Clinical features and prognosis of malignant small bowel tumors: Experience from a university hospital in Chile.

BACKGROUND: Small bowel tumors (SBT) are infrequent and represent a small proportion of digestive neoplasms. There is scarce information about SBT in Latin America. AIM: To describe the epidemiology, clinical characteristics, diagnostic methods, and survival of malignant SBTs. METHODS: Retrospective observational study of adult patients with histopathological diagnosis of SBT between 2007 and 2021 in a university hospital in Chile. RESULTS: A total of 104 patients [51.9% men; mean age 57 years] with SBT. Histological type: neuroendocrine tumor (NET) (43.7%, n=38), gastrointestinal stromal tumors (GIST) (21.8%, n=19), lymphoma (17.2%, n=15) and adenocarcinoma (AC) (11.5%, n=10). GIST was more frequent in duodenum (50%; n=12) and NET in the ileum (65.8%; n=25). Metastasis was observed in 17 cases, most commonly from colon and melanoma. Nausea and vomiting were significantly more often observed in AC (p=0.035), as well as gastrointestinal bleeding in GIST (p=0.007). The most common diagnostic tools were CT and CT enteroclysis with an elevated diagnostic yield (86% and 94% respectively). The 5-year survival of GIST, NET, lymphoma and AC were 94.7% (95%CI: 68.1-99.2), 82.2% (95%CI: 57.6-93.3), 40.0% (95%CI: 16.5-82.8) and 25.9% (95%CI: 4.5-55.7%), respectively. NET (HR 6.1; 95%CI: 2.1-17.2) and GIST (HR 24.4; 95%CI: 3.0-19.8) were independently associated with higher survival compared to AC, adjusted for age and sex. CONCLUSIONS: Malignant SBT are rare conditions and NETs are the most common histological subtype. Clinical presentation at diagnosis, location or complications may suggest a more probable diagnosis. GIST and NET are associated with better survival compared to other malignant subtypes.

Tumor de estroma gastrointestinal: a propósito de un caso y revisión de la literatura / Gastrointestinal stromal tumor: report of a case and review of the literature

Introducción: los tumores del estroma gastrointestinal (GIST), son las neoplasias de origen mesenquimático más frecuentes del tracto digestivo, sin embargo, representan menos del 1 por ciento de todos estos tumores. Actualmente se denominan GIST a los tumores mesenquimales CD117 positivos, fusiformes o epitelioides, primarios del tracto gastrointestinal, epiplón, mesenterio y retroperitoneo. Presentación de caso: presentamos un paciente de 60 años de edad que fue ingresado en el servicio de Medicina Interna por anemia crónica y dispepsia. Al examen físico se constata en la palpación de abdomen un tumor en hipocondrio derecho y epigastrio, no mesurable, fijo, doloroso. Se realizan complementarios como ecografía abdominal, esófago gastroduodenoscopia, TAC abdominal y laparoscopia demostrándose la presencia de un tumor de la curvatura mayor gástrica de aproximadamente 20cm con crecimiento exofitico. Se interviene quirúrgicamente, realizándose gastrectomía total, esplenectomia y esofagoyeyunostomia. Los resultados definitivos de anatomía patológica informan: tumor de pared gástrica, fusocelular del estroma gastrointestinal, de bajo potencial. Tamaño del tumor 25cm. Índice Mitótico de 1 mitosis por 50 HPF con Inmunohistoquímica positiva para CD117, CD34, cumplió tratamiento quimioterapia y presenta una evolución favorable. Discusión: los GIST son tumores cuya presencia de síntomas dependerá del tamaño y localización del tumor y puede variar desde formas asintomáticas a casos que se presenten como una emergencia quirúrgica por perforación gástrica o sangramiento digestivo. Conclusiones: el paciente fue intervenido quirúrgicamente encontrándose un tumor fusocelular del estroma gastrointestinal (GIST) de pared gástrica, de bajo potencial de malignidad, comportamiento agresivo(AU)

Introduction: the Gastrointestinal Stroma Tumors (GIST), the neoplasia from which those stem are those of highest mesenquimatic frequency of the digestive tract, however, it represents less than 1 percent of all these tumors. At present, mesenquimales name the tumors GIST CD117 positive, fusiform or epithelioid, primary of the tract gastrointestinal, epiplón, mesentery and retro-peritoneum. Case presentation: we introduced a 60-year-old patient that went through admittance in the Internal Medicine service due to chronic anemia and dyspepsia. To the physical examination through palpation of the abdomen, a tumor in straight hypochondrium and epigastrium is verified, notmeasurable, it is fixed, painful. Complementary tests are also performed, like abdominal echography, esophagus gastroduedenoscopy abdominal CAT and laparoscopy, showing the presence of a tumor of the bigger gastric curvature of approximately 20cm with exofitic growth. Surgery is performed, coming true total gastrectomy, splenectomy and esofagoyeyunostomy, which have proven to be definite of morbid anatomy, as the inform discloses: Tumor of gastricwall, gastrointestinal fusocellular stroma, of potential bass. Size of the tumor 25cm. Meiotic index of 1 mitosis for 50 HPF with positive Inmunohistochemical for CD117, CD34, treatment fulfilled chemotherapy and it presents a favorable evolution. Discussion: the GIST symptoms are depended of the tumor size and localization. They had some types of clinical presentation such as gastric perforation or haemorrage. Conclusion: in the OR we found a gastrointestinal fusocelullar tumor, at the gastric wall. With very aggressive behavior(EU)

Retrospective Analysis of Chilean and Mexican GI Stromal Tumor Registries: A Tale of Two Latin American Realities.

PURPOSE: Like other malignancies, GI stromal tumors (GIST) are highly heterogeneous. This not only applies to histologic features and malignant potential, but also to geographic incidence rates. Several studies have reported GIST incidence and prevalence in Europe and North America. In contrast, GIST incidence rates in South America are largely unknown, and only a few studies have reported GIST prevalence in Latin America. PATIENTS AND METHODS: Our study was part of a collaborative effort between Chile and Mexico, called Salud con Datos. We sought to determine GIST prevalence and patients' clinical characteristics, including survival rates, through retrospective analysis. RESULTS: Overall, 624 patients were included in our study. Our results found significant differences between Mexican and Chilean registries, such as stage at diagnosis, primary tumor location, CD117-positive immunohistochemistry status, mitotic index, and tumor size. Overall survival (OS) times for Chilean and Mexican patients with GIST were 134 and 156 months, respectively. No statistically significant differences in OS were detected by sex, age, stage at diagnosis, or recurrence status in both cohorts. As expected, patients categorized as being at high risk of recurrence displayed a trend toward poorer progression-free survival in both registries. CONCLUSION: To the best of our knowledge, this is the largest report from Latin America assessing the prevalence, clinical characteristics, postsurgery risk of recurrence, and outcomes of patients with GIST. Our data confirm surgery as the standard treatment of localized disease and confirm a poorer prognosis in patients with regional or distant disease. Finally, observed differences between registries could be a result of registration bias.

Tumor de estroma gastrointestinal gigante de estómago / A Gigantic Gastrointestinal Stroma Tumor of the Stomach

RESUMEN Los tumores del estroma gastrointestinal son los tumores mesenquimales más frecuentes en el tracto gastrointestinal, entre el 20 y el 45 por ciento son malignos. Se originan de las células de Cajal o de sus precursores. La clínica más frecuente es la presencia de un tumor abdominal, la hemorragia digestiva por ulceración de la mucosa y el dolor o sensación de plenitud abdominal. Estos tumores pueden tener un largo período de crecimiento silente hasta que por su tamaño se manifiestan clínicamente, suelen tener un crecimiento expansivo desplazando las estructuras vecinas sin invadirlas. Se presenta a un paciente con un tumor del estroma gastrointestinal de estómago con proporciones inusualmente grandes, que fuera intervenido por nuestro grupo con una evolución satisfactoria(AU)

ABSTRACT Gastrointestinal stromal tumors are the most frequent mesenchymal tumors of the gastrointestinal tract, and are malignant in 20-45 percent. They originate from Cajal cells or their precursors. The most frequent clinical symptoms are the presence of an abdominal tumor, digestive hemorrhage due to mucosa ulceration, and pain or sensation of abdominal fullness. These tumors may have a long period of silent growth until, due to their size, they manifest clinically; they tend to have expansive growth, displacing neighboring structures without invading them. We present the case of a patient with a gastrointestinal stroma tumor of the stomach of unusually large proportions, who underwent surgery by our team and with a satisfactory evolution(AU)

Gastrointestinal stromal tumor: epidemiology, diagnosis, and treatment.

PURPOSE OF REVIEW: The purpose of this review is to review the past year's literature to provide comprehensive information to researchers, physicians, and the general public regarding the epidemiology, diagnosis, and treatment of gastrointestinal stromal tumors (GISTs). Common ground as well as divergent viewpoints will be highlighted and discussed. RECENT FINDINGS: The diagnosis of GISTs may involve imaging tests such as computed tomorgraphy scan and MRI, endoscopy with or without endoscopic ultrasound, and biopsy. Only biopsy, however, can yield a positive diagnosis. As most GISTs express KIT protein, immunostaining for KIT and/or molecular genetic testing for mutations in KIT can diagnose 95% of GISTs. Regorafenib, a drug that inhibits various protein genes that lead to GIST development is a relatively new treatment modality. SUMMARY: The current review should enable clinicians to best select the diagnostic and treatment approaches to GIST.

Additional malignancies in patients with gastrointestinal stromal tumors (GIST): incidence, pathology and prognosis according to a time of occurrence-based classification.

BACKGROUND: The aim of the study is to clarify if a classification based on the time of occurrence of associated malignancies in GIST patients can help in the understanding of the clinical controversies observed in these patients. METHODS: We retrospectively reviewed all the patients diagnosed with GIST tumors between January 1999 and October 2016. They were divided into GIST patients associated with other tumors (A-GIST) and those not associated (NA-GIST). A-GIST patients were also divided into four types according to the proposed classification. RESULTS: Of 104 GIST patients, 32 (30.7%) (A-GIST group) had at least one additional primary malignancy. The most frequent location of the associated malignancy was the GI tract (26%). Compared to NA-GIST, A-GIST were more often asymptomatic with a lower risk of recurrence. The main cause of death in NAGIST was GIST itself, being associated tumors the main cause of death in A-GIST group. No differences were found in DFS and OS between A-GIST and NA-GIST. CONCLUSIONS: The use of the proposed classification classifies GIST patients with associated malignancies in different subtypes that differ substantially in terms of incidence, type of neoplasms associated, cause of the association and prognosis.

Tumor del estroma gastrointestinal: presentación de caso / Gastrointestinal stromal tumor: case report

Fundamento: los tumores del estroma gastrointestinal, conocidos en inglés como gastrointestinal stromal tumors, son tumores mesenquimales que aparecen en cualquier sitio del tracto gastrointestinal. Muchos de los cuales suelen ser pediculados y de difícil diagnóstico preoperatorio. Objetivo:exponer un caso de un tumor del estroma gastrointestinal de localización poco frecuente en duodeno, más raro aún su ubicación en yeyuno. Caso clínico: paciente masculino de 77 años de edad que presenta un tumor del estroma gastrointestinal de localización en yeyuno fue atendido en el Hospital Universitario Amalia Simoni, por el servicio de urgencia en el periodo 2016-2017. Conclusiones: los tumores mesenquimales son pocos frecuentes y los del estroma gastrointestinal lo son aún más, donde la localización yeyunal los menos encontrados y en su mayoría el diagnóstico se realiza durante el acto quirúrgico(AU)

Background: the gastrointestinal stromal tumors, are mesenchymal tumors that appear in any place of gastrointestinal tract. Many of them can be pediculate and difficult pre-operatory diagnosis. Objective: to explain a case of gastrointestinal stromal tumor of difficult localization in duodenum but it is even stranger to find it in jejune. Clinical case: a 77-year-old male patient who presents a gastrointestinal stromal tumor located in jejune, he was attended in Amalia Simoni University Hospital, by the emergency services, in the period of 2016-2017. Conclusions:the mesenchymal tumors are not frequent and those of gastrointestinal stroma are even rarer but, being the localization jejunal less found and in its majority the diagnosis is made during the surgical act(AU)

Effect of imatinib on plasma glucose concentration in subjects with chronic myeloid leukemia and gastrointestinal stromal tumor.

BACKGROUND: Type 2 diabetes mellitus has become one of the most important public health concerns worldwide. Due to its high prevalence and morbidity, there is an avid necessity to find new therapies that slow the progression and promote the regression of the disease. Imatinib mesylate is a tyrosine kinase inhibitor that binds to the Abelson tyrosine kinase and related proteins. It enhances ß-cell survival in response to toxins and pro-inflammatory cytokine. The aim of this study is to evaluate the effect of imatinib on fasting plasma glucose in subjects with normal fasting glucose, subjects with impaired fasting glucose and in subjects with type 2 diabetes mellitus. METHODS: We identified 284 subjects diagnosed with chronic myeloid leukemia or gastrointestinal stromal tumors from the Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran database. 106/284 subjects were treated with imatinib. We compared the effect of imatinib on fasting plasma glucose after 1 and 6 months of treatment. We used ANOVA test of repeated samples to determine statistical significance in fasting plasma glucose before imatinib treatment and the follow-up. Statistical analysis was performed with Statistical Package for the Social Sciences v22. RESULTS: We included a total of 106 subjects: 76 with fasting plasma glucose concentrations < 100 mg/dL (normal FG), 19 subjects with fasting plasma glucose concentrations ≥100 mg/dL (impaired fasting glucose), and 11 subjects with ≥126 mg/dL (type 2 diabetes mellitus). We found a significant increase in fasting plasma glucose concentration in the normal fasting glucose group (p = 0.048), and a significant decrease in fasting plasma glucose concentration in the type 2 diabetes mellitus group (p = 0.042). In the impaired fasting glucose group, we also found a tendency towards a decrease in fasting plasma glucose (p = 0.076). We identified 11 subjects with type 2 diabetes mellitus, of whom, 7 (64%) had a reduction in their fasting plasma glucose concentrations after 6 months. A significant glycosylated hemoglobin reduction (p = 0.04) was observed. CONCLUSION: Subjects with chronic myeloid leukemia or gastrointestinal stromal tumor with type 2 diabetes mellitus had a significant reduction in fasting plasma glucose and glycosylated hemoglobin at 1 and 6 months while using imatinib.

Incidental Finding of Gastrointestinal Stromal Tumors during Laparoscopic Sleeve Gastrectomy in Obese Patients.

INTRODUCTION: The frequency of incidental pathology found during laparoscopic bariatric surgery has been estimated to be 2%. Gastrointestinal stromal tumors (GISTs) are infrequent lesions and account for less than 1% of all digestive tract tumors. The reported incidence of this type of tumors during bariatric surgery is around 0.8%. The objective of this study was to evaluate incidence, characteristics, and evolution of incidentally found GISTs in patients undergoing laparoscopic sleeve gastrectomy (LSG) for the treatment of obesity. MATERIAL AND METHODS: A retrospective analysis from a prospectively collected database was conducted. Demographic data, clinical data, laboratory tests, preoperative esophagogastroduodenoscopy (EGD), postoperative pathology report from surgical specimen with tumor markers, and patient's outcomes were evaluated. RESULTS: From June 2006 to January 2014, 915 patients underwent LSG at our institution. Five (0.5%) patients were found to have incidental GIST. There were four (80%) women; average age was 59.6 ± 6.3 years (range 46-63). None of them had symptoms that served as orientation for preoperative diagnosis. EGD findings were non-suggestive of this pathology in any of these cases. Superficial chronic gastritis was the most common finding in the endoscopic biopsy (60%). All the tumors found in the surgical specimen were of low or very low risk of malignancy, with less than 5 mitoses per 50 fields, less than 2 cm in diameter, and disease-free surgical margins. Cluster of differentiation (CD) 117 and CD 34 were positive in 100% of the cases. None of the patients required adjuvant therapy after the surgery. At 5-year follow-up, all patients were asymptomatic and disease free. CONCLUSION: The incidence of unsuspected GIST in LSG specimens in our series was low and similar to what has been reported. The lack of symptoms and the preoperative EGD findings were not suggestive of this diagnosis in any case. The degree of tumor malignancy was low in all patients and LSG was the definitive treatment, without recurrence at 5-year follow-up.

Tumor del estroma gastrointestinal de localización gástrica. Presentación de un caso / Gastrointestinal stromal tumor of gastric location. Case presentation

Los tumores estromales gastrointestinales son neoplasias que se originan en el tejido mesenquimal de la pared del tracto gastrointestinal. Se ha postulado que las células que originan estos tumores son las células intersticiales de Cajal, las cuales constituyen el marcapaso del tracto digestivo, se hallan en el plexo mientérico de Auerbach y responden al oncogén CD117, c-KIT. Pueden presentarse a cualquier edad pero tienen un pico de incidencia durante la sexta década de la vida. La localización más frecuente es en estómago. Tienen variedad de tamaños desde pocos milímetros hasta 20 cm o más. En este trabajo se presenta un paciente de 56 años de edad que fue ingresado en el centro hospitalario del territorio de Colón por presentar un cuadro de sangrado digestivo alto en forma de melena. Se le realizaron exámenes de laboratorio, estudios imagenológicos (Rx contrastado de estómago-duodeno, ultrasonido y TAC abdominal) y endoscopia digestiva superior. Se comprobó la presencia de una tumoración polipoide de 5 cm de diámetro en la región prepilórica gástrica. Fue sometido a operación quirúrgica y los resultados definitivos de Anatomía Patológica arrojaron un tumor del estroma gastrointestinal de bajo grado de malignidad (AU).

Gastrointestinal stromal tumors are neoplasias originated in the mesenchymal tissue of the gastrointestinal tract wall. It has been stated that cells originating these tumors are the Cajal´s interstitial cells, the digestive tract pacemaker, that are located in he Auerbach´s myoenteric plexus and answer to CD117, c-KIT oncogen. They may occur at any age but have an incidence peak during the sixth decade of life. The most common location is the stomach. There is a variety of sizes, from few millimeters to 20 cm or more. The case of a 56-years-old patient is presented in this work; he was admitted in the territorial hospital of Colon for presenting a high digestive bleeding in a melena form. Laboratory tests, imagine studies (contrasted Rx of stomach and duodena, abdominal ultrasound and CAT) and high digestive endoscopy were made. It was proved the presence of a 5 cm polypoid tumor in the gastric pre-pyloric region. It was surgically operated and the final results of Pathologic Anatomy showed a gastrointestinal stromal tumor of low level malignancy (AU).

Tumor del estroma gastrointestinal de localización gástrica. Presentación de un caso / Gastrointestinal stromal tumor of gastric location. Case presentation

Los tumores estromales gastrointestinales son neoplasias que se originan en el tejido mesenquimal de la pared del tracto gastrointestinal. Se ha postulado que las células que originan estos tumores son las células intersticiales de Cajal, las cuales constituyen el marcapaso del tracto digestivo, se hallan en el plexo mientérico de Auerbach y responden al oncogén CD117, c-KIT. Pueden presentarse a cualquier edad pero tienen un pico de incidencia durante la sexta década de la vida. La localización más frecuente es en estómago. Tienen variedad de tamaños desde pocos milímetros hasta 20 cm o más. En este trabajo se presenta un paciente de 56 años de edad que fue ingresado en el centro hospitalario del territorio de Colón por presentar un cuadro de sangrado digestivo alto en forma de melena. Se le realizaron exámenes de laboratorio, estudios imagenológicos (Rx contrastado de estómago-duodeno, ultrasonido y TAC abdominal) y endoscopia digestiva superior. Se comprobó la presencia de una tumoración polipoide de 5 cm de diámetro en la región prepilórica gástrica. Fue sometido a operación quirúrgica y los resultados definitivos de Anatomía Patológica arrojaron un tumor del estroma gastrointestinal de bajo grado de malignidad (AU).

Gastrointestinal stromal tumors are neoplasias originated in the mesenchymal tissue of the gastrointestinal tract wall. It has been stated that cells originating these tumors are the Cajal´s interstitial cells, the digestive tract pacemaker, that are located in he Auerbach´s myoenteric plexus and answer to CD117, c-KIT oncogen. They may occur at any age but have an incidence peak during the sixth decade of life. The most common location is the stomach. There is a variety of sizes, from few millimeters to 20 cm or more. The case of a 56-years-old patient is presented in this work; he was admitted in the territorial hospital of Colon for presenting a high digestive bleeding in a melena form. Laboratory tests, imagine studies (contrasted Rx of stomach and duodena, abdominal ultrasound and CAT) and high digestive endoscopy were made. It was proved the presence of a 5 cm polypoid tumor in the gastric pre-pyloric region. It was surgically operated and the final results of Pathologic Anatomy showed a gastrointestinal stromal tumor of low level malignancy (AU).

Comparative study of the different degrees of risk of gastrointestinal stromal tumor.

OBJECTIVE: To evaluate the applicability of the main categories of risk and morphological factors in the prognosis of gastrointestinal stromal tumors. METHODS: we retrospectively studied fifty-four cases of GIST, assessing the main prognostic factors of this neoplasis: risk levels, topography, size, mitotic index, necrosis, histological subtype and immunophenotype. We also verified their association and the reduction of overall survival. RESULTS: Univariate analysis showed that tumors with mitoses number greater than 5 per 50CGA (high-power fields), the presence of necrosis and a high risk for both the systems proposed by Fletcher and Miettinen had a significant association with reduced survival (p = 0.00001, 0.0056, 0.03 and 0.009, respectively). The remaining analyzed factors (size, histological subtype, topography and immunophenotype) had no such association. Multivariate analysis (Jacard index) showed that the Miettinen degree of risk was the one that best correlated with prognosis. CONCLUSION: the risk criteria of Fletcher and Miettinen are important in assessing the prognosis of patients with gastrointestinal stromal tumors, especially the latter, which adds to the mitotic index and the presence of tumor necrosis.

Temporal trends of incidence and survival of sarcoma of digestive tract including Gastrointestinal Stromal Tumours (GIST) in two areas of the north-east of Spain in the period 1981-2005: a population-based study.

INTRODUCTION: The diagnostic approach of Gastrointestinal Stromal Tumours (GIST) was established in 2002. Before this, GIST had been classified with a wide range of histological terms. This fact and the consideration of potential malignity of all these tumours led to a false perception of an increasing incidence. PURPOSE: This study aimed at evaluating the accuracy in registration of sarcoma of digestive tract and GIST and to elucidate the trends of incidence and survival of those. MATERIALS AND METHODS: We used data from two population-based cancer registries in Spain. In the Girona's Cancer Registry we previously reclassified all sarcoma of digestive tract performing c-kit to confirm GIST and analysed the time period 1994-2005. In Tarragona's Cancer Registry, where we analysed the time period 1981-2005, this reclassification was not done. RESULTS: We obtained a significant increasing trend in incidence of all sarcoma of digestive tract in the Tarragona Cancer Registry database, with an annual per cent of change of 3.87 but a non-statistically significant trend in incidence in the Girona Cancer Registry database. The incidence of GIST in Girona Cancer Registry was 1.24 cases/100,000 inhabitants/year. Survival rates did not change in time and was high in less aggressive GIST. The 5-year relative survival for low, intermediate and high risk of malignant behaviour GIST groups were, respectively, 80.5, 85.6 and 64.6 %. CONCLUSIONS: The increase in the incidence of GIST could be explained by the improvement in their diagnosis and registration. The survival of low and intermediate risk of malignant behaviour is high and close to normal population survival.

Gastrointestinal stromal tumors: diagnosis and treatment.

BACKGROUND: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms of the digestive tract. They originate from the interstitial cells of Cajal and are characterized by the overexpression of KIT protein (tyrosine kinase). Their prognosis has improved significantly with the discovery of imatinib mesylate for advanced GIST treatment. METHODS: We carried out a retrospective, descriptive study of GISTs diagnosed in our center during the past 5 years. We excluded patients with incidental diagnoses in the context of other pathologies because GIST did not affect outcome or prognosis. The variables studied were clinical characteristics, location, size, imaging techniques, resectability, neoadjuvant imatinib, surgical technique, histology, immunohistochemistry, prognostic classification of Fletcher, morbidity, monitoring, and disease-free and overall survival. RESULTS: Nineteen patients were diagnosed (14 males/5 females) with a mean age of 63 years (range: 30-84 years). Diagnosis was incidental in eight patients (42%). Tumor location of the remaining 11 patients (58%) was six tumors of the small intestine (55%), four gastric (36%) and one rectal (9%). Predominant gastrointestinal bleeding and anemia were diagnosed mainly by abdominal computed tomography (CT). At diagnosis, nine patients were considered resectable with radical intent (82%) and the other two patients (18%) received neoadjuvant treatment with a favorable response after 6 months. Three patients were treated with imatinib after surgery (33%). Median survival was 34 months (range: 5-58 months). CONCLUSIONS: Diagnosis of GIST is often incidental. The predominant clinical symptom is usually gastrointestinal bleeding and anemia and the most widely used imaging test is CT. Treatment is surgical unless advanced GIST is diagnosed, which will be treated with imatinib mesylate neoadjuvant therapy. A multidisciplinary approach to this pathology is essential, a fact that affects prognosis and patient survival.

Resultados del tratamiento quirúrgico de los tumores del estroma gastrointestinal (GIST) en la IV Región de Chile / Surgical outcomes of gastrointestinal stromal tumors (GIST)

Background: Despite that current knowledge regarding the pathology and treatment of Gastrointestinal Stromal Tumors (GIST) is widely available; most patients in the developing world and mainly in rural areas of developing countries have limited access to diagnostic technology and modern specific therapy such as imatinib. Objective: To review the management and outcomes of GISTs treated at the hospitals of the IV Region of Chile. Patients and Methods: This retrospective, observational and descriptive study was performed with data obtained from the medical records of 3 community hospitals were all surgical practice of the IV Region is performed. During the study period, 24 consecutive patients with GISTs at different localizations of the gastrointestinal tract were treated. Results: Five patients were operated on with the preoperative diagnostic of GIST, in 19 patients the diagnostic of GIST was suspected during the operation and confirmed by histology and immunohistochemistry. Most patients were operated on emergency grounds. Of 10 patients requiring imatinib therapy, only 2 are currently receiving the medication sponsored by an international foundation. Conclusions: There were no disparities in the standard surgical care of our patients. The main differences with published series from Chile and developed countries are the available technology to perform a preoperative diagnosis and the availability of imatinib for the treatment of metastatic and recurrent disease.

Introducción: A pesar de que el conocimiento actual sobre la patología y tratamiento de los tumores del estroma gastrointestinal (GIST) se encuentra ampliamente disponible, la mayoría de los pacientes en los países en desarrollo, principalmente en las áreas rurales, tienen un limitado acceso a la tecnología diagnóstica moderna y a tratamientos específicos como el imatinib. Objetivo: Revisión del manejo y resultados de los GIST tratados en los hospitales de la IV Región de Chile. Pacientes y Métodos: Estudio retrospectivo, observacional y descriptivo de la información obtenida de las fichas clínicas de 3 hospitales tipo 2 en los cuales se realiza toda la práctica quirúrgica de la IV Región. Durante el período estudiado, 24 pacientes consecutivos con GISTs en diferentes localizaciones fueron tratados quirúrgicamente. Resultados: Cinco pacientes fueron operados con el diagnóstico preoperatorio de GIST, en los otros 19 pacientes el diagnóstico se sospechó durante la cirugía y fue confirmado por histología e inmunohistoquímica. La mayoría de los pacientes fueron operados de urgencia. Diez pacientes fueron candidatos a tratamiento con imatinib, sólo 2 pacientes se encuentran actualmente en tratamiento gracias a una fundación internacional. Conclusiones: El tratamiento quirúrgico de nuestros pacientes es similar a las publicaciones nacionales e internacionales. Las diferencias se presentan en la disponibilidad de estudios de imagen para el diagnóstico preoperatorio y en la disponibilidad de imatinib para el tratamiento de las recurrencias y metástasis.

Tumores estromales gastrointestinales: evaluación clinicopatológica y sobrevida en el Hospital Nacional Edgardo Rebagliati Martins. Periodo 2002 - 2010 / Gastrointestinal estromals tumors: Clinical and pathologic evaluation and survival in the National Hospital Edgardo Rebagliati Martins. Period 2002 - 2010

INTRODUCCIÓN: Los Tumores Estromales Gastrointestinales (GISTs) son neoplasias poco frecuentes que se originan a partir de las células intersticiales de Cajal, tienen un amplio espectro de manifestaciones clínicas, su comportamiento biológico va de benigno a maligno y los hallazgos histopatológicos son variados, por lo que en la actualidad continúan siendo un tema de investigación. OBJETIVO: Determinar las manifestaciones clínicas, radiológicas, histopatológicas y sobrevida de los pacientes con diagnóstico de Tumor Estromal gastrointestinal en el Hospital Nacional Edgardo Rebagliati Martins (HNERM). MATERIAL Y METODOS: El presente es un estudio descriptivo, retrospectivo, transversal. El cual se realizó a partir de las historias clínicas de 103 pacientes con diagnóstico de Tumor estromal gastrointestinal confirmado mediante pruebas de inmunohistoquímica positivas a CD117 y/o CD34 que fueron evaluados y tratados en el HNERM, hospital de referencia nivelIv, desde Enero del 2002 hasta Diciembre de 2010. RESULTADOS: En los 103 pacientes estudiados el promedio de edad fue 64 años, siendo la mínima 30 años y la máxima 88 años. El 80.6 por ciento de casos fueron mayores de 50 años. Predominó el sexo femenino (52 por ciento). El tiempo de enfermedad promedio fue 7 meses. Las formas de presentación principal fueron: sangrado digestivo (48.3 por ciento), masa abdominal (18.2 por ciento), y dolor abdominal (15.1 por ciento). El diagnóstico se hizo por endoscopia (50.5 por ciento), ecografia (27.2 por ciento), tomografia (15.5 por ciento) y laparotomía exploratoria (6.8 por ciento). La prevalencia de los GISTs por órganos fue la siguiente: estómago 56.3 por ciento, yeyuno 18.5 por ciento, duodeno 8.7 por ciento, íleon 5.8 por ciento, recto 3.9 por ciento, mesenterio 2.9 por ciento, esófago 1.9 por ciento, peritoneo 1 por ciento y epiplon 1 por ciento. El promedio de tamaño de los GISTs fue 98mm, el 49 por ciento de los GISTs tuvieron un tamaño entre 50mm y 100 mm, el mayor tamaño alcanzado fue de 260 mm. El estadio tumoral fue: localizado en 70.9 por ciento, regional en 25.2 por ciento, y con metástasis el 3.9 por ciento de pacientes. Presentaron otro cáncer asociado el 9.7 por ciento de pacientes. El patrón histológico predominante fue el fusiforme 73.8 por ciento, seguido del epitelioide 13.7 por ciento y mixto 12.6 por ciento. El 84.5 por ciento de pacientes tuvieron bajo índice mitótico. Los exámenes de inmunohistoquímica mostraron que para todas las localizaciones la expresión de KIT (CD 117) fue de 94.17 por ciento, CD 34 77.5 por ciento, Vimentina 96.6 por ciento, NSE 84.9 por ciento, Alfa actina 52.7 por ciento, CD56 44.4 por ciento, S-100 32.3 por ciento y Actina 20.0 por ciento. Los marcadores Sinaptofisina, Citoqueratina, Desmina, Cromogranina, Mioglobina y HMB 15 fueron negativos. En general las características tomo gráficas más frecuentes fueron: turnor de aspecto heterogéneo (43.6 por ciento), áreas de baja atenuación central (41 por ciento), refuerzo periférico con contraste (38.5 por ciento), patrón de crecimiento extralurninal (35.9 por ciento) y masa sólida (30.8 por ciento). La distribución según la clasificación de riesgo fue la siguiente: muy bajo riesgo 3.9 por ciento, bajo riesgo 28.2 por ciento, riesgo intermedio 37.7 por ciento y alto riesgo 30.1 por ciento. La resección quirúrgica completa se realizó en el 87.4 por ciento de pacientes y el 4.9 por ciento de pacientes recibió lmatinib. La sobrevida global acumulada a 5 años fue 31.07 por ciento. En el análisis bivariado, se encontró asociación estadística entre el haber sobrevivido y: la no presencia de cáncer asociado p=0.004, CD 34 p=0.01, índice mitótico bajo p=0.00 y tratamiento quirúrgico recibido p=0.000. En el análisis multivariado, se encontró asociación estadística de mayor sobrevida de los pacientes con menor tamaño del tumor p=0.015 (lC -3.67, -0.41), estadio tumoral localizado p=0.036 (lC -5.83, -0.19), menor índice mitótico p=0.038 (lC -0.86, 0.02), paciente asintomático p=0.009 (lC 1.25,8.62), no recidiva del tumor p=0.01 (lC -8.49, -1.17), y el no presentar metástasis p=0.001 (lC 2.66, 10.62). CONCLUSIONES: En general las manifestaciones clínicas, radio lógicas, histopatológicas y sobrevida de los pacientes con diagnóstico de Tumor Estromal gastrointestinal en el HNERM fueron similares a lo que reporta la literatura internacional. Los factores que se asociaron a mayor sobrevida fueron: haber recibido tratamiento quirúrgico, pacientes con menor tamaño del tumor, estadio tumoral localizado, índice mitótico bajo, paciente asintomático, no recidiva del tumor, no metástasis y no cáncer asociado

[Clinical, endoscopic and histologic profile of the Gastrointestinal Stromal Tumours in the Rebagliati Hospital]. / Perfil Clínico, Endoscópico e Histológico de los Tumores Estromales Gastrointestinales en el Hospital Rebagliati.

INTRODUCTION: Gastrointestinal Stromal Tumors (GIST) are lesions which origin from Intersticial Cells of Cajal that may be asymptomatic or cause complications like digestive bleeding or malignant transformation. AIM: To determine the clinical, endoscopic and histological profile, as well as the main demographic features of patients with GIST in Hospital Edgardo Rebagliati (Lima-Perú) from January 2002 to December 2004. METHODS: Descriptive, transversal and retrospective study in which we reviewed clinical and anatomopathological archives from 34 patients with GIST during the above mentioned period. The collected data were placed on a sheet of recollection. RESULTS: There was male predominance (58.82%).The most affected age group was 71-80 years old (26.47%). The average age was 64.7 years old. The most frequent clinical presentation was abdominal pain (47.06%) followed by melena (44.12%). Diagnosis was made by endoscopic method in 58.82%. The main histological type was spindle (47.06%). There was high risk of malignancy in 50% and the most often involved site was stomach (61.77%). Endoscopicall the characteristic image was a submucosal tumor with ulceration, the average size was 6.44 cm. There was significant statistical relation (p<0.05) between endoscopic method and melena. CONCLUSION: Patients with GIST in HNERM are mostly men, older than 60 years that come with abdominal pain and melena, being endoscopy the best method to diagnose if patients present melena. Predominating histology was fusiform.

Tumor del estroma gastrointestinal (TEGI): un estudio de su frecuencia y perfil de inmunohistoquímica / Gastrointestinal stromal tumors: analysis of 51 cases, immunohistochemical expression

Background: Gastrointestinal Stromal Tumors (GIST) have a mesenchymal origin and correspond to 1 percent of all gastrointestinal tumors. They have a benign behavior in approximately 75 percent of cases. They express CD117, CD34, smooth muscle actin, S-100 and desmin, markers that are useful in the differential diagnosis of smooth muscle tumors and those of neurogenic origin. Aim: To report our experience with GIST. Material and Methods: A retrospective, observational study. The pathology reports of GIST in the period 2004-2008 were reviewed. Immunohistochemical expression, pathological grade, mitotic index and histological patterns were reviewed. The medical records of patients were reviewed to obtain age and gender, location, size and presence of metastases. Results: A total of 51 GIST were identified, coming from 21 males and 30 females. Nineteen tumors were located in the small bowel, 18 in the stomach, four in the rectum, two in the colon and in five, the location was not specified. In 28 cases, the pathological pattern was spindle cell, in 12 mixed, in six epithelioid, in three pleomorphic, in one signet ring cell and giant cell in one. Forty nine percent of tumors were of high grade. Metastases were found in the liver in two cases, in the omentum in two and in the spleen, kidney, retroperitoneum and pancreas, in one case each. Two had lymph node involvement. Conclusions: GIST tumor corresponded to a 0.12 percent of all pathology reports during the study period. Most tumors in this series were of high grade.

Introducción: Los Tumores del Estroma Gastrointestinal (TEGI) son de origen mesenquimal comprendiendo el 1 por ciento de todos los tumores GI. Son benignos del 70 a 80 por ciento. Expresan CD117, CD34, actina de músculo liso, S-100 y desmina, marcadores útiles en el diagnóstico diferencial de tumores de músculo liso y tumores de origen neurogénico. Material y Método: Es un estudio retrospectivo y descriptivo. Se revisaron los reportes en el período 2004-2008 registrados como TEGI, valorando la expresión Inmunohistoquímica, grado histológico, índice mitótico, y patrones histológicos. Del reporte histológico se obtuvo la edad y sexo del paciente, localización, tamaño y metástasis. Resultados: Se recolectaron 51 casos corroborados como TEGI. Encontrando una prevalencia del sexo femenino (30) y una edad media de 52 años. Las localizaciones fueron: Intestino delgado (19), estómago (18), no especificado (5), recto (4), colon (2), retroperitoneo (2), no encontramos en esófago. Los patrones encontrados fueron el fusocelular (28), mixto (12), epitelioide (6), pleomórfico (3), células en anillo de sello (1), células gigantes (1). La mayoría (49 por ciento) fue de alto grado, presentando metástasis a hígado (2), ganglios (2), epiplón (2), bazo (1), riñón (1), retroperitoneo (1) y páncreas (1). Discusión: Se realizaron un total de 41.035 estudios histopatológicos, de los cuales 51 casos corresponden a LEGI, esto equivale al 0,12 por ciento. Encontramos tumores en los que su morfología, tamaño e índice mitótico fueron de bajo grado y presentaron metástasis y recidivas al momento del diagnóstico. Veinticinco casos fueron de alto grado (49 por ciento), lo cual es mayor a lo reportado por la literatura 20-30 por ciento, probablemente porque este es un hospital de concentración y generalmente los pacientes acuden a atención médica en una etapa avanzada de la enfermedad.

Occurrence of other tumors in patients with GIST.

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.

Tumores estromais gastrointestinais (GIST): revisão da literatura / Gastrointestinal stromal tumors (GIST): a literature review

Os tumores estromais gastrointestinais (GIST) são os tumores mesenquimais mais comuns do trato gastrointestinal (TGI) e representam cerca de 1% de todos os tumores do TGI. Estes tumores teriam origem nas células precursoras das células intersticiais de Cajal. 95% dos GIST expressam a proteína CD117, receptor transmembrana, que sofre várias mutações e ativações, proporcionadas pelo proto - oncogene KIT e irá desenvolver finalmente a neoplasia. Eles se desenvolvem com a mesma prevalência em homens e mulheres, geralmente acima de 50 anos. A maior incidência é observada entre a quinta e a sexta década de vida, podendo-se desenvolver em qualquer parte do TGI, contudo o estômago é a localização mais comum... A avaliação do prognóstico vai depender principalmente do tamanho do tumor e o índice mitótico. A adequada compreensão e utilização dos critérios diagnósticos e classificação dos GISTs é fundamental para o tratamento do paciente.