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Introdução: Tumor estromal gastrointestinal (GIST) é uma doença relativamente rara e a cirurgia se constitui no tratamento principal deste tumor. Existem apenas poucos centros com experiência no tratamento desta neoplasia. O objetivo do trabalho é analisar os resultados do tratamento cirúrgico dos pacientes portadores de GIST gástrico operados em uma mesma instituição. Métodos: Estudo retrospectivo baseado na análise de 20 pacientes com diagnóstico confirmado de GIST gástrico operados no Instituto Nacional do Câncer (INCA- Rio de Janeiro) entre 1986 e 2000. Todos os dados foram revisados, enfocando-se as características histopatológicas (localização, número de mitoses e tamanho do tumor), as características dos pacientes (idade, sexo e apresentação clínica) e os resultados cirúrgicos (tipos de cirurgia, morbidade, mortalidade e sobrevida de acordo com a classificação proposta por Shiu). Resultados: Onze pacientes eram do sexo masculino e 9 do sexo feminino. A mediana de idade foi de 57 anos. O tamanho médio do tumor foi de 14,7 cm. Setenta por cento dos pacientes tinham tumor de alto grau e 65% dos tumores se localizavam na porção proximal do estômago. Gastrectomia subtotal foi realizada em 50% dos casos, gastrectomia total em 35% e gastrectomia atípica em 10%. Ressecção gástrica isolada foi realizada em seis casos (grupo 1), ressecção de dois órgãos foi feita em cinco casos (grupo 2) e ressecção de mais de dois órgãos em oito casos (grupo 3). A morbidade operatória global foi de 35%. O grupo 1 não teve morbidade, o grupo 2 teve morbidade de 20% e o grupo 3, de 70%. A mortalidade operatória foi de 10% (2 pacientes). Dez por cento dos pacientes foram classificados como estádio 0, 25% como estádio I e 65%, estádio II de acordo com a classificação de Shiu. A sobrevida em 5 anos para o estádio 0 foi de 100% enquanto que o estádio II apresentou sobrevida nula em 5 anos. Conclusão: A alta morbi-mortalidade desta casuística é devido à presença de doença avançada ao diagnóstico, havendo necessidade de ressecções multiorgânicas a fim de se obter cirurgia R0. A cirurgia é o tratamento principal, porém isolada é insuficiente para se atingir sobrevida longa em doença avançada.

OBJETIVO: Avaliar os resultados do tratamento de GIST no INCA. MÉTODOS: Análise retrospectiva de todos os casos de GIST tratados no INCA no período de 1997 a 2009. RESULTADOS: Analisamos 146 pacientes, com média de idade de 44,5 anos e predomínio do sexo feminino. O principal sintoma foi dor abdominal. Tivemos ocorrência de segundo primário em 22% dos casos e na imuno-histoquímica, 92% foram positivos para CD117. A localização mais frequente foi estômago e predominou o grupo de alto risco. A cirurgia foi R0 (extenso) em 70% e os principais sítios de metástases foram fígado e peritônio. A sobrevida global foi, respectivamente, em dois e cinco anos de 86% e 59%. Houve significante diferença entre a sobrevida global (p=0,29) do grupo de alto risco versus os demais. CONCLUSÃO: Os nossos pacientes apresentam-se principalmente sob forma de doença de alto risco com repercussão óbvia na sobrevida. O uso de Imatinib melhorou a sobrevida dos pacientes com doença metastática e recidivada. Devemos estudar seu uso no cenário de adjuvância e neoadjuvancia visando melhorar os índices do grupo de alto risco. A criação de centros referenciais é uma necessidade para o estudo de doenças pouco frequentes. OBJECTIVE: To evaluate the treatment of GIST in INCA. METHODS: We conducted a retrospective analysis of all cases of GIST treated at INCA in the period from 1997 to 2009. RESULTS: We analyzed 146 patients with a mean age of 44.5 years and female predominance. The main symptom was abdominal pain. We observed the occurrence of a second primary tumor in 22% of cases and 92% of the immunohistochemistry exams were positive for CD117. The most frequent location was in the stomach and the high-risk group was predominant. Surgery was considered R0 (extensive) in 70% of the cases and the main sites of metastases were liver and peritoneum. Overall survival in two and five years was, respectively, 86% and 59%. There was a significant difference between overall survival (p = 0.29) of the high-risk group versus the other. CONCLUSION: Our patients presented mainly in the form of high-risk disease, with obvious impact on survival. The use of imatinib improved survival of patients with recurrent and metastatic disease. We should study its use in the setting of adjuvant and neoadjuvant therapy to improve results of the high risk group. The creation of reference centers is a need for the study of rare diseases.

Gastrointestinal stromal tumors (EGIST) are mesenchymal tumors which may originate in sites outside the gastrointestinal tract with clinico-pathological and molecular profiles similar to GISTs. Here is a series of case reports from The National Cancer Institute (INCA) about this rare type of tumor.We retrospectively analyzed 142 patients with a diagnosis of GIST who were treated at INCA between 1997 and 2009, among which 9 were confirmed as having EGIST.We identified 9 patients with a diagnosis of EGIST, 7 of which (77.8%) were female patients with an average age of 56.8 (36-81) years. All patients were symptomatic, with abdominal pain present in 55% of cases. The tumors were located in the pancreas (n=2), in the retroperitoneum (n=3), and in the mesocolon, in the mesentery of the small intestine, in the spleen and in the pelvis (all n=1), respectively. The average tumor size was 18(8.5-27)cm. Immunohistochemistry revealed CD 117 positive in 100% of cases and CD34 positive in 62.5%. Five patients were treated surgically, three of which were submitted to R0 resection. Among those patients, all recurred after 31, 23 and 17 months respectively, and the most frequent sites were the liver and the peritoneum. Out of these three recurrences, two were "rescued" surgically (66%). Five patients were given imatinib (three patients considered unresectable, one submitted to R2 resection and another patient with recurrent disease not amenable to surgery) two patients did not receive any treatment due to compromised clinical conditions. The average overall survival was 26.4 (04-114) months.The occurrence of EGIST is extremely rare and little is known about their actual origin. They manifest through bulky masses, which compromise the prognosis. Aggressive surgical intervention is the most effective treatment associated with the use of imatinib, and a strict followup is necessary due to high recurrence rates.

To evaluate the treatment of GIST in INCA.We conducted a retrospective analysis of all cases of GIST treated at INCA in the period from 1997 to 2009.We analyzed 146 patients with a mean age of 44.5 years and female predominance. The main symptom was abdominal pain. We observed the occurrence of a second primary tumor in 22% of cases and 92% of the immunohistochemistry exams were positive for CD117. The most frequent location was in the stomach and the high-risk group was predominant. Surgery was considered R0 (extensive) in 70% of the cases and the main sites of metastases were liver and peritoneum. Overall survival in two and five years was, respectively, 86% and 59%. There was a significant difference between overall survival (p = 0.29) of the high-risk group versus the other.Our patients presented mainly in the form of high-risk disease, with obvious impact on survival. The use of imatinib improved survival of patients with recurrent and metastatic disease. We should study its use in the setting of adjuvant and neoadjuvant therapy to improve results of the high risk group. The creation of reference centers is a need for the study of rare diseases.

This papers is a retrospective analysis of all cases of colorectal GIST treated at our institution. Considering the increased interest in this pathology, in 2008, we recently established a unit supported by Novartis in our department to research and treat GIST. Since then, there was a dramatic increase in the referral of this type of tumor to our center. Before initiating our research group, we had the permission of the ethics committee and the medical directors to study and publish the analysis of our cases. Now, we have a cohort of 98 patients treated during the last 10 years and from that, we selected all cases of Colorectal GIST's. They numbered 13 cases. We performed an analysis of these cases from the presentation to the results of treatment.

Prediction of biological behavior is crucial for selection of new therapeutic modalities in GIST. Here, we aimed to assess whether KIT and PDGFRA mutations have survival impact in gastrointestinal stromal tumors (GIST).Fifty-five Brazilian patients with completely resected GIST were examined for KIT and PDGFRA mutations. The 5-year disease-free survival (DFS) was analyzed.KIT and PDGFRA mutations were identified in 74.5% and 7.3% of patients, respectively. The 5-year DFS rate for all patients was 52.8%. The 5-year DFS rate was lower in patients with tumors having in-frame deletions or concomitant in-frame deletions and insertions affecting codons 557-558 than in patients with tumors having other exon 11 KIT mutations (p=0.023). Conversely, when the patients with concomitant deletion-insertion mutations affecting codons 557-558 were excluded from the analysis, deletions involving codons 557-558 had no influence on 5-year DFS rates.Our findings indicate that a specific KIT mutation may be associated with unfavorable behavior in GIST. This finding may have implications on selecting patients for adjuvant therapy.

O objetivo do presente trabalho é demonstrar o panorama atual do câncer colorretal em um hospital geral no estado do Rio de Janeiro, enfocando aspectos relacionados à apresentação clínica e ao diagnóstico tardio. Trata-se de um estudo retrospectivo que incluiu os pacientes em acompanhamento no ambulatório de seguimento de câncer colorretal da II Clínica Cirúrgica do Hospital Federal de Bonsucesso nos últimos 5 anos (2004-2009). Os sintomas mais comuns foram dor abdominal (60,1%), obstrução intestinal (41,1%), emagrecimento (36,7%), sangramento (33.5%), anemia (14,5%), perfuração (6,3%) e fístula (1,2%). Em relação aos pacientes com tumores de cólon direito, os sintomas mais prevalentes foram emagrecimento (54,5%), dor (45,4%), obstrução (45,4%) e anemia (27,2%). Nos pacientes com tumores de cólon esquerdo e sigmóide foram dor (60,5%), obstrução (42,9%), emagrecimento (38,5%), sangramento (32,4%) e anemia (16,6%). Enquanto nos pacientes com tumores de reto foram sangramento (70%), obstrução (60%), dor (60%), emagrecimento (20%) e perfuração (10%). No momento da cirurgia, 53 pacientes apresentavam metástases à distância (33,5%), sendo o fígado o órgão mais acometido, em 36 pacientes (67,9%), seguido pelo peritônio com 11 casos (20,7%) e pelos anexos (ovários) com 4 casos (7,5%). Oitenta e oito pacientes (55,6%) apresentavam metástase linfonodal.Quanto ao estadiamento, observamos que os pacientes operados na emergência apresentavam a seguinte distribuição: 0% Estádio I, 28,2% Estádio II, 30,4% Estádio III e 41,3 % Estádio IV. Os pacientes operados eletivamente foram estratificados como Estádio I 2,7%, Estádio II 27,7 %, Estádio III 25% e Estádio IV 44%. Conclusão: O perfil dos pacientes operados na nossa instituição (e que reflete a realidade nacional) é de doença avançada. Com base nos dados apresentados, fica clara a necessidade de implementação de um programa de rastreamento para câncer colorretal. The aim of this study is to demonstrate the current panorama of colorectal cancer in a general hospital in Rio de Janeiro, focusing on aspects related to the clinical presentation and delayed diagnosis. This is a retrospective study that included patients followed at the outpatient section of colorectal cancer follow-up of the Segunda Clínica Cirúrgica do Hospital Federal Bonsucesso in the past five years (2004-2009). The most common symptoms were abdominal pain (60.1%), intestinal obstruction (41.1%), weight loss (36.7%), bleeding (33.5%), anemia (14.5%), perforation (6.3 %) and fistula (1.2%). Patients with right colon tumors presented with weight loss (54.5%), pain (45.4%), obstruction (45.4%) and anemia (27.2%). In patients with tumors of the left colon and sigmoid, the most common symptoms were pain (60.5%), obstruction (42.9%), weight loss (38.5%), bleeding (32.4%) and anemia (16.6%). Patients with rectal tumors presented with bleeding (70%), obstruction (60%), pain (60%), weight loss (20%) and perforation (10%). At surgery, 53 patients had distant metastases (33.5%), the liver being the organ most affected in 36 patients (67.9%), followed by the peritoneum in 11 cases (20.7%) and ovaries with 4 cases (7.5%). Eighty-eight patients (55.6%) had nodal metastasis. Regarding TNM staging, we found that the emergency group distribution was as follows: Stage I 0%, 28.2% Stage II, Stage III 30.4% and 41 3% Stage IV, whereas the elective group staging distribution was: 2.7% Stage I, Stage II 27.7%, 25% Stage III and Stage IV 44%. Conclusion: The profile of patients operated in our institution (which reflects the national reality) is composed mainly with patients with advanced cancer stages. Based on the data presented, it is necessary to implement a national screening program for colorectal cancer.

Objective Evaluate the presence of other tumors in cohort of patients with GIST treated at a cancer treatment referral center – INCA. Methods We reviewed the medical records of patients diagnosed with GIST who were treated at INCA between 1998 and 2008. Immunohistological diagnosis was confirmed by a pathologist specialized in sarcomas. Patients presenting second non-GIST tumors were identified. Age, sex, tumor location, risk groups (according to the National Institutes of Health criteria), characteristics of non-GIST tumors and treatment results were analyzed. Results Among the 101 patients diagnosed with GIST who were evaluated during the study period, 14 (13.8%) had other non-GIST tumors, 9 females (64.3%), with a median age of 68 years (10–79 years). The stomach was the location of GISTs in 8 cases (57.1%), followed by the small bowel in 4 cases (28.5%), colon and mesentery with 1 case (7.1%) each. The mean size of lesions was 4.79 cm (0.3–15 cm), with malignant potential low/very low in 7 cases (50%), intermediate in 5 cases (35.7%) and high in 2 cases (14.3%). The diagnosis of GIST was incidental in 6 cases and in one case the non-GIST tumor was incidental. The non-GIST tumors were most frequent in the stomach (adenocarcinoma), in 4 cases (28.5%) and colon/rectum (adenocarcinoma) in 4 other cases. The other sites involved were breast (ductal carcinoma), kidney (clear cell carcinoma), prostate (adenocarcinoma), endometrium (adenocarcinoma), ovary (adenocarcinoma) and adrenal (neuroblastoma), with one case each. The tumors were synchronous in 7 cases (50%). With a median follow-up after GIST resection of 41 months (2–87 months), 9 patients were alive without evidence of disease, 2 died due to GIST, 2 died due to non-GIST tumors and the remaining patient died due to postoperative complications. Conclusions We discovered a 13.8% incidence of non-GIST tumors in a series of 101 GIST cases under our care. This association should always be considered in the management of patients with GIST.

Recent progress in gastrointestinal stromal tumor's (GIST) treatment were responsible for changing GIST's natural history. Knowledge acquirement of molecular mechanism-based systemic therapy gave rise to the development of targeted antineoplastic drugs capable of reaching outcomes that had never been reached before. The introduction of imatinib in the clinical practice not only changed GIST's patients survival but also shifted paradigms. However, besides all these new advances and the improved results with imatinib, the surgeon still plays a pivotal role in the management of the primary GIST tumor and even in the metastatic setting.

OBJETIVO: Descrever os achados tomográficos do tumor estromal gastrintestinal de origem gástrica. MATERIAIS E MÉTODOS: No período de janeiro de 1999 a dezembro de 2006, foram selecionados 14 pacientes com diagnóstico histopatológico e imuno-histoquímico de tumor estromal gastrintestinal gástrico que apresentavam tomografia computadorizada realizada anteriormente ao tratamento. As variáveis tomográficas analisadas foram: topografia da lesão, dimensões, homogeneidade, contornos, limites, morfologia, padrão e intensidade do realce pelo meio de contraste venoso, padrão de crescimento, invasão de órgãos adjacentes, presença de ulceração, fístula, calcificações, infiltração da gordura mesentérica, linfonodomegalias e metástases a distância. RESULTADOS: Os tumores foram localizados no corpo (57,1%) ou fundo gástrico (42,9%), com dimensões variando entre 6,0 e 23,0 cm (média de 11,5 cm). O crescimento foi predominantemente extraluminal (57,1%) ou intra/extraluminal (35,7%). O realce pelo contraste venoso foi discreto em 50% dos casos, moderado em 50% e heterogêneo em 64,3%. Foram ainda observadas hipodensidade central em 64,3% dos casos, invasão de órgãos adjacentes em 42,9% e metástases hepáticas em 7,2%. CONCLUSÃO: No presente estudo, a maioria dos tumores localizava-se no corpo gástrico, com tamanho médio de 11,5 cm, apresentando área hipodensa central, realce heterogêneo pelo meio de contraste e crescimento predominantemente extraluminal. OBJECTIVE: The purpose of this study was to describe the tomographic findings of gastric gastrointestinal stromal tumor. MATERIALS AND METHODS: Fourteen patients with histopathologically and immunohistochemically confirmed gastric gastrointestinal stromal tumors, who had already been submitted to computed tomography scans before the treatment, were evaluated in the period between January 1999 and December 2006. The following tomographic variables were analyzed: lesion topography, size/dimensions, homogeneity, contour, margins, morphology, pattern and intravenous contrast-enhancement intensity, growth pattern, invasion of adjacent organs, presence of ulceration, fistula, calcifications, mesenteric fat infiltration, lymphadenomegaly and presence of distant metastasis. RESULTS: Tumors were found in the body (57.1%) or in the gastric fundus (42.9%), with sizes ranging between 6.0 cm and 23.0 cm (mean, 11.5 cm). Predominantly extraluminal growth was observed in 57.1% of cases and intra/extraluminal in 35.7%. Subtle contrast-enhancement was observed in 50%, moderate in 50%, and heterogeneous in 64.3% of cases. Additionally, central hypodensity was observed in 64.3%, invasion of adjacent organs in 42.9%, and hepatic metastasis in 7.2% of cases. CONCLUSION: In the present study, the majority of tumors were found in the gastric body, with an average size of 11.5 cm, presenting with central hypodensity, heterogeneous contrast-enhancement and predominantly extraluminal growth.

So many variables have been identified as prognostic factors influencing survival after curative resection in gastrointestinal stromal tumors (GIST), but the role of lymph node metastasis remains uncertain.Twenty-nine patients with c-Kit positive gastric GIST who underwent surgical resection at the Brazilian National Cancer Institute between 1983 and 2004 were reviewed retrospectively. The prognostic significance of lymph node metastasis was investigated. The endpoints were overall survival and disease free survival.The median follow-up was 35 months. The 5-years estimate survival rate was 53%. Three patients presented lymph node metastasis and developed recurrence disease. Univariate analysis for overall survival identified the size13.5cm (p = 0.01) and recurrence (p = 0.03) as prognostic factors. Size13.5cm and recurrence were independent factors (p = 0.01 and p = 0.03, respectively) in the multivariate analysis. Univariate analysis for disease free survival identified the size13.5cm (p = 0.04) and the grade (p = 0.04) as prognostic factors but, only the size13.5cm was an independent factor in the multivariate analysis. Lymph node metastasis had no prognostic significance for overall and disease free survival (p = 0.65 and p = 0.57, respectively).GIST lymph node metastasis was not related to poor survival in this study, but more studies are needed to identify the real incidence and the proper role of the GIST metastatic nodal disease.

Recent progress in gastrointestinal stromal tumor's (GIST) treatment were responsible for changing GIST's natural history. Knowledge acquirement of molecular mechanism-based systemic therapy gave rise to the development of targeted antineoplastic drugs capable of reaching outcomes that had never been reached before. The introduction of imatinib in the clinical practice not only changed GIST's patients survival but also shifted paradigms. However, besides all these new advances and the improved results with imatinib, the surgeon still plays a pivotal role in the management of the primary GIST tumor and even in the metastatic setting.

The colorectal cancer has become a world public health problem as a consequence of the great number of new cases which have been diagnosed each year and the existence of some conditions related to the disease's natural history that can be identified and the cancer prevented. Knowing the fact that 20% out of all colorectal cancers develops as part of a hereditary cancer syndrome, it is crucial that the physician (not only the surgeon) be updated with this entity, being able to recognize, and mainly, implement screening programs to identify family members at risk of developing cancer and to allow the intervention to prevent the occurrence of the adenoma-carcinoma sequence.

e16131 Background: Induction chemotherapy (IC) followed by chemoradiation (CRT) is an attractive approach in locally-advanced, high-risk rectal cancer. Additionally, aspirin has shown potential to lower recurrence rate in colorectal cancer, and improve outcomes alongside CRT in rectal cancer, with higher rate of tumor downstaging. Methods: Randomized, double-blind phase 2 trial to evaluate induction treatment with XELOX, followed by capecitabine-based chemoradiotherapy with aspirin or placebo in a high risk population selected by MRI. The aim of this study was to evaluate MRI response after total neoadjuvant treatment with aspirin or placebo. Results: Of the 25 pts who started treatment between January 2018 and March 2019, 4 pts did not complete (1 pt grade 5 diarrhea; 1pt treatment adherence). Median age was 55yo (32.9 - 73.6), 8 pts (32%) were women, and 80% had 3 or more high-risk criteria. 20 pts (80%) showed symptom improvement in the first cycle of IC. Of the 21 patients who finished treatment until interim analysis, 11 pts received aspirin (2 pts had MRI complete response (CR), 3 pts had minor/no response, and 3 pts had progression disease (PD)). 10 pts received placebo (4 MRI CR, 3 pts had minor/no response, and no PD). Conclusions: Aspirin added to chemoradiotherapy was safe but did not improve response to total neoadjuvant treatment. The study was closed due absence of benefit. Clinical trial information: NCT03170115 .