A REVIEW TO HONOR THE HISTORICAL CONTRIBUTIONS OF PAULINE GROSS, ALDRED WARTHIN, AND HENRY LYNCH IN THE DESCRIPTION AND RECOGNITION OF INHERITANCE IN COLORECTAL CANCER.

The present manuscript aimed to review the historical development and most important contributions regarding Lynch Syndrome since its first description, more than a century ago. In 1895, a reputed pathologist from Michigan University, Dr. Aldred Scott Warthin, got intrigued by the family history of a local seamstress called Pauline Gross. According to her prevision, she would present an early death due to cancer, which actually happened (from the uterus). Historically, her family was designated "Family G", comprising a group recognized as the longest and most detailed cancer genealogy that has ever been studied. Warthin concluded that its members had genetic susceptibility for cancer, and they are, nowadays, considered the first reported Lynch Syndrome family. At that time, however, the medical cancer community was far less receptive to the association between heredity and cancer, despite the description of other families with similar heredograms. Unfortunately, this historical fact remained somewhat dormant until another investigator inaugurated a new era in the understanding of family cancer clusters. After reports and studies from this family and many others, the condition initially called Cancer Family Syndrome was changed to the eponym Lynch Syndrome. This was a recognition of the extensive and dedicated work developed by Dr. Henry Lynch in describing various characteristics of the disease, and his efforts to establish the correct recommendations for its diagnosis and treatment. Although the future announces there is still far to go for a complete understanding of Lynch Syndrome, the remarkable contributions of Pauline's intuition, Warthin's perseverance, and Lynch's work consistency must never be forgotten by those who already have or will still benefit from this knowledge.

Controversies in IPAA for Ulcerative Colitis: A Systematic Review of Different Anastomotic Techniques.

BACKGROUND: Available techniques for IPAA in ulcerative colitis include handsewn, double-stapled, and single-stapled anastomoses. There are controversies, indications, and different outcomes regarding these techniques. OBJECTIVE: To describe technical details, indications, and outcomes of 3 specific types of anastomoses in restorative proctocolectomy. DATA SOURCE: Systematic literature review for articles in the PubMed database according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria. STUDY SELECTION: Studies describing outcomes of the 3 different types of anastomoses, during pouch surgery, in patients undergoing restorative proctocolectomy for ulcerative colitis. INTERVENTION: IPAA technique. MAIN OUTCOME MEASURES: Postoperative outcomes (anastomotic leaks, overall complication rates, and pouch function). RESULTS: Twenty-one studies were initially included: 6 studies exclusively on single-stapled IPAA, 2 exclusively on double-stapled IPAA, 6 studies comparing single-stapled to double-stapled techniques, 6 comparing double-stapled to handsewn IPAA, and 1 comprising single-stapled to handsewn IPAA. Thirty-seven studies were added according to authors' discretion as complementary evidence. Between 1990 and 2015, most studies were related to double-stapled IPAA, either only analyzing the results of this technique or comparing it with the handsewn technique. Studies published after 2015 were mostly related to transanal approaches to proctectomy for IPAA, in which a single-stapled anastomosis was introduced instead of the double-stapled anastomosis, with some studies comparing both techniques. LIMITATIONS: A low number of studies with handsewn IPAA technique and a large number of studies added at authors' discretion were the limitations of this strudy. CONCLUSIONS: Handsewn IPAA should be considered if a mucosectomy is performed for dysplasia or cancer in the low rectum or, possibly, for re-do surgery. Double-stapled IPAA has been more widely adopted for its simplicity and for the advantage of preserving the anal transition zone, having lower complications, and having adequate pouch function. The single-stapled IPAA offers a more natural design, is feasible, and is associated with reasonable outcomes compared to double-stapled anastomosis. See video from symposium.

IMPACT OF THE COVID-19 PANDEMIC ON THE EMERGENCY SURGICAL TREATMENT OF COLORECTAL CANCER.

BACKGROUND: Colorectal cancer (CRC) is the most common malignancy of the gastrointestinal tract and the third most common type of cancer worldwide. The COVID-19 pandemic, during the years 2020 and 2022, increased the difficulties in offering adequate early diagnosis and treatment to CRC patients worldwide. During this period, it was only possible to treat patients who evolved with complications, mainly intestinal obstruction and perforation. AIMS: To assess the impact of the COVID-19 pandemic on the treatment of patients with CRC. METHODS: A review of data from a total of 112 patients undergoing emergency surgical treatment due to complications of CRC was carried out. Of these, 78 patients underwent emergency surgery during the COVID-19 pandemic (2020/2021), and 34 were treated before the pandemic (2018/2019). Ethnic aspects, clinical symptoms, laboratory tests, histopathological variables, intra and postoperative complications, and 90-day postoperative follow-up were compared between the two groups. RESULTS: Between the years 2018 and 2019, 79.4% (27/34) of patients had intestinal obstruction, while 20.6% (7/34) had intestinal perforation. During the period of the COVID-19 pandemic (2020/2021), 1.3% (1/78) of patients underwent surgery due to gastrointestinal bleeding, 6.4% (5/78) due to intestinal perforation, and 92.3% (72/78) due to intestinal obstruction. No statistically significant differences were recorded between the two groups in ethnic aspects, laboratory tests, type of complications, number of lymph nodes resected, compromised lymph nodes, TNM staging, pre or intraoperative complications, length of stay, readmission, or mortality rate. When considering postoperative tumor staging, among patients operated on in 2018/2019, 44.1% were classified as stage III and 38.2% as stage IV, while during the pandemic period, 28.2% presented stage III and 51.3% stage IV, also without a statistically significant difference between the two periods. Patients operated on during the pandemic had higher rates of vascular, lymphatic and perineural invasion. CONCLUSIONS: The COVID-19 pandemic increased the rate of complications related to CRC when comparing patients treated before and during the pandemic. Furthermore, it had a negative impact on histopathological variables, causing worse oncological prognoses in patients undergoing emergency surgery.

Mucin levels in glands of the colonic mucosa of rats with diversion colitis subjected to enemas containing sucralfate and n-acetylcysteine alone or in combination.

PURPOSE: To evaluate the tissue content of neutral and acidic mucins, sulfomucins and sialomucins in colonic glands devoid of intestinal transit after enemas containing sucralfate and n-acetylcysteine alone or in combination. METHODS: Sixty-four rats underwent intestinal transit bypass. A colonic segment was collected to compose the white group (without intervention). After derivation, the animals were divided into two groups according to whether enemas were performed daily for two or four weeks. Each group was subdivided into four subgroups according to the substance used: control group: saline 0.9%; sucralfate group (SCF): SCF 2 g/kg/day; n-acetylcysteine group (NAC): NAC 100 mg/kg/day; and SCF+NAC group: SCF 2 g/kg/day + NAC 100 mg/kg/day.Neutral and acidic mucins were stained by periodic acid-Schiff and alcian-blue techniques, respectively. The distinction between sulfomucins and sialomucin was made by the high alcian-blue iron diamine technique. The content of mucins in the colonic glands was measured by computerized morphometry. The inflammatory score was assessed using a validated scale. The results between the groups were compared by the Mann-Whitney's test, while the variation according to time by the Kruskal-Wallis' test (Dunn's post-test). A significance level of 5% was adopted. RESULTS: There was reduction in the inflammatory score regardless of the application of isolated or associated substances. Intervention with SCF+NAC increased the content of all mucin subtypes regardless of intervention time. CONCLUSIONS: The application of SCF+NAC reduced the inflammatory process of the colonic mucosa and increased the content of different types of mucins in the colonic glands of segments excluded from fecal transit.

MULTIPLE NEUROENDOCRINE NEOPLASIA IN A PATIENT WITH TYPE I NEUROFIBROMATOSIS (NF1): REPORT OF A NEW MUTATION (NF1, EXONS 2-30 DELETION) AND LITERATURE REVIEW.

BACKGROUND: Plexiform neurofibromas represent a common neoplasia of type 1 neurofibromatosis in which neurofibromas arise from multiple nerves involving connective tissue and skin and rarely affect the colon and rectum. Co-occurrence of plexiform neurofibromas, neuroendocrine tumors with primary involvement of the rectum, and medullary thyroid carcinoma in patients with neurofibromatosis type 1 is a previously undescribed condition. The aim of this manuscript was to present a case of primary plexiform neurofibroma and neuroendocrine tumors of the upper rectum in a patient with neurofibromatosis type 1 whose genetic sequencing found a novel mutation in the neurofibromatosis type 1 gene and to review the literature. CASE REPORT: A 49-year-old woman with a familial history of neurofibromatosis type 1 complained of abdominal cramps for 6 months. She had previously been submitted for a total thyroidectomy due to medullary thyroid carcinoma. She was submitted to a colonoscopy, which identified a submucosa lesion located in the upper rectum. The patient was referred for a laparoscopic rectosigmoidectomy, and the histopathological study of the surgical specimen identified two different tumors. An immunohistochemical panel was done for histopathological confirmation of the etiology of both lesions. The results of the panel showed intense immunoexpression of S100 protein in the largest and superficial lesion, as well as positivity for chromogranin and synaptophysin in the minor and deep lesion confirming the diagnosis of rectal plexiform neurofibromas concomitant with neuroendocrine tumors. The proliferative activity rate using Ki-67 antibodies showed that both tumors had a low rate of mitotic activity (<1%). Genetic sequence panel identified an undescribed mutation in the neurofibromatosis type 1 gene (deletion, exons 2-30). The patient's postoperative evolution was uneventful, and she remains well, without recurrence, 3 years after surgery. CONCLUSION: The co-occurrence of medullary thyroid carcinoma, plexiform neurofibromas, and neuroendocrine tumors of the rectum in patients with neurofibromatosis type 1 is an exceptional and undescribed possibility, whose diagnosis can be confirmed by the immunohistochemical staining and genetic panel.

PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS.

BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.

Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?

OBJECTIVE: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. AIM: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. METHODS: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. RESULTS: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. CONCLUSION: Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.

Mucin levels in glands of the colonic mucosa of rats with diversion colitis subjected to enemas containing sucralfate and n-acetylcysteine alone or in combination

ABSTRACT Purpose: To evaluate the tissue content of neutral and acidic mucins, sulfomucins and sialomucins in colonic glands devoid of intestinal transit after enemas containing sucralfate and n-acetylcysteine alone or in combination. Methods: Sixty-four rats underwent intestinal transit bypass. A colonic segment was collected to compose the white group (without intervention). After derivation, the animals were divided into two groups according to whether enemas were performed daily for two or four weeks. Each group was subdivided into four subgroups according to the substance used: control group: saline 0.9%; sucralfate group (SCF): SCF 2 g/kg/day; n-acetylcysteine group (NAC): NAC 100 mg/kg/day; and SCF+NAC group: SCF 2 g/kg/day + NAC 100 mg/kg/day.Neutral and acidic mucins were stained by periodic acid-Schiff and alcian-blue techniques, respectively. The distinction between sulfomucins and sialomucin was made by the high alcian-blue iron diamine technique. The content of mucins in the colonic glands was measured by computerized morphometry. The inflammatory score was assessed using a validated scale. The results between the groups were compared by the Mann-Whitney's test, while the variation according to time by the Kruskal-Wallis' test (Dunn's post-test). A significance level of 5% was adopted. Results: There was reduction in the inflammatory score regardless of the application of isolated or associated substances. Intervention with SCF+NAC increased the content of all mucin subtypes regardless of intervention time. Conclusions: The application of SCF+NAC reduced the inflammatory process of the colonic mucosa and increased the content of different types of mucins in the colonic glands of segments excluded from fecal transit.

Impact of the covid-19 pandemic on the emergency surgical treatment of colorectal cancer / Impacto da pandemia da covid-19 no tratamento cirúrgico emergencial do câncer colorretal

ABSTRACT BACKGROUND: Colorectal cancer (CRC) is the most common malignancy of the gastrointestinal tract and the third most common type of cancer worldwide. The COVID-19 pandemic, during the years 2020 and 2022, increased the difficulties in offering adequate early diagnosis and treatment to CRC patients worldwide. During this period, it was only possible to treat patients who evolved with complications, mainly intestinal obstruction and perforation. AIMS: To assess the impact of the COVID-19 pandemic on the treatment of patients with CRC. METHODS: A review of data from a total of 112 patients undergoing emergency surgical treatment due to complications of CRC was carried out. Of these, 78 patients underwent emergency surgery during the COVID-19 pandemic (2020/2021), and 34 were treated before the pandemic (2018/2019). Ethnic aspects, clinical symptoms, laboratory tests, histopathological variables, intra and postoperative complications, and 90-day postoperative follow-up were compared between the two groups. RESULTS: Between the years 2018 and 2019, 79.4% (27/34) of patients had intestinal obstruction, while 20.6% (7/34) had intestinal perforation. During the period of the COVID-19 pandemic (2020/2021), 1.3% (1/78) of patients underwent surgery due to gastrointestinal bleeding, 6.4% (5/78) due to intestinal perforation, and 92.3% (72/78) due to intestinal obstruction. No statistically significant differences were recorded between the two groups in ethnic aspects, laboratory tests, type of complications, number of lymph nodes resected, compromised lymph nodes, TNM staging, pre or intraoperative complications, length of stay, readmission, or mortality rate. When considering postoperative tumor staging, among patients operated on in 2018/2019, 44.1% were classified as stage III and 38.2% as stage IV, while during the pandemic period, 28.2% presented stage III and 51.3% stage IV, also without a statistically significant difference between the two periods. Patients operated on during the pandemic had higher rates of vascular, lymphatic and perineural invasion. CONCLUSIONS: The COVID-19 pandemic increased the rate of complications related to CRC when comparing patients treated before and during the pandemic. Furthermore, it had a negative impact on histopathological variables, causing worse oncological prognoses in patients undergoing emergency surgery.

RESUMO RACIONAL: O câncer colorretal (CCR) é a doença maligna mais comum do trato gastrointestinal sendo o terceiro tipo de câncer mais comum em todo o mundo. A pandemia de COVID-19 durante os anos de 2020 e 2022 aumentou as dificuldades em se oferecer diagnóstico e tratamento precoce adequado aos pacientes com CCR em todo o mundo. Nesse período, só foi possível tratar os pacientes que evoluíram com complicações representadas, principalmente, pela obstrução e perfuração intestinal. OBJETIVOS: Avaliar o impacto da pandemia de COVID-19 no tratamento de pacientes com CCR. MÉTODOS: Foi realizada uma revisão dos dados de um total de 112 pacientes submetidos ao tratamento cirúrgico de urgência devido complicações do CCR. Destes, 78 pacientes foram submetidos a cirurgia de emergência durante o período da pandemia de COVID-19 (2020/2021), e 34 pacientes foram operados no período anterior à pandemia (2018/2019). Aspectos étnicos, sintomas clínicos, exames laboratoriais, variáveis histopatológicas, complicações intra e pós-operatórias e acompanhamento pós-operatório de 90 dias foram analisados comparando os dois grupos. RESULTADOS: Entre os anos 2018 e 2019, 79,4% (27/34) dos pacientes apresentaram obstrução intestinal enquanto 20,6% (7/34) perfuração intestinal. Durante o período da pandemia de COVID-19 (2020/2021) 1,3% (1/78) dos pacientes foram operados por hemorragia digestiva baixa, 6,4% (5/78) por perfuração intestinal e 92,3% (72/78) por obstrução intestinal. Não foram registradas diferenças estatisticamente significativas entre os dois grupos nos aspectos étnicos, exames laboratoriais, tipo de complicações, número de linfonodos ressecados, linfonodos comprometidos, estadiamento TNM, complicações pré ou intraoperatórias, tempo de internação, readmissão e taxa de mortalidade. Ao considerar o estadiamento tumoral pós-operatório, entre os pacientes operados em 2018/19, 44,1% foram classificados como estágio III e 38,2% como estágio IV, enquanto no período pandêmico, 28,2% apresentaram estágio III e 51,3% estágio IV, também sem diferença estatisticamente significativa entre os dois períodos. Doentes operados durante a pandemia apresentaram maiores índices de invasão vascular, linfática e perineural. CONCLUSÕES: A pandemia de COVID-19 aumentou as taxas de complicações relacionadas ao CCR, comparando pacientes tratados antes e durante a pandemia. Além disso, teve impacto negativo nas variáveis histopatológicas, causando piores prognósticos oncológicos em pacientes submetidos a cirurgias de emergência.

Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?

Abstract Objective: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. Aim: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. Methods: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. Results: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. Conclusion: Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.

IS IT POSSIBLE TO OPTIMIZE STAPLED HEMORRHOIDOPEXY OUTCOMES BY ENLARGING OPERATIVE CRITERIA INDICATIONS? RESULTS OF A TAILORED PROCEDURE WITH ASSOCIATED RESECTION IN A COMPARATIVE PERSONAL SERIES.

BACKGROUND: Since its introduction, stapled hemorrhoidopexy has been increasingly indicated in the management of hemorrhoidal disease. AIM: Our primary end point was to evaluate the incidence of recurrent disease requiring another surgical intervention. On a secondary analysis, we also compared pain, complications, and patient's satisfaction after a tailored surgery. METHODS: We retrospectively reviewed 196 patients (103 males and 93 females) with a median age of 47.9 years (range, 17-78) who were undergoing stapled hemorrhoidopexy alone (STG; n=65) or combined surgery (CSG; n=131, stapled hemorrhoidopexy associated with resection). RESULTS: Complications were detected in 11 (5.6%) patients (4.6% for STG vs. 6.1% for CSG; p=0.95). At the same time, symptoms recurrence (13.8% vs. 8.4%; p=034), reoperation rate for complications (3.1% vs. 3.0%; p=1.0), and reoperation rate for recurrence (6.1% vs. 4.6%; p=1.0) were not different among groups. Grade IV patients were more commonly managed with simultaneous stapling and resection (63% vs. 49.5%), but none of them presented symptoms recurrence nor need reoperation due to recurrence. Median pain score during the first week was higher in CSG patients (0.8 vs. 1.7). After a follow-up of 24.9 months, satisfaction scores were similar (8.6; p=0.8). CONCLUSION: Recurrent symptoms were observed in 10% of patients, requiring surgery in approximately half of them. Even though the association of techniques may raise pain scores, a tailored approach based on amplified indication criteria and combined techniques seems to be an effective and safe alternative, with decreased relapse rates in patients suffering from more advanced hemorrhoidal disease. Satisfaction scores after hemorrhoidopexy are high.

Tissue Content and Pattern of Expression of Claudin-3 and Occludin in Normal and Neoplastic Tissues in Patients with Colorectal Cancer.

BACKGROUND: Metastasis is the worst prognostic variable of patients with colorectal cancer (CRC). For the development of metastases, it is necessary that cancer cells detach from the primary tumor, migrate into the angiolymphatic system, and invade the tissue where they will develop. The breakdown of the tight junctions (TJs) plays an important role in colorectal metastatic tumors. Claudin-3 and occludin are the main component proteins of TJs. AIM: To analyze the expression and tissue content of claudin-3 and occludin in normal and neoplastic tissues of patients with metastatic CRC. METHODS: Fifty-seven consecutive patients with stage III and IV CRC were included. Fragments of neoplastic tissue were collected from the tumor margins, and samples of the normal tissue were collected from the same patient in a standardized distance of 10 cm from the cranial margin of the tumor. Immunohistochemistry technique was used to identify the tissue staining of claudin-3 and occludin. To measure the content of both proteins in cellular membranes of normal and cancer cells, a validated immunoscore was used. RESULTS: Claudin-3 and occludin in normal tissues are in the apical and lateral membranes of cells, while in the neoplastic, in cytoplasm. The mean of the tissue content of claudin-3 in the normal tissue was 2.57 ± 0.16, while in the neoplastic tissue was 1.03 ± 0.13. The contents of occludin were 2.77 ± 0.1 in normal tissue, while in the neoplastic were 1.08 ± 0.14. CONCLUSION: There is a reduction in the content of the claudin-3 and occludin in the cell membranes of the neoplastic tissue in patients with CRC.

Multiple Jejunal diverticulosis complicated by perforation: case report and a brief literature review

Background: Multiple small-bowel diverticulosis comprises a rare entity with probable underestimated incidence, and that may be the reason why it is sometimes overlooked when managing cases with peritonitis. Case report: In the present paper, we report the case of a 76-year-old male presenting abdominal pain and fever in an acute setting. Computed tomography (CT) scans revealed jejunal thickening and numerous images of saccular addition that were interpreted as jejunoileal diverticulitis. After an initial period of clinical treatment, surgical management was indicated based on a worsening clinical picture and the presence of an extraluminal focus of gas detected in a subsequent CT scan. Through a laparoscopic approach, multiple small-bowel diverticula and a tamponade perforation were found. A segmental intestinal resection was performed, and the patient was discharged after a ten days. Conclusions: Multiple jejunal diverticulosis is a rare condition that should be remembered in the setting of an acute abdomen. As it prevails among older patients, early diagnosis with radiological aid is crucial to establish the most adequate management, including intestinal resection, if necessary. (AU)

CAN SUCRALFATE ENEMA PREVENT COLITIS IN COLONIC SEGMENTS WITHOUT FECAL TRANSIT? / ENEMA DE SULCRALFATO PODE PREVENIR COLITE EM SEGMENTOS COLÔNICOS SEM TRÂNSITO FECAL?

AIM: Oxidative stress is one of the main mechanisms associated with the rupture of the defense mechanisms of the colonic epithelial barrier; it reduces the tissue content of the claudin-3 and occludin proteins, which are the main constituents of intercellular tight junctions. Sucralfate (SCF) has antioxidant activity and has been used to treat different forms of colitis. This study aimed to measure the tissue claudin-3 and occludin content of the colon mucosa without fecal transit, subjected to intervention with SCF. METHODS: Thirty-six rats were subjected to left colon colostomy and distal mucous fistula. They were divided into two groups according to euthanasia that was performed 2 or 4 weeks after the intervention. Each group was divided into three subgroups according to the enema applied daily: saline alone, SCF at 1 g/kg/day, or SCF at 2 g/kg/day. Colitis was diagnosed by the histological analysis adopting the previous validate scale. The tissue expression of both proteins was identified by immunohistochemical technique. The content of proteins was quantified by computer-assisted image analysis. RESULTS: The inflammatory score was high in colonic segments without fecal transit, and enemas with SCF reduced the inflammatory score in these segments, mainly in those animals submitted to intervention with SCF in greater concentration and for a longer period of intervention. There was an increase in tissue content of claudin-3 and occludin, related to SCF concentration. The tissue content of both proteins was not related to the intervention time. CONCLUSION: Enemas with SCF reduced the inflammation and increased the tissue content of claudin-3 and occludin in colonic mucosa without fecal stream.

OBJETIVO: O estresse oxidativo é um dos principais mecanismos associados à ruptura dos mecanismos de defesa que formam a barreira epitelial cólica e reduz o conteúdo tecidual das proteínas claudina-3 e ocludina principais constituintes das junções de oclusão intercelulares. O sucralfato, possui atividade antioxidante e tem sido usado para tratar diferentes formas de colite. Mensurar o conteúdo tecidual de claudina-3 e ocludina da mucosa do cólon sem trânsito fecal, submetido à intervenção com sucralfato. MÉTODO: Trinta e seis ratos foram submetidos à colostomia do cólon esquerdo e fístula mucosa distal. Os animais foram divididos em dois grupos de acordo com a eutanásia ser realizada duas ou quatro semanas após a intervenção. Cada grupo foi dividido em três subgrupos de acordo com o tipo de intervenção realizada diariamente: solução salina isolada; sucralfato a 1 g/kg/dia ou sucralfato a 2g/kg/dia. A colite foi diagnosticada por análise histológica adotando escala de validação prévia. A expressão tecidual de ambas as proteínas foi identificada por imunoistoquímica. O conteúdo das proteínas foi quantificado por análise de imagem assistida por computador. RESULTADOS: O escore inflamatório foi maior nos segmentos cólicos sem trânsito fecal e os enemas com sucralfato reduziram o escore inflamatório nesses segmentos, principalmente nos animais submetidos à intervenção com sucralfato em maior concentração e por período mais longo de intervenção. Houve aumento no conteúdo tecidual das proteínas claudina-3 e ocludina, relacionado com a concentração de sucralfato. O conteúdo tecidual de ambas as proteínas não se modificou com a duração da intervenção. CONCLUSÃO: Enemas com sucralfato reduzem a inflamação e aumentam o conteúdo tecidual de claudina-3 e ocludina na mucosa cólica sem trânsito intestinal.

Is it possible to optimize stapled hemorrhoidopexy outcomes by enlarging operative criteria indications? results of a tailored procedure with associated resection in a comparative personal series / é possível otimizar os resultados da hemorroidopexia grampeada pela ampliação dos critérios de indicação operatória? resultados de um procedimento personalizado com ressecção associada em uma série comparativa pessoal

ABSTRACT BACKGROUND: Since its introduction, stapled hemorrhoidopexy has been increasingly indicated in the management of hemorrhoidal disease. AIM: Our primary end point was to evaluate the incidence of recurrent disease requiring another surgical intervention. On a secondary analysis, we also compared pain, complications, and patient's satisfaction after a tailored surgery. METHODS: We retrospectively reviewed 196 patients (103 males and 93 females) with a median age of 47.9 years (range, 17-78) who were undergoing stapled hemorrhoidopexy alone (STG; n=65) or combined surgery (CSG; n=131, stapled hemorrhoidopexy associated with resection). RESULTS: Complications were detected in 11 (5.6%) patients (4.6% for STG vs. 6.1% for CSG; p=0.95). At the same time, symptoms recurrence (13.8% vs. 8.4%; p=034), reoperation rate for complications (3.1% vs. 3.0%; p=1.0), and reoperation rate for recurrence (6.1% vs. 4.6%; p=1.0) were not different among groups. Grade IV patients were more commonly managed with simultaneous stapling and resection (63% vs. 49.5%), but none of them presented symptoms recurrence nor need reoperation due to recurrence. Median pain score during the first week was higher in CSG patients (0.8 vs. 1.7). After a follow-up of 24.9 months, satisfaction scores were similar (8.6; p=0.8). CONCLUSION: Recurrent symptoms were observed in 10% of patients, requiring surgery in approximately half of them. Even though the association of techniques may raise pain scores, a tailored approach based on amplified indication criteria and combined techniques seems to be an effective and safe alternative, with decreased relapse rates in patients suffering from more advanced hemorrhoidal disease. Satisfaction scores after hemorrhoidopexy are high.

RESUMO RACIONAL: Desde sua introdução, a hemorroidopexia por grampeamento tem sido cada vez mais indicada no manuseio da doença hemorroidária. OBJETIVOS: Nosso objetivo primário foi avaliar a incidência de doença recidivada que requeira tratamento cirúrgico. Numa análise secundária, também comparamos dor, complicações e satisfação do paciente após uma operação ajustada a cada caso. MÉTODOS: Foram revistos retrospectivamente 196 pacientes (103 homens e 93 mulheres) com idade média de 47,9 anos (17-78) submetidos a hemorroidopexia mecânica isoladamente (STG; n=65) ou cirurgia combinada (CSG; n=131, hemorroidopexia por grampeamento com ressecção). RESULTADOS: Complicações foram detectadas 11 (5,6%) pacientes (4,6% para STG vs. 6,1% para CSG; p=0,95). Ao mesmo tempo, recidiva de sintomas (13,8% vs. 8,4%; p=034), reoperações por complicações (3,1% vs. 3,0%; p=1,0) ou por recidiva (6,1 vs. 4,6%; p=1,0) não foram diferentes entre os dois grupos. Pacientes com grau IV foram mais comumente manuseados com grampeamento e ressecção simultâneos (63% vs. 49,5%), mas nenhum deles apresentou recidiva ou necessitou reoperação. O escore médio de dor na primeira semana foi maior no grupo CSG (0,8 vs. 1,7). Após seguimento de 24,9 meses, os índices de satisfação foram similares (8,6; p=0,8). CONCLUSÕES: Sintomas de recidiva foram observados em 10%, requerendo cirurgia em aproximadamente metade dos doentes. Embora a associação de técnicas aumente os escores de dor, um procedimento sob medida baseado em critérios ampliados de indicação e técnicas combinadas parece ser uma alternativa efetiva e segura, com menor recidiva em pacientes portadores de doença hemorroidária mais avançada. Os escores de satisfação após hemorroidopexia são altos.

Pancreatic solid-pseudopapillary neoplasm in patients with familial adenomatous polyposis / Neoplasia sólida pseudopapilífera pancreática em pacientes com polipose adenomatosa familiar

ABSTRACT BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.

RESUMO RACIONAL: A neoplasia sólida pseudopapilífera do pâncreas é um tumor pancreático incomum, mais frequente em mulheres jovens. A polipose adenomatosa familiar, por sua vez, é uma condição genética associada a câncer colorretal e que também aumenta o risco de desenvolvimento de outros tumores. OBJETIVOS: Discutir a associação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera, que é bastante rara. MÉTODOS: Reportamos dois casos de pacientes com polipose adenomatosa familiar, um homem e uma mulher, que desenvolveram neoplasia sólida pseudopapilífera do pâncreas e foram submetidos a ressecção laparoscópica com preservação esplênica. RESULTADOS: As vias de sinalização da ß-catenina e Wnt tem um papel importante na tumorigênese da neoplasia sólida pseudopapilífera, e sua ativação constitutiva devido a inativação do gene adenomatous polyposis coli na polipose adenomatosa familiar pode explicar a relação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera. CONCLUSÕES: A ressecção do cólon deve ser priorizada, com preferência pela abordagem minimamente invasiva para minimizar o risco de desenvolvimento de tumor desmoide. A ressecção pancreática geralmente não requer linfadenectomia extensa para neoplasia sólida pseudopapilífera, portanto, a preservação esplênica é factível.

Multiple neuroendocrine neoplasia in a patient with type i neurofibromatosis (nf1): report of a new mutation (nf1, exons 2-30 deletion) and literature review / Neoplasia neuroendócrina múltipla em doente com neurofibromatose tipo 1 (nf1). descrição de uma nova mutação e revisão da literatura

ABSTRACT BACKGROUND: Plexiform neurofibromas represent a common neoplasia of type 1 neurofibromatosis in which neurofibromas arise from multiple nerves involving connective tissue and skin and rarely affect the colon and rectum. Co-occurrence of plexiform neurofibromas, neuroendocrine tumors with primary involvement of the rectum, and medullary thyroid carcinoma in patients with neurofibromatosis type 1 is a previously undescribed condition. The aim of this manuscript was to present a case of primary plexiform neurofibroma and neuroendocrine tumors of the upper rectum in a patient with neurofibromatosis type 1 whose genetic sequencing found a novel mutation in the neurofibromatosis type 1 gene and to review the literature. CASE REPORT: A 49-year-old woman with a familial history of neurofibromatosis type 1 complained of abdominal cramps for 6 months. She had previously been submitted for a total thyroidectomy due to medullary thyroid carcinoma. She was submitted to a colonoscopy, which identified a submucosa lesion located in the upper rectum. The patient was referred for a laparoscopic rectosigmoidectomy, and the histopathological study of the surgical specimen identified two different tumors. An immunohistochemical panel was done for histopathological confirmation of the etiology of both lesions. The results of the panel showed intense immunoexpression of S100 protein in the largest and superficial lesion, as well as positivity for chromogranin and synaptophysin in the minor and deep lesion confirming the diagnosis of rectal plexiform neurofibromas concomitant with neuroendocrine tumors. The proliferative activity rate using Ki-67 antibodies showed that both tumors had a low rate of mitotic activity (<1%). Genetic sequence panel identified an undescribed mutation in the neurofibromatosis type 1 gene (deletion, exons 2-30). The patient's postoperative evolution was uneventful, and she remains well, without recurrence, 3 years after surgery. CONCLUSION: The co-occurrence of medullary thyroid carcinoma, plexiform neurofibromas, and neuroendocrine tumors of the rectum in patients with neurofibromatosis type 1 is an exceptional and undescribed possibility, whose diagnosis can be confirmed by the immunohistochemical staining and genetic panel.

RESUMO RACIONAL: Os neurofibromas plexiformes representam uma neoplasia frequente na neurofibromatose tipo 1 onde os neurofibromas surgem de múltiplos nervos envolvendo, também, o tecido conjuntivo e a pele raramente acometendo o cólon e o reto. A co-ocorrência de neurofibromas plexiformes, tumores neuroendócrinos, com envolvimento primário do reto, e carcinoma medular de tireoide em pacientes com neurofibromatose tipo 1 é uma condição ainda não descrita. O objetivo deste manuscrito é descrever um caso de neurofibromas plexiformes e tumores neuroendócrinos primários do reto superior, em paciente com neurofibromatose tipo 1, cujo sequenciamento genético identificou uma nova mutação no gene neurofibromatose tipo 1 e revisar a literatura sobre o tema. DESCRIÇÃO DO CASO: Mulher de 49 anos, com história familiar de neurofibromatose tipo 1 queixava-se de cólicas abdominais há seis meses. Tinha sido previamente submetida a tireoidectomia total por carcinoma medular de tireoide. Foi submetida a colonoscopia, que identificou lesão submucosa localizada no reto superior. A doente foi encaminhada para retossigmoidectomia laparoscópica, cujo estudo histopatológico do espécime cirúrgico identificou dois tumores distintos. Realizou-se painel imuno-histoquímico para confirmação histopatológica da etiologia de ambas as lesões. O resultado do painel mostrou intensa imunoexpressão da proteína S-100 da lesão superficial e de maior tamanho,e positividade para cromogranina e somatofisina da lesão mais profunda e de menores dimensões confirmando o diagnóstico de neurofibromas plexiformes retal e tumores neuroendócrinos. O índice de atividade proliferativa utilizando o anticorpo ant-Ki-67 mostrou que ambos os tumores apresentavam baixa índice mitótico (<1%). O painel genético identificou mutação no gene neurofibromatose tipo 1 (deleção, exons 2-30) ainda não descrita. A doente apresentou evolução pós-operatória sem intercorrências, permanecendo bem, sem recorrência, três anos após a cirurgia. CONCLUSÕES: A co-ocorrência de carcinoma medular de tireoide, neurofibromas plexiformes e tumores neuroendócrinos do reto em pacientes com neurofibromatose tipo 1 é uma possibilidade excepcional e ainda não descrita, cujo diagnóstico pode ser confirmado pela coloração imuno-histoquímica e painel genético.