (CBD). Subsequent endoscopic retrograde cholangiopancreatography displayed severe stenosis in the CBD. A plastic stent was placed into the CBD, resulting in successful biliary decompression. Biliary brushings and biopsy showed atypical cells, suspicious for carcinoma. Ensuing pancreaticoduodenectomy confirmed cholangiocarcinoma. Although challenging, endoscopic ultrasound and endoscopic retrograde cholangiopancreatography in SIT can be successfully performed in preoperative evaluation for possible pancreaticobiliary cancers.

Clostridioides difficile infection in US hospitals: a national inpatient sample study.

BACKGROUND: Hypervirulent strains of Clostridioides difficile have altered the landscape of hospital and community outbreaks. We aim to examine and compare spatiotemporal trends, incidence, hospital teaching status, mortality, and cost associated with hospital-acquired Clostridioides difficile infection (HCDI) and community-acquired Clostridioides difficile infection (CCDI). METHODS: Retrospective cohorts were studied using data from the Healthcare Cost and Utilization Project (HCUP) Nationwide Inpatient Sample (NIS) from 2006 to 2015. RESULTS: A total of 76,124 cases of HCDI and 190,641 cases of CCDI were identified within the study period. The incidence of HCDI decreased from 8555 in 2006 to 7191 in 2015. Mortality also decreased during the same period (5.9% in 2006 to 1.4% 2015, p < 0.0001). Conversely, CCDI cases increased from 13,823 in 2006 to 20,637 in 2015. CCDI mortality decreased during the same period (4.3% in 2006 to 1.9% 2015, p < 0.0001). Rural hospital centers experienced the sharpest decline in HCDI mortality compared to urban and urban teaching centers (3.8%, p < 0.0001 vs 2.8%, p < 0.0001 vs 2.1%, p < 0.0001). Multivariate logistic regression indicated that increasing age (p = 0.0001), increasing hospital length of stay (p = 0.0001), and Medicare insurance (p = 0.002) were significant predictors of mortality for CDI mortality. Geospatial mapping of CCDI and HCDI revealed that the Eastern and Southern US experienced the largest incidence of CDI over 10 years. CONCLUSION: The incidence of HCDI has decreased in the past decade while the incidence of CCDI hospitalization is sharply on the rise. While hospital length of stay and mortality has decreased over time, the cost of treating CDI remains high.

Similarities and differences in guidelines for the management of pancreatic cysts.

Accurate diagnosis of Pancreatic cysts (PC) is key in the management. The knowledge of indications for surgery, the role of endoscopic ultrasound-guided fine needle aspiration, cyst fluid analysis, imaging, and surveillance of PC are all important in the diagnosis and management of PC. Currently, there are many guidelines for the management of PC. The optimal use of these guidelines with a patient-centered approach helps diagnose early cancer and prevent the spread of cancer.

Gas embolism during endoscopic retrograde cholangiopancreatography: diagnosis and management.

Air embolism is rarely diagnosed and is often fatal. The diagnosis is often not made in a timely manner given the rapid and severe clinical deterioration that often develops, frequently leading to cardiac arrest. Many patients are only diagnosed post-mortem. With the increasing use of endoscopic retrograde cholangiopancreatography, air embolism should be considered in the differential diagnosis in patients who experience sudden clinical deterioration during or immediately after the procedure. Clinical suspicion is key in the diagnosis and management of air embolism. Use of precordial Doppler ultrasound and transesophageal echocardiogram can aid in the diagnosis of air embolism. Once the diagnosis is made, supportive management of airway, breathing and circulation is pivotal. Advanced cardiac life support should be initiated when necessary. Fluid resuscitation and vasopressors can improve cardiac output. Hyperbaric oxygen therapy should be considered when possible in cases of suspected cerebral air embolism cases to improve neurological outcome. A multidisciplinary team approach and effective communication with experts, potentially including an anesthesiologist, cardiologist, intensivist, radiologist and surgeon, can improve the outcome in air embolism.

Biliary Decompression in Perihilar Cholangiocarcinoma Improves Survival: A Single-Center Retrospective Analysis.

BACKGROUND AND AIMS: The complex biliary strictures of perihilar cholangiocarcinoma present significant challenges for providing adequate and long-lasting biliary drainage. The best approach to relieve obstruction remains controversial. The purpose of this study was to assess stenting outcomes in perihilar cholangiocarcinoma. METHODS: This study was approved by the center's institutional review board. Subjects with a diagnosis of perihilar cholangiocarcinoma who underwent endoscopic retrograde cholangiopancreatography (ERCP) were identified from endoscopic and pathologic databases from 1997 to 2014. Patient characteristics, endoscopic data, and follow-up evaluation data were retrospectively collected via review of available medical records. RESULTS: A total of 199 patients with perihilar cholangiocarcinoma who underwent a total of 504 ERCPs were included in the study. Nine of 504 (1.8%) procedures were technical failures. Among the 495 technically successful procedures, 347 (70.1%) procedures were clinical successes. Clinical success was significantly associated with longer overall survival (HR 0.57; p = 0.002). A higher proportion of patients with bilateral drainage had clinical success, compared with those with unilateral drainage. Cholangitis was not more common in the bilateral group compared to the unilateral group except in the group where a segment was not drained (1.9% vs 1.6% vs 7.1%, respectively). Patients with metal stents were 3.8 times more likely to have clinical success than those with plastic stents. CONCLUSIONS: In conclusion, adequate biliary drainage improves overall survival. Bilateral stenting if anatomy permits with self-expanding metal stents rather than plastic stents appears to provide the optimal chance of clinical success.

Clinical update on the management of pseudopapillary tumor of pancreas.

Solid pseudopapillary neoplasm (SPN) is a rare tumor with malignant potential which is generally located in the tail of pancreas. The prevalence of SPN has increased with widespread use of cross sectional imaging. SPN is often misdiagnosed due to nonspecific clinical presentation and accurate diagnosis is essential for optimal management. Endoscopic ultrasound-FNA with immunohistochemistry can help in preoperative diagnosis. Surgery is the treatment of choice and a successful R0 resection is curative. Overall, SPN has a good prognosis. This review article focuses on pathogenesis, diagnosis and management of SPN.

Pancreatic cancer screening in high-risk individuals with germline genetic mutations.

BACKGROUND AND AIMS: Pancreatic cancer (PC) is a deadly disease that is most commonly diagnosed at an incurable stage. Different high-risk genetic variants and cancer syndromes increase the lifetime risk of developing PC. This study aims to assess the yield of initial PC screening in patients with high-risk germline mutations. METHODS: Asymptomatic adults underwent PC screening by EUS, magnetic resonance imaging, or CT during a 10-year period and were retrospectively identified. High-risk individuals were defined as carrying germline mutations in BRCA1, BRCA2, p53 (Li-Fraumeni), STK11 (Peutz-Jeghers), MSH2 (Lynch), ATM (ataxia-telangiectasia), or APC (familial adenomatous polyposis). Patients without germline mutations were excluded. RESULTS: In total, 86 patients met the study criteria. The median age was 48.5 years (interquartile range, 40-58), 79.1% (68) were women, and 43.0% (37) had a family history of PC. The genetic mutations were BRCA2 (50, 58.1%), BRCA1 (14, 16.3%), p53 (12, 14.0%), STK11 (5, 5.8%), MSH2 (3, 3.5%), ATM (1, 1.2%), and APC (1, 1.2%). Screening detected a pancreatic abnormality (PA) in 26.7% (23/86), including cysts (11, 47.8%), hyperechoic strands and foci (10, 43.5%), and mild pancreatic duct dilation (2, 8.7%). Patients older than 60 years were more likely to have a PA detected (P = .043). EUS detected more PAs than magnetic resonance imaging or CT. No cases of PC were diagnosed by screening or during follow-up (median, 29.8 months; interquartile range, 21.7-43.5). CONCLUSIONS: Unless indicated otherwise by family or personal history, PC screening under the age of 50 is low yield. Linear EUS may be the preferred modality for initial PC screening.

How best to manage gastrointestinal stromal tumor.

Gastrointestinal stromal tumors (GISTs) are rare but most common nonepithelial tumor of gastrointestinal tract. They are often found incidentally on computed tomography and endoscopic investigations. Increasing knowledge of the pathogenesis of GISTs and the advent of tyrosine kinase inhibitors revolutionized the management of GISTs. The newer advanced endoscopic techniques have challenged the conventional surgery although the true efficacy and safety of endoscopic approach is not clear at this time. This review article focuses on pathogenesis, diagnosis and management of GISTs.

Comparison of nonampullary duodenal adenomas in patients with familial adenomatous polyposis versus patients with sporadic adenomas.

BACKGROUND AND AIMS: Nonampullary duodenal adenomas are either sporadic or associated with a hereditary syndrome such as familial adenomatous polyposis (FAP). The aim of this study is to compare characteristics and outcomes of sporadic and FAP-associated duodenal adenomas. METHODS: We retrospectively collected clinical, endoscopic, and pathologic data in patients diagnosed with duodenal adenomas at our institution and included all available follow-up. RESULTS: Two hundred thirteen subjects were identified; 118 had FAP and 95 had sporadic adenomas. FAP subjects were more likely to have multifocal disease. Initial size was not significantly associated with dysplasia. Fourteen (12%) with FAP and 33 (35%) with sporadic adenomas underwent EMR. Among those subjects who did not undergo EMR or surgery, there was no difference between the FAP and sporadic groups with progression to new dysplasia or cancer. However, the FAP group was significantly more likely to have dysplasia at follow-up (P = .05). There was a significant difference in overall survival between the FAP and sporadic groups (log-rank test, P < .001). In the subgroup of patients aged 40 years old and older who did not undergo intervention, the FAP group had a shorter time to pathology progression compared with the similar sporadic subgroup. Range of time to progression to cancer was 3 to 161 months. CONCLUSIONS: FAP subjects were more likely to be younger and have multifocal disease. Progression of pathology was more likely in the older FAP group compared with the sporadic group. Time to progression to cancer was widely variable and, therefore, unpredictable.

Bone morphogenetic protein type I receptor antagonists decrease growth and induce cell death of lung cancer cell lines.

Bone morphogenetic proteins (BMPs) are highly conserved morphogens that are essential for normal development. BMP-2 is highly expressed in the majority of non-small cell lung carcinomas (NSCLC) but not in normal lung tissue or benign lung tumors. The effects of the BMP signaling cascade on the growth and survival of cancer cells is poorly understood. We show that BMP signaling is basally active in lung cancer cell lines, which can be effectively inhibited with selective antagonists of the BMP type I receptors. Lung cancer cell lines express alk2, alk3, and alk6 and inhibition of a single BMP receptor was not sufficient to decrease signaling. Inhibition of more than one type I receptor was required to decrease BMP signaling in lung cancer cell lines. BMP receptor antagonists and silencing of BMP type I receptors with siRNA induced cell death, inhibited cell growth, and caused a significant decrease in the expression of inhibitor of differentiation (Id1, Id2, and Id3) family members, which are known to regulate cell growth and survival in many types of cancers. BMP receptor antagonists also decreased clonogenic cell growth. Knockdown of Id3 significantly decreased cell growth and induced cell death of lung cancer cells. H1299 cells stably overexpressing Id3 were resistant to growth suppression and induction of cell death induced by the BMP antagonist DMH2. These studies suggest that BMP signaling promotes cell growth and survival of lung cancer cells, which is mediated through its regulation of Id family members. Selective antagonists of the BMP type I receptors represents a potential means to pharmacologically treat NSCLC and other carcinomas with an activated BMP signaling cascade.