Updated European guidelines for clinical management of familial adenomatous polyposis (FAP), MUTYH-associated polyposis (MAP), gastric adenocarcinoma, proximal polyposis of the stomach (GAPPS) and other rare adenomatous polyposis syndromes: a joint EHTG-ESCP revision.

BACKGROUND: Hereditary adenomatous polyposis syndromes, including familial adenomatous polyposis and other rare adenomatous polyposis syndromes, increase the lifetime risk of colorectal and other cancers. METHODS: A team of 38 experts convened to update the 2008 European recommendations for the clinical management of patients with adenomatous polyposis syndromes. Additionally, other rare monogenic adenomatous polyposis syndromes were reviewed and added. Eighty-nine clinically relevant questions were answered after a systematic review of the existing literature with grading of the evidence according to Grading of Recommendations, Assessment, Development, and Evaluation methodology. Two levels of consensus were identified: consensus threshold (≥67% of voting guideline committee members voting either 'Strongly agree' or 'Agree' during the Delphi rounds) and high threshold (consensus ≥ 80%). RESULTS: One hundred and forty statements reached a high level of consensus concerning the management of hereditary adenomatous polyposis syndromes. CONCLUSION: These updated guidelines provide current, comprehensive, and evidence-based practical recommendations for the management of surveillance and treatment of familial adenomatous polyposis patients, encompassing additionally MUTYH-associated polyposis, gastric adenocarcinoma and proximal polyposis of the stomach and other recently identified polyposis syndromes based on pathogenic variants in other genes than APC or MUTYH. Due to the rarity of these diseases, patients should be managed at specialized centres.

Endoscopic ultrasound-guided drainage of pancreatic fluid collections: The impact of evolving experience and new technologies in diagnosis and treatment over the last two decades.

BACKGROUND: Endoscopic ultrasound (EUS)-guided drainage is the preferred approach for drainage of pancreatic fluid collections (PFCs) due to the better experience and significant progress using newer stents and access devices during last decade. This study aimed to evaluate the role of the evolving experience and possible influence of new technological devices on the outcome of patients evaluated for PFCs and submitted to EUS-guided drainage during two different periods: the early period at the beginning of experience when a standardized technique was used and the late period when the increased experience of the operator, combined with different stents quality were introduced in the management of PFCs. METHODS: We retrospectively analyzed the clinical data of a cohort of 91 consecutive patients, who underwent EUS-guided drainage of symptomatic PFCs from October 2001 to September 2017. Demographic, therapeutic results, complications, and outcomes were compared between early years' group (2001-2008) and late years' group (2009-2017). RESULTS: Endoscopic treatment was successfully achieved in 55.6% (20/36) of patients in the early years' group, and in 96.4% (53/55) in the late years' group. Eighteen patients (12 in early years' and 6 in the late year's group) required additional open surgery. Procedural complications were observed in 5 patients, 4 in early years' and 1 in late years' group. Mortality was registered in two patients (2.2%), one for each group. CONCLUSIONS: During our long-term survey using EUS-guided endoscopic drainage of PFCs, significantly better outcomes in term of improved success rate and decrease complications rate were observed during the late period.

Colonic Acquired Fistulae: The Potential of CT Colonography.

The diagnosis of colonic fistulae is crucial in defining the best therapeutic approach. Fistulae can be suspected from clinical history or specific symptoms, but imaging plays a key role in confirming the diagnosis. Previous studies described conventional studies with barium as methods of choice for the diagnosis; more recently intestinal ultrasound, CT and MR have been considered promising tests. CT Colonography is the best radiologic test to evaluate the colon, but only few case reports described its role in this setting. Aim of this paper is to highlight the potentials of CT colonography in evaluating patients with suspected colonic fistulae.

Recurrent oncocytic adrenocortical carcinoma: implementing diagnostic criteria in a case report with the longest survival.

INTRODUCTION: Oncocytic adrenocortical tumors represent a subtype of the adrenal cortex neoplasms. These tumors can be divided into oncocytomas, oncocytic neoplasms of uncertain malignancy and carcinomas (OACs). To date, only 34 cases of OAC have been reported. CASE EXPERIENCE: We reported a case of a 54-year-old male patient with a history of laparoscopic right adrenalectomy for an OAC and subsequent chemotherapy for a recurrence. He was referred to our emergency room for diffuse abdominal pain, vomit, change in bowel habits, fever, asthenia. He underwent a laparotomy and a complete excision of the known bilobate lesion. The histopathological findings matched the features of a recurrent OAC. No chemotherapy was administered after surgery and the patient was disease-free after a follow-up of twenty-eight months. RESULTS - DISCUSSION: The most questionable issue in treating adrenocortical oncocytic neoplasms is the determination of malignancy. According to the Helsinki Score, which is the best prognostic system, the primary lesion was an OAC. We also implemented the score systems to the recurrent lesion, that seemed to be malignant. We believe that the adjuvant treatment can delay a recurrent lesion development, but finally, radical surgical excision is necessary. Moreover, we reported the longest survival after the primary adrenalectomy. CONCLUSIONS: This study described the first case of recurrent oncocytic adrenocortical carcinoma with the longest followup. Adrenocortical oncocytoma is an extremely rare tumor of the adrenal gland with variable biological behavior without definitive consensus about diagnostic criteria. This was also the first case in which different histopathological criteria have been implemented in a recurrence. KEY WORDS: Oncocytic adrenocortical carcinoma, Surgical oncology, Survival.

Efficacy of Tumour Necrosis Factor-alpha therapy in paediatric Crohn's disease patients with perianal lesions: a systematic review.

Introduction: Anti-Tumor Necrosis Factor-alpha (TNF-α) therapy, primarily infliximab and adalimumab, are now increasingly used to induce and maintain disease remission in the pediatric perianal Crohn's disease (CD) population, however, their optimal use has not yet been defined in the pediatric setting.Areas covered: In accordance with a published protocol (PROSPERO no. CRD42019118838), we systematically and critically evaluated all published evidence on the efficacy and safety of anti-TNF-α in children with perianal CD, in the PubMed, MEDLINE, Embase, Cochrane and clinicalTrials.gov databases until October, 18th, 2018. We included in our systematic review 29 articles yielding a total of 565 perianal CD patients aged between 9 months to 18 years.Expert opinion: According to low-quality evidence from small, uncontrolled and heterogeneous descriptive studies, and very few randomized controlled trial, nearly three-fifths children with perianal CD achieved remission with anti-TNF-α treatment and in approximately 40% remission was maintained after 12 months, with practically low discontinuation rate due to serious adverse events. More than half of the patients achieved complete fistula closure. There is still a need for more robust evidence adequately assessing the efficacy and safety of anti-TNF-α therapy in pediatric perianal CD, as well as in comparison with other therapies.