PANCREATODUODENECTOMY IN PATIENT WITH VON HIPPEL-LINDAU DISEASE: A LITERATURE REVIEW.

BACKGROUND: The von Hippel-Lindau disease is a highly penetrant autosomal dominant syndrome characterized by tumor predisposition in different organs. AIM: This study aimed to describe a case of a pancreatoduodenectomy for a 30-year-old male patient with von Hippel-Lindau disease. METHODS: We present a case study and the literature review aiming at the state-of-the-art management of a patient with pheochromocytoma, capillary hemangioblastoma in the peripheral retina, and two neuroendocrine tumors in the pancreas. RESULTS: A larger pancreatic lesion was located in the uncinate process, measuring 31 mm. The smaller lesion was located in the proximal pancreas and was detected only on the positron emission tomography-computed tomography scan with DOTATOC-68Ga. Genetic investigation revealed a mutation in the locus NM_000551.3 c.482G>A (p.Arg161Gln) of the Von Hippel-Lindau Human Suppressor gene. The uncinate process tumor was larger than 30 mm and the patient had a mutation on exon 3; therefore, we indicated a pancreatoduodenectomy involving the proximal pancreas to resect both tumors en bloc. During the postoperative period, the patient presented a peripancreatic fluid collection, which was treated as a grade B pancreatic fistula with clinical resolution of the complication. On postoperative day 21, he was discharged home. CONCLUSION: The management of patients with von Hippel-Lindau disease and pancreatic neuroendocrine tumors is complex and must be centered on tertiary institutions with a large volume of pancreatic surgery. Although the current literature assists in decision-making in most situations, each step of the treatment requires analysis and discussion between different medical specialties, including surgeons, clinicians, radiologists, and anesthesiologists.

Laparoscopic Pancreatectomy in Rats: The Development of an Experimental Model.

INTRODUCTION: several pancreatectomy techniques in rats have been described and utilized for research concerning the pancreas endocrine and exocrine functions. However, we did not find a description of any kind of laparoscopic pancreatectomy in rats in the consulted databases. The objective of this study is to describe a laparoscopic splenic lobe pancreatectomy in rats. METHODS: ten Wistars rats weighting more then 300 g were operated for standardizing the laparoscopic pancreatosplenectomy technique, aided by previous descriptions of laparoscopic splenectomy and open pancreatectomy in rats. Adjustments have been progressively adopted for technical refinement. RESULTS: In five animals a low-cost rat laparoscopic set was used. In other five animals we used a standard laparoscopic set. Three rats died early due to different causes: transection of the gastroesophageal junction, hemorrhagic shock and inadvertent colonic injury. The postoperative period of the other seven rats was uneventful until the seventh postoperative day. DISCUSSION: laparoscopic distal pancreatectomy and splenectomy in rats is feasible and safe, even with a low-cost set, in which the results were alike the standard laparoscopic set.

Pancreatoduodenectomy in patient with von hippel-lindau disease: a literature review / Pancreaticoduodenectomia em paciente com doença de von hippel-lindau - revisão da literatura

ABSTRACT BACKGROUND: The von Hippel-Lindau disease is a highly penetrant autosomal dominant syndrome characterized by tumor predisposition in different organs. AIM: This study aimed to describe a case of a pancreatoduodenectomy for a 30-year-old male patient with von Hippel-Lindau disease. METHODS: We present a case study and the literature review aiming at the state-of-the-art management of a patient with pheochromocytoma, capillary hemangioblastoma in the peripheral retina, and two neuroendocrine tumors in the pancreas. RESULTS: A larger pancreatic lesion was located in the uncinate process, measuring 31 mm. The smaller lesion was located in the proximal pancreas and was detected only on the positron emission tomography-computed tomography scan with DOTATOC-68Ga. Genetic investigation revealed a mutation in the locus NM_000551.3 c.482G>A (p.Arg161Gln) of the Von Hippel-Lindau Human Suppressor gene. The uncinate process tumor was larger than 30 mm and the patient had a mutation on exon 3; therefore, we indicated a pancreatoduodenectomy involving the proximal pancreas to resect both tumors en bloc. During the postoperative period, the patient presented a peripancreatic fluid collection, which was treated as a grade B pancreatic fistula with clinical resolution of the complication. On postoperative day 21, he was discharged home. CONCLUSION: The management of patients with von Hippel-Lindau disease and pancreatic neuroendocrine tumors is complex and must be centered on tertiary institutions with a large volume of pancreatic surgery. Although the current literature assists in decision-making in most situations, each step of the treatment requires analysis and discussion between different medical specialties, including surgeons, clinicians, radiologists, and anesthesiologists.

RESUMO RACIONAL: A doença de von Hippel Lindau é uma síndrome autossômica dominante que se caracteriza por maior incidência de tumores em diferentes órgãos. OBJETIVO: Descrever um caso de pancreatoduodenectomia em paciente do sexo masculino de 30 anos com von Hippel Lindau. MÉTODO: Apresentamos o caso e a revisão da literatura realizada para otimizar o manejo do paciente, que apresentava feocromocitoma, hemangioblastoma capilar na retina periférica e dois tumores neuroendócrinos no pâncreas. RESULTADOS: O maior tumor pancreático localizava-se no processo uncinado medindo 31 mm. A lesão menor estava localizada no corpo proximal do pâncreas e foi detectada apenas na tomografia computadorizada por emissão de pósitrons com DOTATOC-68Ga. A investigação genética revelou uma mutação no locus NM_000551.3 c.482G>A (p.Arg161Gln) do gene supressor humano de Von Hippel-Lindau. O tumor no processo era maior que 30mm e o paciente apresentava mutação no exon 3. Indicamos pancreatoduodenectomia envolvendo o corpo proximal do pâncreas para ressecar em bloco ambos os tumores. No pós-operatório o paciente apresentou coleção líquida peripancreática que foi tratada como fístula pancreática grau B, com resolução clínica da complicação. Ele recebeu alta hospitalar no vigésimo primeiro dia pós-operatório. CONCLUSÕES: o manejo de pacientes com doença de von Hippel Lindau e tumores neuroendócrinos pancreáticos é complexo e deve ser centrado em instituições terciárias com grande volume de cirurgia pancreática. Embora a literatura atual auxilie na tomada de decisão na maioria das situações, cada etapa do tratamento requer análise e discussão entre diferentes especialidades médicas, incluindo cirurgiões, clínicos, radiologistas e anestesiologistas.

A technical note on low cost rat laparoscopy an initial experience.

PURPOSE: To evaluate a novel and adapted low-cost set model for laparoscopic surgery in rats. METHODS: Nine Wistar rats underwent two different laparoscopic procedures, splenectomy (n=3) and distal pancreatectomy with splenectomy (n = 6), after assembling a low-cost set replacing the conventional one (monitor, micro camera, image processor, light source, laparoscope and insufflator). The new set included an Android Tablet 10.5 ", a 5mm USB Endoscope and semiautomatic sphygmomanometer monitor. RESULTS: The same surgeon performed the laparoscopic procedures. Total surgical time ranged from 36 to 60 minutes with a mean of 45.8 minutes. Three rats died during the distal pancreatic and splenectomy procedure (33.3%), due to respiratory failure (n = 1), uncontrolled abdominal hemorrhage (n=1) and iatrogenic gastric perforation (n = 1). We followed the other six rats (66.6%) for seven days with no further evidence of complications. CONCLUSIONS: The laparoscopic partial pancreatectomy and splenectomy can be performed with the novel low-cost set assembled in the present experimental study. Both specific training and skills development are required to validate more advanced laparoscopic procedures and achieve a desirable outcome.

A technical note on low cost rat laparoscopy an initial experience

Abstract Purpose: To evaluate a novel and adapted low-cost set model for laparoscopic surgery in rats. Methods: Nine Wistar rats underwent two different laparoscopic procedures, splenectomy (n=3) and distal pancreatectomy with splenectomy (n = 6), after assembling a low-cost set replacing the conventional one (monitor, micro camera, image processor, light source, laparoscope and insufflator). The new set included an Android Tablet 10.5 ", a 5mm USB Endoscope and semiautomatic sphygmomanometer monitor. Results: The same surgeon performed the laparoscopic procedures. Total surgical time ranged from 36 to 60 minutes with a mean of 45.8 minutes. Three rats died during the distal pancreatic and splenectomy procedure (33.3%), due to respiratory failure (n = 1), uncontrolled abdominal hemorrhage (n=1) and iatrogenic gastric perforation (n = 1). We followed the other six rats (66.6%) for seven days with no further evidence of complications. Conclusions: The laparoscopic partial pancreatectomy and splenectomy can be performed with the novel low-cost set assembled in the present experimental study. Both specific training and skills development are required to validate more advanced laparoscopic procedures and achieve a desirable outcome.

Ischemia-reperfusion rat model of acute pancreatitis: protein carbonyl as a putative early biomarker of pancreatic injury.

Acute pancreatitis (AP) is an inflammatory disorder that can affect adjacent and/or remote organs. Some evidence indicates that the production of reactive oxygen species is able to induce AP. Protein carbonyl (PC) derivatives, which can also be generated through oxidative cleavage mechanisms, have been implicated in several diseases, but there is little or no information on this biomarker in AP. We investigated the association between some inflammatory mediators and PC, with the severity of ischemia-reperfusion AP. Wistar rats (n = 56) were randomly assigned in the following groups : control; sham, 15- or 180-min clamping of splenic artery, with 24 or 72 h of follow-up. The relationships between serum level of PC and thiobarbituric acid reactive species (TBARS) to myeloperoxidase (MPO) activity in tissue homogenates and to cytokines in culture supernatants of pancreatic samples were analyzed. MPO activity was related to the histology scores and increased in all clamping groups. Tumor necrosis factor-alpha (TNF-α), interleukin 1 beta (IL-1ß), and interleukin-6 were higher in the 180-min groups. Significant correlations were found between MPO activity and the concentrations of TNF-α and IL-1ß. PC levels increased in the 15-min to 24-h group. TBARS levels were not altered substantially. MPO activity and TNF-α and IL-1ß concentrations in pancreatic tissue are correlated with AP severity. Serum levels of PC appear to begin to rise early in the course of the ischemia-reperfusion AP and are no longer detected at later stages in the absence of severe pancreatitis. These data suggest that PC can be an efficient tool for the diagnosis of early stages of AP.