A Case Report of Insulinoma Relapse on Background Nesidioblastosis: A Rare Cause of Adult Hypoglycemia.

Context: Nesidioblastosis is a rare cause of adult hypoglycemia. Current medical therapy can mitigate disease symptoms. However, side effects and limited efficacy may prevent long-term disease management. Case Description: A 63-year-old white woman presented at our institution on April 2017 with a history of distal spleno-pancreatectomy for well-differentiated insulinoma in 2013. Hypoglycemic events did not resolve after surgery, and residual nesidioblastosis near the pancreatic resection margins was identified. Hypoglycemic episodes increased in frequency and severity despite high-dose diazoxide (DZX) therapy. On April 2016, octreotide was introduced but soon discontinued for inefficacy. When the patient arrived at our attention, add-on pasireotide was started and glucose levels monitored by subcutaneous sensor. Compared with DZX, 225 mg/d alone, sensor glucose during pasireotide + DZX 75 mg/d showed occurrence of severe hypoglycemia. Pasireotide was discontinued, and the instrumental workup (68Ga-DOTATOC CT/positron emission tomography, 99mTc-nanocolloid scintigraphy and echo-endoscopy + fine-needle aspiration biopsy) identified an insulinoma relapse. Subtotal pancreatectomy was performed without further recurrence of hypoglycemia over 9 months of follow-up. Conclusions: Although insulinoma relapses on background nesidioblastosis rarely occur, they should be considered as an alternate diagnosis when medical therapy fails to prevent hypoglycemia. Further studies are warranted to test whether the immunophenotypic signature of nesidioblastosis/insulinoma may provide insights for a tailored use of pasireotide.

Clinical and Morphological Features of Paraduodenal Pancreatitis: An Italian Experience With 120 Patients.

OBJECTIVES: This study aimed to evaluate the clinical and radiological features and clinical outcomes of paraduodenal pancreatitis (PP). METHODS: A final diagnosis of PP was based on surgical specimens in resected patients and on imaging in nonoperated patients. Clinical, radiological, and pathological data were collected and reevaluated. RESULTS: We studied 120 patients, 97.5% of whom were drinkers and 97.5% were smokers. Symptoms at clinical onset were acute pancreatitis in 78 patients (65%) and continuous pain in 68 patients (55.8%). Other symptoms were vomiting (36.7%), weight loss (25.8%), and jaundice (11.7%). Cystic variant was diagnosed in 82 patients (68.0%), and solid variant was diagnosed in 38 patients (32.0%). Pure and diffuse forms were observed in 22 (18.3%) and 98 (81.7%) patients, respectively. Pancreatic calcifications were present at clinical onset in 5.0% of the patients and in 61.0% at the end of follow-up. Somatostatin analogs were used in 13 patients (10.8%), and 81 patients (67.0%) underwent surgery. CONCLUSIONS: The clinical profile of PP was found to be middle-aged men who were heavy drinkers and smokers with painful pancreatitis and was associated with vomiting and weight loss. In nonresponders, alcohol withdrawal and medical therapy can be proposed as a first-line treatment, and surgery as a second-line treatment.

Endoscopic submucosal dissection in the treatment of gastric submucosal tumors: results from a retrospective cohort study.

BACKGROUND: A submucosal tumor (SMT) of the stomach, which is an occasional finding during routine upper gastrointestinal endoscopy, may pose diagnostic and therapeutic challenges. METHODS: To assess whether endoscopic submucosal dissection (ESD) is a feasible approach to definitively cure SMTs, the authors performed a retrospective cohort study with two endoscopic italian centers. RESULTS: The study consisted of 20 patients with SMTs who underwent ESD. The patients underwent ESD and were followed up by endoscopy. We analyzed complete resection rate, frequency of complications, and survival. The overall rate of R0 resection was 90 % (18/20), with two endoscopic failures, one for a submucosal tumor and one for a neoplasm deeply infiltrating the proper muscle layer. The median procedure time was 119.1 min (range 40-240 min). The median size of the resected specimens was 29 mm (range 15-60 mm). Perforation occurred in 3 patients; all were treated conservatively. There were no cases of severe bleeding. Based on histopathological findings, 6 cases of ectopic pancreas, 1 of ectopic spleen, 3 of leiomyoma, and 10 of gastrointestinal stromal tumor (GIST) were diagnosed. Complete resection was obtained in all GIST cases. Among the 10 GIST cases treated by ESD, no death occurred: the 5-year disease-specific survival rate was 100 %. CONCLUSIONS: The high success rate of 90 % and the low incidence of complications should indicate ESD is the correct diagnostic and definitive treatment in selected patients.