Introduction: Insulinoma is the most common functioning neuroendocrine tumor of the pancreas. Although rare, is the main cause of endogenous hyperinsulinemic hypoglycemia.The most common clinical manifestations are neurovegetative and neuroglycopenic symptoms secondary to hypoglycemia. Progressive weight gain is also an important clinical feature, due to the anabolic action of insulin and the need to feed periodically in an attempt to reduce hypoglycemia. The presence of neuroglycopenic symptoms, which sometimes may be confounded with psychiatric symptoms, may lead to delay or misdiagnosis. In our country, there are few publications about the early diagnosis and treatment of this neoplasm. Surgery still seems to be the best treatment option but, in selected case in which there are high surgical risk, the minimally invasive procedures, such as endoscopic ultrasound (EUS) guided ethanol ablation can be a good choice of treatment. We report a case of an insulinoma in a patient with extreme obesity,that was submitted to a less invasive treatment due to surgical risks. Case report: A 22-years old woman, presented progressive weight gain of 65 kg in 1 year (163kg, BMI: 59.6kg/m2). After using intragastric balloon for 6 months, she developed hypoglycemic symptoms. Prolonged fasting test (PFT), was performed and showed after 40 minutes plasma glucose: 44mg / dl (< or = 55mg/dl); C peptide: 3.2 ng / ml ( > or = 0,6ng/ml) , insulin: 47 mU / ml (> or = 3mU/ ml), anti-insulin antibody negative. EUS showed a homogeneous hypoechoic lesion in the cephalicregion of uncinate pancreas process and fine needle puncture showed cells with atypia of indeterminate significance. Magnetic Resonance Image of the abdomen showed a 1.9 cm hypervascular nodule in the cephalic portion of the pancreas, very close to cava vein. The main hypothesis was an insulinoma. Due to the patient's severe obesity and the tumor localization, a multidisciplinary team decided to do a minimally invasive procedure. EUS guided ethanol ablation with absolute ethanol and a fine needle aspiration were performed with general anesthesia without complications. On the following days of the procedure, surveillance for pancreatitis was performed. Anatomopathological showed immunohistochemistry positive for insulin and ki67% <1%. Laboratory exams of the 30th postoperative day: fasting glycemia: 89mg / dl, peptide C: 5.18ng / dl, insulin: 45.51 Uui / ml. After 6 months of the procedure, the patient remains without hypoglycemia and lost 10 kg. Conclusion: Insulinoma as described above is a rare condition, but should always be part of the differential diagnosis of obesity and hypoglycemia. Careful analysis and multidisciplinary decision-making for best behavior are key. Minimally invasive treatment is promising, with reduced surgical risks, shorter hospital stay and increased quality of life after the procedure. As seen in our case.

Background Carney's complex (CNC) is a rare genetic multiple neoplasia syndrome, with involvement in several systems, among them, acromegaly is a possible condition, according to the literature, seen in about 10% of cases. PPRKAR1A is the most known gene evolved. The genetic origin of 59% of patients with CNC has not yet been elucidated. ClinicalCases A 17-year-old malewithtall stature, withstigmaofgigantism, GH 9.89 ng/ml and IGF-1 1.5 times higher reference value, MRI with pituitary adenoma of 0.7cm. After resection transfenoidal endoscopic (TSE) thepathology of the tumor revealed a pure GH pituitary adenoma. Echocardiogram showed cardiac myxomas. For that reason, a Carney complex was established. In the search for other alterations present in the syndrome, a nodule was found in the right testicle, which after nodulectomy shown a Leydig Cell Tumor. After these findings, we invited all family members to perform screening to identify possible involvement of the CNC. Two brothers, including a twin, and their mother also had stigma of acromegaly, all with pituitary adenoma and laboratorial diagnostic. The two menunderwentTSE surgery reassuring GH secretion by the tumor. The elderly brother had aggressive microcalcilfications in bilateral testicles. After bilateral orchiectomy was diagnosed witha large cell calcifying sertoli cell tumor. The twin brother also had cardiac mixomas and macrocalcifications in testicle but without signs of malignancy. The mother had athyroid ultrasound showing a multinodular goiter and breast lesions also with no signs of malignancy. She also had a pituitary microadenoma,butrefused the surgery and is currently using thesomatostatinanalog. CNC is a multiple neoplasia syndromeusually characteriszed by lentiginosis, multiple neoplasias and signs of endocrine overactivity, particularly Primary Pigmented Nodular Adrenal Disease (PPNAD) and myxomas. It is a rare condition and its prevalence remains unknown. A patogenic allelic variant (PAV) of PRKAR1A is found in 37% of patients with sporadic CNC and more than 70% of patients with familial CNC. To this date, at least 130 different mutations have been described in more than 400 families around the world, distributed across the ten coding exons (PRKAR1A has 11 exons, but exon 1 is non-coding and rarely mutated) and in the adjacent intronic sequences. In 2014, a germline triplication of the exon of PRKACB gene has been identified in pacient who did not harbour a PRKAR1A mutation. In our case, a search of PAV was realized by exome NGS and CG-Array but we found no evidence of variant that can explain the occurence of CNC. Conclusion We describe the follow-up of a family with CNC without PAV identified so far. Presentation: No date and time listed

Background and aims: The physiopathology of hyperinsulinemic hypoglycemia (HH) after gastric bypass (GB) is not well understood, although it is a common adverse event after this procedure. The fast absorption of glucose after a meal, the high glucose variability, the increase in glucagon peptide 1 secretion or the hyperplasia of beta cell have been postulated as possible hypothesis. Mixed Meal Tolerance test (MMT) is used in clinical practice during HH investigation, but there is no consensus for HH diagnosis after bariatric surgery. In this scenario, we evaluated the MMT for the diagnosis of HH after GB. Material and Methods: This is an observational cross sectional descriptive study of adult (&gt; 18 years) patients submitted to a MMT after GB from July 2016 to October 2019. 51 patients were divided in two groups: Group 1, with a history of predominantly neuroglycopenic symptoms (n = 24) and Group 2 (control) without symptoms of postprandial hypoglycemia (n= 27). The patients had no diagnosis of diabetes and weren’t using any hypoglycemic drugs. All subjects performed the MMT composed by a typical Brazilian breakfast with the following composition: 494 Kcal with 63.4% carb, 27.5% fat and 9.1% protein, in the morning after 8h fasting and blood samples (glucose, insulin, C peptide) were collected before the meal and every 30 minutes for 5 h after it. A positive test was considered if patient presents Whipple’s Triad: HH (plasma glucose was ≤ 55mg/dL with insulinemia ≥ 3 µU/mL, C peptide ≥ 0.6 ng/mL) and hypoglycemic symptoms. Statistical analysis were done using SPSS 13.0 version. Results:​ From 51 patients, 46 were female, mean age was 46.8 ± 9.2 years. 15 of the 24 patients with predominantly neuroglycopenic symptoms (Group 1) developed laboratory HH, but only 9 (37.5%) presented the Whipple’s Triad. Only one subject of the control group presented HH. All patients with neuroglycopenic symptoms during the test presented HH. From those with HH, 43% presented hypoglycemia at 90’, 50% at 120’ and 6.2% at 150’ during MMT. There were no difference between the two groups when compared the amount of weight loss neither the period of follow up after GB. Conclusion: Since all patients with neuroglycopenic symptoms during the test presented HH it might be a useful tool to exclude HH in patients with hypoglycemic symptoms after GB. This study suggests that the BMT doesn’t need to be 5h, since all of hypoglycemic episodes occur until 150’.

Background : Insulinoma is the most common functioning pancreatic neuroendocrine tumor. It’s an important endocrine etiology of obesity, which is characterized by a hyperinsulinemic hypoglycemia (HH). Case reports: Case 1: A 23 years old woman developed hypoglycemia symptoms and weight gain (13kg in 6 months). The prolonged fasting test (PFT) was performed: plasma glucose 29 mg/dL, C-peptide 2,6ng/mL (< 0,6 ng/mL), plasma insulin 29,9 μU/mL (< 3,0 μU/mL), showing a HH. Abdominal CT showed a 1.3 cm hypervascularity nodule, next to the distal portion of the pancreatic tail close to the vein and splenic artery. A successful partial pancreatectomy and splenectomy were performed and she remains asymptomatic. Case 2: A 41 years old man, with severe obesity Body Mass Index (BMI) 65 kg/m 2 and impaired fasting glucose presented HH (glucose 41 mg/dL, C-peptide 27,3 ng/mL, insulin 637 μU/mL) and weight gain. MRI showed a 3,0 cm hypervascularity nodule between the body and pancreas tail. Octreotide, diazoxide, dextrose continuous infusion pump and hydrochlorothiazide were prescribed to control hypoglycemia. Partial pancreatectomy and splenectomy were performed but in the postoperative period, patient developed renal failure, sepsis, acute respiratory distress syndrome and death. Case 3: A 21 years old woman with severe obesity (BMI 60 kg/m 2 ), after using intragastric balloon for 6 months, developed hypoglycemic symptoms, including seizures. The PFT showed plasma glucose 44 mg/dl, plasma insulin 47 μU/mL, C-peptide 3,2 ng/mL. MRI showed a 1.9 cm hypervascularity nodule in the cephalic portion of the pancreas, very close to cava vein. Diazoxide was prescribed with improvement of hypoglycemia. Due to the high surgical risk given the location of the tumor and obesity, an endoscopic ultrasound (EUS) guided ethanol ablation was performed. After the procedure, diazoxide was discontinued and two months later she had no hypoglycemia and is still loosing weight. Antiinsulin antibodies were negative and all biopsies confirmed insulinoma. Conclusion: Insulinomas can be a challenging diagnose and management. Once, most of the patients are overweight, the surgical treatment became a threat , therefore advanced techniques are an important advance for reducing mortality.