Pseudo solid-appearing pancreatic serous microcystic adenomas: Histologic diagnosis with the EUS core biopsy fork-tip needle

Background and Objectives: Despite rarely, serous cystic adenoma (SCA) can assume a pseudo-solid aspect mimicking other pancreatic neoplasm as neuroendocrine tumor. EUS-FNA cytology has low diagnostic accuracy due to the scant cellularity of the collected samples. Histological diagnosis is usually made after resection. Recently, end-cutting needles for EUS-fine-needle biopsy (EUS-FNB), which obtain tissue cores by penetrating the lesions, have been developed. We aimed to assess the capability of EUS-FNB with SharkCore (TM) needles in the preoperative diagnosis of serous cystic adenoma pseudo-solid-appearing on imaging (Sa-SCA). Materials and Methods: Between January 2016 and January 2018, data from consecutive adult patients, who were referred for EUS-FNB of a solid pancreatic lesion and were diagnosed with having SCA, were retrieved from a single-center institutional database. Results: Two patients were excluded because of microcystic aspect at EUS. Histological diagnosis of SCA was made by EUS-FNB in the remaining 7 patients (5 females; mean age of 62.5 years). Lesions (mean size of 19.8 mm) were hypervascular on cross-sectional imaging, slightly hyperdense magnetic resonance imaging with T2-weighted images can, and negative at Ga-68-somatostatin receptor positron emission tomography and (18)fluoro-deoxyglucose positron emission tomography. EUS-FNB samples were judged adequate for a definitive diagnosis in all cases, achieving specimens suitable for histological evaluation and several ancillary stains. Histochemical positivity for periodic acid-Schiff (PAS) and PAS with diastase digestion was observed in 7/7 cases. Immunohistochemical positivity for alpha-inhibin (7/7), GLUT1 (6/6), MUC6 (5/5), and negativity for synaptophysin (7/7) and chromogranin A (2/2) favored SCA diagnosis. Conclusions: In the case of preoperative workup suspected for Sa-SCA, a "forward acquiring" needle could improve the rate of preoperative histological diagnosis

Preliminary experience with pancreatic sphincterotomy as treatment for intraductal papillary mucinous neoplasm-associated recurrent pancreatitis

\u2002Pancreatic intraductal papillary mucinous neoplasms (IPMN) are cystic tumors of the pancreas characterized by a malignant potential. IPMN have been associated with recurrent pancreatitis (RP). Obstruction of the main pancreatic duct by thick mucus has been postulated to be the cause of pancreatitis. In a few isolated reports, pancreatic sphincterotomy (PS) has been reported to reduce the frequency of pancreatitis. The aim of this study was to assess the efficacy of PS in patients with IPMN-associated RP

Azathioprine Maintenance Therapy to Prevent Relapses in Autoimmune Pancreatitis

Steroids are used to induce remission in autoimmune pancreatitis (AIP). Low-dosage steroid therapy or immunosuppressant (IMs) has been proposed as maintenance therapy to prevent AIP relapse. Few and conflicting data have been published on the efficacy of azathioprine (AZA) in preventing AIP relapse. The aim of this study was to evaluate the indication and efficacy of AZA as maintenance therapy to prevent disease relapse in AIP

CFTR function is impaired in a subset of patients with pancreatitis carrying rare CFTR variants

Background: Many affected by pancreatitis harbor rare variants of the cystic fibrosis (CF) gene, CFTR, which encodes an epithelial chloride/bicarbonate channel. We investigated CFTR function and the effect of CFTR modulator drugs in pancreatitis patients carrying CFTR variants. Methods: Next-generation sequencing was performed to identify CFTR variants. Sweat tests and nasal potential difference (NPD) assays were performed to assess CFTR function in vivo. Intestinal current measurement (ICM) was performed on rectal biopsies. Patient-derived intestinal epithelial monolayers were used to evaluate chloride and bicarbonate transport and the effects of a CFTR modulator combination: elexacaftor, tezacaftor and ivacaftor (ETI). Results: Of 32 pancreatitis patients carrying CFTR variants, three had CF-causing mutations on both alleles and yielded CF-typical sweat test, NPD and ICM results. Fourteen subjects showed a more modest elevation in sweat chloride levels, including three that were provisionally diagnosed with CF. ICM indicated impaired CFTR function in nine out of 17 non-CF subjects tested. This group of nine included five carrying a wild type CFTR allele. In epithelial monolayers, a reduction in CFTR-dependent chloride transport was found in six out of 14 subjects tested, whereas bicarbonate secretion was reduced in only one individual. In epithelial monolayers of four of these six subjects, ETI improved CFTR function. Conclusions: CFTR function is impaired in a subset of pancreatitis patients carrying CFTR variants. Mutations outside the CFTR locus may contribute to the anion transport defect. Bioassays on patient-derived intestinal tissue and organoids can be used to detect such defects and to assess the effect of CFTR modulators

Mechanism, assessment and management of pain in chronic pancreatitis: Recommendations of a multidisciplinary study group

AbstractDescriptionPain in patients with chronic pancreatitis (CP) remains the primary clinical complaint and source of poor quality of life. However, clear guidance on evaluation and treatment is lacking.MethodsPancreatic Pain working groups reviewed information on pain mechanisms, clinical pain assessment and pain treatment in CP. Levels of evidence were assigned using the Oxford system, and consensus was based on GRADE. A consensus meeting was held during PancreasFest 2012 with substantial post-meeting discussion, debate, and manuscript refinement.ResultsTwelve discussion questions and proposed guidance statements were presented. Conference participates concluded: Disease Mechanism: Pain etiology is multifactorial, but data are lacking to effectively link symptoms with pathologic feature and molecular subtypes. Assessment of Pain: Pain should be assessed at each clinical visit, but evidence to support an optimal approach to assessing pain character, frequency and severity is lacking. Management: There was general agreement on the roles for endoscopic and surgical therapies, but less agreement on optimal patient selection for medical, psychological, endoscopic, surgical and other therapies.ConclusionsProgress is occurring in pain biology and treatment options, but pain in patients with CP remains a major problem that is inadequately understood, measured and managed. The growing body of information needs to be translated into more effective clinical care

Diagnosis and treatment of exocrine pancreatic insufficiency in chronic pancreatitis: An international expert survey and case vignette study.

Despite evidence-based guidelines, exocrine pancreatic insufficiency is frequently underdiagnosed and undertreated in patients with chronic pancreatitis. Therefore, the aim of this study is to provide insight into the current opinion and clinical decision-making of international pancreatologists regarding the management of exocrine pancreatic insufficiency. An online survey and case vignette study was sent to experts in chronic pancreatitis and members of various pancreatic associations: EPC, E-AHPBA and DPSG. Experts were selected based on publication record from the past 5 years. Overall, 252 pancreatologists participated of whom 44% had ≥ 15 years of experience and 35% treated ≥ 50 patients with chronic pancreatitis per year. Screening for exocrine pancreatic insufficiency as part of the diagnostic work-up for chronic pancreatitis is performed by 69% and repeated annually by 21%. About 74% considers nutritional assessment to be part of the standard work-up. Patients are most frequently screened for deficiencies of calcium (47%), iron (42%), vitamin D (61%) and albumin (59%). In case of clinically steatorrhea, 71% prescribes enzyme supplementation. Of all pancreatologists, 40% refers more than half of their patients to a dietician. Despite existing guidelines, 97% supports the need for more specific and tailored instructions regarding the management of exocrine pancreatic insufficiency. This survey identified a lack of consensus and substantial practice variation among international pancreatologists regarding guidelines pertaining the management of exocrine pancreatic insufficiency. These results highlight the need for further adaptation of these guidelines according to current expert opinion and the level of available scientific evidence

Evidence-based Guidelines for the Management of Exocrine Pancreatic Insufficiency After Pancreatic Surgery

To provide evidence-based recommendations for the management of exocrine pancreatic insufficiency (EPI) after pancreatic surgery

Evolving concept of chronic pancreatitis.

Chronic pancreatitis (CP) is an inflammatory disease of the pancreas characterized histologically by the presence of fibrosis and inflammation of the parenchyma with the involvement of both acini and Langherans islets. CP has been considered in the past \u201cone\u201d disease, caused by alcohol, at least in the Western Countries. Epidemiological data documented an association of the disease with an excessive alcohol consumption up to the end of the last century. However, the quantification of alcohol intake was heterogeneous and a concept of risk for CP in alcohol drinkers was introduced in the absence of alcohol abuse. The clinical consequence was that the treatment of CP was mainly "symptomatic", based on alcohol withdraw and pain management in an early stage of the disease and treatment of exocrine and endocrine insufficiency in a later stage. The integration between pathological/molecular biology and radiological findings identified distinct type of chronic pancreatitis, namely autoimmune pancreatitis, paraduodenal pancreatitis and pancreatitis associated with gene mutations. Other forms of CP on which there is still no international agreement are represented by organic spincter of Oddi dysfunction (SOD), CP caused by scar(s) secondary to previous episodes of necrotizing pancreatitis, pancreatitis associated with anatomical abnormalities (pancreas divisum, annular pancreas) and pancreatitis associated to slow-growing tumours of the pancreas or of the periampullar region

Endoscopic ultrasonography in chronic asymptomatic pancreatic hyperenzymemia: The more we see, the less we know

Editorial on endoscopic ultrasonography in chronic asymptomatic pancreatic hyperenzymemi

Chapter 16: The Italian Experience

This book offers a manageable understanding of key aspects of IgG4 related disease while providing in-depth information that should well serve those seeking a deeper understanding of this unique disorder