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INTRODUCTION: One of the topics that show differences of opinion in the scientific field of nutrition is the recommendation by clinical practice guidelines (CPGs) of an immunomodulatory diet with arginine, nucleotides and omega-3 for individuals diagnosed with cancer undergoing major surgery. The quality of the recommendations is directly related to credibility, transparency and rigour in their development, but also to the quality of the studies published and available for inclusion in the recommendation, such as systematic reviews (SRs) and randomised clinical trials. The aim of this study is to evaluate the methodological quality of the recommendation of perioperative immunomodulatory supplementation for individuals with gastrointestinal and head and neck cancer, the CPGs, and the studies that support the recommendations. METHODS AND ANALYSIS: We will conduct a systematic search for CPGs. Recommendations for nutritional supplementation with immunomodulatory substrates for individuals undergoing major oncological surgery will be analysed using the Appraisal of Guidelines Research and Evaluation-Recommendations Excellence tool. CPGs will be analysed using the Appraisal of Guidelines Research and Evaluation II tool. The SRs cited in the recommendations will be analysed using the A Measurement Tool to Assess Systematic Reviews II tool and additional questions regarding heterogeneity in reviews. The clinical trials cited in the SRs and in the guideline recommendations (when applicable) will be analysed according to questions regarding heterogeneity in trials. The results will be presented in tables or charts using descriptive analyses. ETHICS AND DISSEMINATION: The results of this study will be disseminated through relevant conferences and peer-reviewed journals. PROTOCOL REGISTRATION NUMBER: 10.17605/OSF.IO/X2GYT.

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El tumor neuroectodérmico maligno del tracto gastrointestinal es una neoplasia rara con pocos casos reportados en la literatura, especialmente en América Latina. Descrito por primera vez en 2003, se trata de una entidad sin tratamiento estandarizado y de pobre pronóstico. Se presenta el caso de una paciente de 22 años de edad que acude a la consulta por dolor abdominal, anemia y masa abdominal palpable. Luego de estudios pertinentes se decide la conducta resectiva y el posterior tratamiento oncológico. (AU)

Malignant gastrointestinal neuroectodermal tumor (GNET), formerly known as clear cell sarcoma of the gastrointestinal tract, is an extremely rare tumor of mesenchymal origin, which presents great microscopic and molecular similarity to clear cell sarcoma found in other parts of the body, such as tendons and aponeurosis. It is characterized by its rapid evolution, high recurrence rate and frequent diagnosis as metastatic disease.1,2 (AU)

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Introdução: O primeiro sistema robótico utilizado em procedimentos cirúrgicos foi desenvolvido em 1994. Os primeiros pacientes do Sistema Único de Saúde do Brasil (SUS) foram beneficiados com essa tecnologia apenas a partir de 2012, após a instalação da plataforma robótica no Instituto Nacional de Câncer (INCA). Esse estudo avalia 10 anos de experiência do programa robótico na referida instituição. Método: Todos os procedimentos robóticos realizados pelo serviço de cirurgia abdominopélvica dessa instituição entre julho de 2012 a dezembro de 2022 foram analisados. Os dados foram coletados a partir do banco de dados do programa de robótica e por revisão de prontuários físicos e eletrônicos. Resultados: Foram realizados 280 procedimentos cirúrgicos robóticos. A maioria dos casos operados foi conduzida pelo grupo de cirurgia colorretal, correspondendo a 87,5% do total, sendo a ressecção anterior de reto a cirurgia mais realizada. Os demais casos foram operados pelo grupo hepatobiliar (4,6%), estômago (4,2%) e esôfago (3,6%). A taxa de conversão para cirurgia aberta foi de 10,3%. O período médio de internação foi inferior a 10 dias e 9,6% dos pacientes apresentaram reinternação dentro de 90 dias após a cirurgia. Do total, 43,5% dos pacientes foram submetidos a tratamento neoadjuvante com quimioterapia e/ou radioterapia. Durante o período analisado, 18,5% dos pacientes evoluíram com recidiva de doença, sendo a sobrevida global em 5 anos de aproximadamente 60%. Conclusão: Nos últimos 10 anos o uso da tecnologia robótica cresceu significativamente, promovendo melhora dos resultados clínicos. O programa de cirurgia robótica do INCA para tratamento do câncer foi o pioneiro no âmbito nacional dentro do SUS e possibilitou a divulgação e consolidação desta excelente ferramenta tecnológica no tratamento do câncer gastrointestinal. Os resultados apresentados revelam o êxito do programa, demonstrando taxa de complicações aceitável, tempo de internação reduzido e resultados oncológicos satisfatórios, disponibilizando aos pacientes do SUS tratamento cirúrgico atualizado e eficaz.

Introduction: The first robotic system used in surgical procedures was developed in 1994. In 2012, the first robotic surgeries were performed in the Brazilian Public Health System (Sistema Único de Saúde - SUS), after the installation of the robotic platform at Brazilian National Cancer Institute (INCA). This study evaluates 10 years of experience with the robotic program at this Center. Method: All robotic procedures performed by the abdomino-pelvic surgery service of this center between july 2012 and december 2022 were reviewed. Data were collected from the database of the robotics program and review of physical and electronic medical records. Results: 280 robotic surgical procedures were performed. Most cases were conducted by the colorectal surgery group, corresponding to 87.5% of the cases, with anterior resection of the rectum being the most common procedure. The remaining cases were operated by the hepatobiliary group (4.6%), stomach group (4.2%) and esophagus group (3.6%). The conversion rate to open surgery was 10.3%. The average length of stay was less than 10 days and 9.6% of patients were readmitted within 90 days following surgery. Of all, 43.5% of the patients underwent neoadjuvant treatment with chemotherapy and/or radiotherapy. During the analyzed period, 18.5% of the patients had disease recurrence, with an overall 5-year survival of approximately 60%. Conclusion: In the last 10 years, the use of robotic technology has grown significantly, promoting improved clinical outcomes. INCA's robotic surgery program was a pioneer on the national scene within the SUS and enabled the dissemination and consolidation of this excellent technological tool in treatment of gastrointestinal cancer. The results presented reveal the success of the program, demonstrating an acceptable complication rate, reduced length of stay and satisfactory oncological results, providing SUS patients with updated and effective surgical treatment

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RESUMEN El tumor estromal gastrointestinal representa el 3% de las neoplasias gastrointestinales; es el tumor mesenquimático más frecuente. Afecta a hombres mayores de 50 años. El 80% son benignos, la mayoría afectan el estómago e intestino delgado. La incidencia de localización extragastrointestinal es desconocida. Paciente masculino de 56 años, tabaquista, obeso, con hipertensión arterial (HTA) y diabético (DBT), anticoagulado, consulta por dolor en fosa ilíaca derecha, posterior a esfuerzo físico. Se realiza tomografía computarizada (TC) donde se visualiza lesión de aspecto expansivo intraperitoneal que muestra realce periférico. Se decide conducta quirúrgica. Se halla un tumor mesentérico. En su presentación, estos tumores hasta en un 60% suelen ser asintomáticos por lo que resultan solo un hallazgo imagenológico; es indispensable, pues, su sospecha clínica y fundamentalmente el aporte de la inmunohistoquímica para la definición de la patología. El CD 117 es el principal marcador. Su tratamiento de preferencia es siempre quirúrgico, acompañado de tratamientos quimioterápicos.

ABSTRACT Gastrointestinal stromal tumors (GISTs) account for < 3% of gastrointestinal neoplasms and are the most common mesenchymal tumors. They are more common in men > 50 years. They are benign in 80% of the cases and usually occur in the stomach and small intestine. The incidence of extragastrointestinal GISTs is unknown. A 56-year-old male patient sought medical care for abdominal pain in the right iliac fossa that appeared after exercising. The patient was a current smoker, obese, had a history of hypertension (HTN) and diabetes (DBT) and was receiving anticoagulants. A computed tomography (CT) scan showed an expansive mass within the peritoneum with peripheral enhancement. Surgical management was decided. During the procedure, a tumor was found in the mesentery. Up to 60% of these tumors are usually asymptomatic and are incidentally found in imaging tests; therefore, clinical suspicion and, most importantly immunohistochemistry, are essential for the diagnosis. CD117 is the main marker. Surgery is the treatment of choice for GISTs and chemotherapy is also indicated.

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AIM: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the digestive tract and has a wide variation in biological behavior; surgical resection remains the main form of treatment. This study aimed to analyze clinicopathological characteristics and survival of patients with GIST in a reference institution for oncological diseases. METHODS: An observational, longitudinal, and retrospective study of patients diagnosed with GIST from January 2011 to January 2020 was carried out by analyzing epidemiological and clinical variables, staging, surgical resection, recurrence, use of imatinib, and curves of overall survival (OS) and disease-free survival (DFS). RESULTS: A total of 38 patients were included. The majority (58%) of patients were males and the median age was 62 years. The primary organs that were affected by this tumor were stomach (63%) and small intestine (17%). Notably, 24% of patients had metastatic disease at diagnosis; 76% of patients received surgical treatment and 13% received neoadjuvant treatment; and 47% of patients received imatinib as adjuvant or palliative therapy. Tumor recurrence was 13%, being more common in the liver. The 5-year OS was 72.5% and DFS was 47.1%. The operated ones had better OS (87.1% vs. 18.5%) and DFS (57.1% vs. 14.3%) in 5 years. Tumor size ≥5 cm had no difference in OS at 5 years, but DFS was 24.6%, when compared with 92.3% of smaller tumors. Patients who were undergoing neoadjuvant therapy and/or using imatinib did not show any significant differences. CONCLUSIONS: Surgical treatment with adequate margins allows the best gain in survival, and the use of imatinib in more advanced cases has prognostic equity with less advanced-stage tumors. Treatment of metastatic tumors seems promising, requiring further studies.

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RESUMEN Los tumores GIST son un motivo de consulta cada vez más frecuente en las entrevistas de cirugía gastroenterológica. Suelen ser derivados como hallazgos incidentales o por presentar síntomas derivados de su crecimiento. Se presenta el caso clínico de una paciente que requirió internación de urgencia por síndrome anémico agudo. Se comenta su algoritmo diagnóstico y su resolución quirúrgica. Asimismo se comentan los estándares de diagnóstico y tratamiento actuales con especial foco en la estrategia quirúrgica, la cual debe ser individualizada según cada caso.

ABSTRACT Gastrointestinal stromal tumors (GISTs) are becoming an increasingly common reason for consultation in gastroenterology surgery interviews. Patients are usually referred for surgery due to an incidental finding or symptoms associated with tumor growth. We report the case of a female patient who required urgent hospitalization due to acute anemic syndrome. The diagnostic algorithm and surgical approach are described. The current standards of diagnosis and treatment are also discussed, with special focus on the surgical strategy, which must be tailored to each case.

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Resumen Objetivos: Presentar caso clínico y revisión de la literatura sobre asociación de tumores poco frecuentes compatibles con diagnóstico de tríada de Carney. Paciente y Métodos: Revisión de ficha clínica de paciente de sexo femenino de 39 años de edad con antecedentes de asma, quien acude a servicio de urgencias por síntomas respiratorios. En estudio con imágenes se evidencia masa pulmonar en lóbulo superior derecho probablemente hamartoma y masa en la bifurcación carotídea izquierda compatible con posible paraganglioma. Se completó el estudio con endoscopia digestiva alta sin evidencia de tumor gástrico y PET-CT (tomografía de emisión de positrones-tomografía computarizada) que descartó otras lesiones. Resultados: La paciente fue sometida a resección quirúrgica de ambos tumores (pulmonar y carotídeo). En estudio histopatológico diferido, se plantean los diagnósticos de paraganglioma carotideo y hamartoma pulmonar, el cual, luego de una segunda revisión histopatológica, es corregido a condroma pulmonar. Discusión: La tríada de Carney se compone por la asociación de al menos 2 de 3 tumores: tumor estromal gastrointestinal (GIST), paraganglioma extra-adrenal y condroma pulmonar. Su expresión es variable, coexistiendo en forma completa en solo el 22% de los casos. Conclusión: Los pacientes con sospecha de tríada de Carney deben recibir evaluación multidisciplinaria, estudio completo en búsqueda de tumores asociados y seguimiento a largo plazo por posibles recurrencias o metástasis.

Objective: To present a clinical case and review of the literature on the infrequent association of pulmonary and extra thoracic tumors compatible with Carney's triad. Patient and Methods: Review of clinical records of a 39 years-old female patient with history of asthma who presented in the emergency department with respiratory symptoms. An imaging study showed a pulmonary mass in the right upper lobe with the aspect of hamartoma and a mass in the left carotid artery bifurcation compatible with a possible paraganglioma. Upper gastrointestinal endoscopy showed no evidence of gastric tumor and a PET-CT (Positron Emission Tomography - Computed Tomography) excluded other lesions. Results: Patient underwent surgical resection of both tumors (pulmonary and carotid). Diagnosis of carotid paraganglioma and pulmonary hamartoma were stated by histopathology. However, lung tumor after a second pathological analysis was confirmed to be a pulmonary chondroma. Discussion: Carney's triad is defined by the association of at least 2 of 3 tumors: Gastrointestinal Stromal Tumor (GIST), extraadrenal paraganglioma and pulmonary chondroma. Its expression is variable, coexisting completely in only 22% of cases. Conclusion: Patients with suspected Carney's triad should receive a multidisciplinary assessment, a complete study searching associated tumors and long-term follow-up for recurrences or metastases.

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Gastrointestinal Stromal Sarcomas (GIST) are mesenchymal neoplasms whose incidence accounts for 1-2% of digestive tumors, being located in the stomach (55-60%) and small intestine (30%). The advances in its knowledge and management succeeded in the last years have being spectacular. This review aims to summarize the most important of them for surgeons. We identified four areas of interest: molecular oncology, laparoscopic approach, management of GIST located at unusual locations, and management of advanced GIST. Advances in the field of molecular oncology lead to the discovery of new oncogenic mutations making the term Wil Type GIST obsolete. Moreover, these advances allow for the development of 2 new drugs: Avapritinib and Ripretinib, that added to the previous 3 commercially available drugs (imatinib, sunitinib and regorafenib) make possible the management of GIST with resistant mutations. The principles of the surgical management of primary GIST are well stablished which laparoscopic approach must accomplish. This approach is limited by 2 main factors: location and size. The diagnosis of GIST in unusual locations as esophagus, duodenum, rectum of out of the gastrointestinal tract (EGIST), implies an extraordinary therapeutic challenge, being imperative to manage them by surgeons and oncologist among others in the setting of a multidisciplinary team. The management of advanced/metastatic GIST has changed in a revolutionary fashion because surgery is now part of its treatment as adjuvant to tyrosine kinase inhibitors.

Los tumores del estroma gastrointestinal (GIST) suponen el 1-2% de los tumores digestivos, siendo su localización más frecuente el estómago (55-60%) y el intestino delgado (30%). Los avances más importantes sucedidos en los últimos años se centran en cuatro áreas: biología molecular, abordaje quirúrgico laparoscópico, manejo técnico del GIST en localizaciones inusuales y tratamiento e integración de la cirugía en el manejo del GIST avanzado. Los avances en el conocimiento de la biología molecular del GIST han dado lugar a la progresiva identificación de nueva mutaciones oncogénicas que hacen del concepto wild type obsoleto. Estos avances han permitido el desarrollo de dos nuevos fármacos, avapritinib y ripretinib, lo que permite el tratamiento de pacientes con mutaciones resistentes a las tres líneas terapéuticas clásicas. El tratamiento quirúrgico del GIST se rige por unos principios técnicos bien establecidos que el abordaje laparoscópico debe cumplir, abordaje que queda limitado por dos factores clave: localización y tamaño. El GIST de localización infrecuente (esófago, duodeno o recto, o extradigestivo) supone un reto terapéutico. Estos pacientes deben ser manejados en un contexto multidisciplinario. La cirugía queda integrada en el manejo del GIST avanzado, considerándose como adyuvante a los inhibidores de la tirosina cinasa.

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INTRODUCTION AND AIMS: Surgical resection of gastrointestinal (GI) cancer is the cornerstone of curative treatment but entails considerable morbidity. The surgical Apgar score (SAS) is a practical and objective instrument that provides immediate feedback. The aim of the present study was to evaluate the performance of the SAS for predicting complications at 30 days in patients with primary GI cancer that underwent curative surgery. MATERIALS AND METHODS: A prospective observational study was conducted that included 50 patients classified into a low SAS (≤ 4) group or a high SAS (≥ 5) group. Complications were defined as any event classified as a Clavien-Dindo grade II to V event. Bivariate and multivariate analyses were performed through the Cox regression and a p<0.05 was considered significant. RESULTS: Overall postoperative morbidity was 50.0%, with no mortality. Eighty-six percent of cases were catalogued as having an ASA≥3. Eighty-eight percent had a high SAS, of whom 45.5% presented with a complication, whereas 12.0% had a low SAS and a complication rate of 83.3%. In the multivariate analysis, the BMI (OR: 3.351, 95% CI: 1.218-9.217, P=.019), SAS (OR: 0.266, 95% CI: 0.077-0.922, P=.037), surgery duration (OR: 3.170, 95% CI: 1.092-9.198, P=.034), and ephedrine use (OR: 0.356, 95% CI: 0.144-0.880, P=.025) were significantly associated with the development of adverse outcomes. CONCLUSIONS: SAS was shown to be an independent predictive factor of postoperative morbidity at 30 days in the surgical management of GI cancer and appears to offer a reliable sub-stratification in a high-risk population with an ASA≥3.

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