Ricardo Jureidini

Penetrance of functioning and nonfunctioning pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 in the second decade of life.

CONTEXT Data are scarce on the penetrance of multiple endocrine neoplasia type 1 (MEN1)-related nonfunctioning pancreatic neuroendocrine tumors (NF-PETs) and insulinomas in young MEN1 patients. A potential positive correlation between tumor size and malignancy (2-3 cm, 18%; >3 cm, 43%) has greatly influenced the management of MEN1 adults with NF-PETs. OBJECTIVE The aim of the study was to estimate the penetrance of NF-PETs, insulinomas, and gastrinomas in young MEN1 carriers. DESIGN The data were obtained from a screening program (1996-2012) involving 113 MEN1 patients in a tertiary academic reference center. PATIENTS Nineteen MEN1 patients (aged 12-20 y; 16 patients aged 15-20 y and 3 patients aged 12-14 y) were screened for NF-PETs, insulinomas, and gastrinomas. METHODS Magnetic resonance imaging/computed tomography and endoscopic ultrasound (EUS) were performed on 10 MEN1 carriers, magnetic resonance imaging/computed tomography was performed on five patients, and four other patients underwent an EUS. RESULTS The overall penetrance of PETs during the second decade of life was 42% (8 of 19). All eight PET patients had NF-PETs, and half of those tumors were multicentric. One-fifth of the screened patients (21%; 4 of 19) harbored at least one large tumor (>2.0 cm). Insulinoma was detected in two NF-PET patients (11%) at the initial screening; gastrinoma was not present in any cases. Six of the 11 (54%) screened patients aged 15-20 years who underwent an EUS had NF-PETs. Potential false-positive EUS results were excluded based on EUS-guided biopsy results, the reproducibility of the NF-PET findings, or the observation of increased tumor size during follow-up. Distal pancreatectomy and the nodule enucleation of pancreatic head tumors were conducted on three patients with large tumors (>2.0 cm; T2N0M0) that were classified as grade 1 neuroendocrine tumors (Ki-67<2%). CONCLUSIONS Our data demonstrated high penetrance of NF-PETs in 15- to 20-year-old MEN1 patients. The high percentage of the patients presenting consensus criteria for surgery for NF-PET alone or NF-PET/insulinoma suggests a potential benefit for the periodic surveillance of these tumors in this age group.

Unusual clinical presentation of annular pancreas in the adult

Annular pancreas (AP) is a rare congenital anomaly, usually present in childhood, with symptoms due to duodenal obstruction; however, this condition can manifest in adulthood with abdominal pain, pancreatitis and pancreatic head mass. The authors present a case of AP observed in a 22-year-old patient that presented an unusual dual-phase clinical manifestation of duodenal obstruction in infancy that was treated by a duodenojejunostomy, and abdominal pain due to chronic pancreatitis in the adult age. MRI with cholangiopancreatography played a decisive role in achieving the correct diagnosis. The patient was treated by a pylorus-preserving Whipple procedure, with resection of the previous duodenojejunostomy. Pancreatic changes characteristic of chronic pancreatitis were demonstrated both in the AP and in the resected pancreatic segment. A marked biliopancreatic ductal anomaly not previously described in the literature was demonstrated by radiologic examination of the surgical specimen. The pathogenesis of AP, the importance of its association with benign and malignant pancreatic disease and the treatment alternatives are discussed by the authors.

A modified laparoscopic sleeve gastrectomy for the treatment of diabetes mellitus type 2 and metabolic syndrome in obesity

BACKGROUND Ghrelin is a gastrointestinal peptide hormone (a 28-amino acid peptide) produced primarily by X/A cells in the oxyntic glands of the stomach fundus and cells lining the duodenum cavern. It suppresses insulin secretion and action and commands a significant role in regulating food intake. The aim of the present study was to show that modified laparoscopic sleeve gastrectomy (MLSG), in which a significant part of the gastric fundus and body of the stomach is removed up to 1 inch from the pylorus vein, may contribute to decreasing circulating ghrelin levels. METHODS A study population consisting of 150 individuals was monitored after undergoing a MLSG, with individuals chosen based on a documented history of diabetes mellitus type 2 and metabolic syndrome, clinical results determining a body mass index (BMI) of 35 to 60 kg/m(2), peptide C level greater than 1, negative anti-glutamic acid decarboxylase, negative anti-insulin, and confirmed stability of drug/insulin treatment and glycosylated hemoglobin greater than 6.5% for at least 24 and 3 months, respectively, before enrollment. RESULTS Twenty-four months after surgery, 150 patients (86.6%) presented with normal glycemic levels between 77 and 99 mg/dL. All patients improved average serum insulin levels by 9 mU/L and average glycosylated hemoglobin levels by 5.1% (normal range, 4%-6%). All patients tested negative for Helicobacter pylori and stopped using insulin, with 3 patients prescribed twice-daily use of an oral hypoglycemiant. In 14% of cases, patients experienced partial hair loss with low serum zinc levels and were prescribed oral zinc reposition and topical hair stimulants. The average weight loss recorded was 44.6% for patients with a BMI less than 45 kg/m(2) and 58% for patients with a BMI greater than 50 kg/m(2). CONCLUSIONS The MLSG is a safe procedure with a low morbidity rate (2.7%) (4 cases of fistula and 2 of bleeding) and no surgical mortality in this study. This surgery can promote control of diabetes mellitus type 2 and aid the treatment of exogenous overweight and morbidly obese individuals. The results of this study show that only through resection of the ghrelin-producing gastric area can most obesity cases and diabetes type II conditions be reverted to nonobese and controlled diabetes.

Intestinal intussusception and occlusion caused by small bowel polyps in the Peutz-Jeghers syndrome. Management by combined intraoperative enteroscopy and resection through minimal enterostomy: case report

UNLABELLED The Peutz-Jeghers syndrome is a hereditary disease that requires frequent endoscopic and surgical intervention, leading to secondary complications such as short bowel syndrome. CASE REPORT This paper reports on a 15-year-old male patient with a family history of the disease, who underwent surgery for treatment of an intestinal occlusion due to a small intestine intussusception. DISCUSSION An intra-operative fiberscopic procedure was included for the detection and treatment of numerous polyps distributed along the small intestine. Enterotomy was performed to treat only the larger polyps, therefore limiting the intestinal resection to smaller segments. The postoperative follow-up was uneventful. CONCLUSION We point out the importance of conservative treatment for patients with this syndrome, especially those who will undergo repeated surgical interventions because of clinical manifestation while they are still young.

MINIMALLY INVASIVE SURGERY FOR PSEUDOPAPILLARY NEOPLASM OF THE PANCREAS

ABSTRACT Background: Solid pseudopapillary pancreatic neoplasia is usually a large well-circumscribed pancreatic mass, with cystic and solid areas more frequently found in young women. It is a benign pancreatic neoplasia in most cases, therefore minimally invasive surgery could be an interesting approach. Aim: Evaluate the results of minimally invasive surgery for this neoplasia. Methods: Patients with this tumor who underwent minimally invasive pancreatectomies between 2009 and 2015 in a single institution, were analyzed regarding demographic, clinical-pathological futures, post-operative morbidity and disease-free survival. Results: All were women, and their median age was 39 (18-54) years. Two patients with tumor in the head of the pancreas underwent laparoscopic pancreaticoduodenectomy, and another one underwent laparoscopic enucleation. Two patients with tumor in the neck underwent central pancreatectomy. Distal pancreatectomies were performed in the other five, one with splenic preservation. None required blood transfusion or conversion to open surgery. Two (20%) developed clinical relevant pancreatic fistulas, requiring readmission. Median length of postoperative hospital stay was five days (2-8). All resection margins were negative. Patients were followed for a median of 38 months (14-71), and there was no recurrence. Conclusions: Minimally invasive surgery for solid pseudopapillary pancreatic neoplasia is feasible for tumors in different locations in the pancreas. It was associated with acceptable morbidity and respected the oncologic principles for treatment.

Transduodenal Endosonography-Guided Biliary Drainage and Duodenal Stenting for Palliation of Malignant Obstructive Jaundice and Duodenal Obstruction

CONTEXT Endosonography-guided biliary drainage has been used over the last few years as a salvage procedure when endoscopic retrograde cholangiopancreatography fails. Malignant gastric outlet obstruction may also be present in these patients. We report the results of both procedures during the same session in patients with duodenal and biliary obstruction due to malignant disease. METHODS A retrospective review from a prospective collected database. RESULTS Technical success was achieved in all five patients; however, only three patients experienced relief of jaundice and gastric outlet obstruction. CONCLUSIONS Endosonography-guided biliary drainage and duodenal stenting in the same session is feasible. However, severe complications may limit the procedure. This is a challenging procedure and should be done by experts with special attention to patients selection.

Tumor primitivo ectodérmico pancreático: relato de caso

Os tumores neuroectodermicos primitivos (PNETs) sao pequenas celulas redondas de tumores malignos classificadas como parte da familia do sarcoma de Ewing e representa aproximadamente 1% de todos as sarcomas1. Predominantemente ocorrendo em tecidos moles, ao longo das extremidades, eles tambem tem sido relatados em uma variedade de orgaos, tais como rim, bexiga, testiculo, ovario, utero, coracao e pulmao2,3. PNETs pancreaticos (PPNET) sao extremamente raros e precisam ser distinguidos de carcinomas neuroendocrinos, carcinoma indiferenciado de celulas pequenas, pancreatoblastomas e tumores pancreaticos. O conhecimento sobre PPNET e escasso; apenas 17 relatos podem ser encontrados na literatura. Aqui e descrito o caso de uma mulher de 25 anos com uma massa solida-cistica na cabeca do pâncreas, que mais tarde revelou ser PPNET.

Evaluation of microvessel density and p53 expression in pancreatic adenocarcinoma

OBJECTIVE: To evaluate the prognostic significance of microvessel density and p53 expression in pancreatic cancer. METHODS: Between 2008 and 2012, 49 patients with pancreatic adenocarcinoma underwent resection with curative intention. The resected specimens were immunohistochemically stained with anti-p53 and anti-CD34 antibodies. Microvessel density was assessed by counting vessels within ten areas of each tumoral section a highpower microscope. RESULTS: The microvessel density ranged from 21.2 to 54.2 vessels/mm2. Positive nuclear staining for p53 was found in 20 patients (40.6%). The overall median survival rate after resection was 24.1 months and there were no differences in survival rates related to microvessel density or p53 positivity. Microvessel density was associated with tumor diameter greater than 3.0 cm and with R0 resection failure. CONCLUSIONS: Microvessel density was associated with R1 resection and with larger tumors. p53 expression was not correlated with intratumoral microvessel density in pancreatic adenocarcinoma.

Advanced age does not increase morbidity in pancreaticoduodenectomy

Background Due to rising life expectancy of population, very complex surgical procedures such as pancreaticoduodenectomy (PD), are more commonly being performed in elderly patients. The objective of this study was to evaluate the safety of PD in patients older than 75 years old and the risk factors associated with severe complications. Methods Patients who underwent PD for periampullary tumors were retrospectively reviewed and divided into two groups, A (<75 years) and B (>75 years). The primary endpoint was severe postoperative complications (Clavien 3 or greater). Secondary endpoints were mortality and any complications. Results Ninety consecutive patients underwent PD for periampullary tumors in the studied period, 70 in group A and 20 in group B. Clinical and pathological characteristics of both groups were equivalents except for age. There were no difference regarding the incidence of severe complications (17% vs. 10%, P=0.72) or all complications (50% vs. 50%, P>0.999). Although mortality was greater in group A (8.6% vs. 0%), it was not statistically significant (P=0.333). On multivariate analyses, only male sex, obesity and American Society of Anesthesiology Score of 3 or 4 were associated with severe postoperative complications. Conclusions Poor clinical status, obesity and male gender are associated with severe complications in PD, but not age greater than 75 years, therefore PD can be considered safe in elderly patients and age itself should not be a contraindication for PD.

LAPSG Society News

The LAPSG is a multidisciplinary group dedicated to the study of the biology, pathophysiology and disease of the endocrine and exocrine pancreas. In particular the fi elds of gastroenterology, endocrinology, oncology, surgery, pharmacology, cell and molecular biology, nutrition, immunology and epidemiology will receive attention. It is also the study group’s aim to enhance the communication among scientists from different areas (especially the fi elds of gastroenterology and endocrinology) who otherwise rarely meet. The means to achieve these goals will be meetings of the members, postgraduate courses, clinical-research workshops and symposia. Pancreatologists and those working in related areas that live and work in Latin America, and who have published at least once during the last 5 years, are warmly invited to join the LAPSG. Members of the LAPSG will automatically become members of the International Association of Pancreatology (IAP) and the European Pancreatic Club (EPC) and will get a free online and print subscription to the journal Pancreatology.