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)

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).

Gastrointestinal stromal tumor: Analysis of outcome and correlation with c-kit status in Indian population.

AIMS: The aim of the present study is to analyse the outcome and genotypic pattern of metastatic GIST patients which is largely unknown in India. MATERIALS AND METHODS: The present study was a retrospective analysis of 24 patients of metastatic GIST. The case records were analysed for clinical profile, treatment response and prognostic factors. The archival samples were retrieved for c‑kit mutation analysis in all but 5 patients for mutation analysis. RESULTS: The median age of the study population was 56 years. At a median follow up of 29 months, the PFS was 45% at 2 years. Activating c‑kit mutations were detected in 10 cases (52.6%). 80% of the mutations were located in Exon 11. CONCLUSIONS: The outcome of metastatic GIST patients has definitely improved from a virtually incurable state to a disease where median OS has reached 60 months. The genotype of Indian patients with GIST may be different from the western population which needs to be confirmed in a larger study.

Clinicopathologic spectrum of gastro intestinal stromal tumours - Experience at a tertiary care center.

Context: Accurate diagnosis of Gastrointestinal stromal tumors (GISTs) and categorization of risk groups is crucial in the wake of targeted drug therapy. Aim: This study was undertaken to obtain insight into the clinicopathologic features of GISTs. Materials and Methods: Retrospective study of 20 cases of c-KIT (CD 117) positive GISTs .Various histologic parameters were studied. Immunohistochemical panel included CD-34, Smooth muscle actin, S-100, Desmin, and Vimentin. Risk categorization was done as per standard protocol. Clinical details and follow up were retrieved from cases files. Results: Of the total 20 cases, 15 were males. The mean age of presentation was 52 years. 50 % of the cases presented with gastro intestinal bleeding .The cases were categorized as high risk (60%), intermediate risk (20%), low risk (15%) and very low risk (5%). Stomach and small intestine were commonly involved and in 6 of the cases there were metastatic nodules in the mesentery/omentum/ peritoneum. Spindle cell morphology was the commonest histological pattern (55%). Nuclear pleomorphism, perivascular whorling, mucosal and fat invasion were seen only in the high risk group. CD-34, SMA and S-100 were positive in 90%, 30%, and 10% of the cases, respectively, with consistent Desmin negativity. Conclusions: Cases of GISTs in our clinical setting presented a decade earlier and mostly belonged to the high risk group. Certain histological features such as necrosis, and perivascular whorling were more commonly associated with the high risk groups.

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.

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.

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.

Tumor estromal intestinal / Intestinal stromal tumor

Los tumores estromales son un tipo de neoplasias de distintas líneas de diferenciación y de muy diverso comportamiento clínico. Son tumores mesenquimatosos originados en la pared muscular de vísceras huecas. Debido a su baja incidencia, existen pocas series publicadas. También es conocida la dificultad de predecir el riesgo de que estos tumores metasticen. Los criterios para determinar el grado de malignidad de los tumores estromales no están claramente definidos, por lo que el tratamiento quirúrgico de estas lesiones es controvertido. Generalmente se recomienda la resección completa pero limitada de estos tumores, dada la menor complejidad técnica, la menor morbilidad y el idéntico pronóstico al de las resecciones más amplias(AU)

Stromal tumors are a type of neoplasias of various lines of differentiation and of very diverse clinical behavior. They are mesenchymatous tumors originated on the muscular wall of hollow viscera. Due to their low incidence, a few series have been published. It is also known the difficulty to predict their risk for metastasizing. The criteria to determine the malignancy degree of the stromal tumors have not been clearly defined. That's why, the surgical treatment of these lesions is controverted. Generally, it is recommended the complete but limited resection of these tumors, taking into account the minor technical complexity, the lower morbidity and the prognosis identical to that of the wider resections(AU)

Gastrointestinal stromal tumors: a demographic, morphologic and immunohistochemical study.

BACKGROUND AND AIM: There is limited published information on gastrointestinal stromal tumors (GIST) in the South Asian region. This study was conducted to describe the demographic characteristics, organ distribution and frequency of risk categories in cases of GIST referred to a tertiary hospital. METHODS: Data pertaining to 37 cases of gastrointestinal stromal tumors received at the histopathology section of the Aga Khan University Hospital between December 2004 and July 2005 were analyzed. Immunohistochemical stains including vimentin, CD34, CD117 (c-kit), ASMA, desmin and S-100 were performed. RESULTS: Of 37 tumors, 24 (64.9%) were from males. The mean age of the patients was 50.0 years (95% CI 45.3-54.6). Tumors were categorized as high risk (27 cases; 69.2%), intermediate risk (4 cases; 10.3%) and low risk (3 cases; 7.7%). The most common site of involvement was the stomach (29.7%), followed by small bowel (24.3%), mesentery (10.8%), pancreas (8.1%), rectum (2.7%) and retroperitoneum (2.7%). In 21.6% of cases, the site of origin was not specified. The mean age at diagnosis was 50.9 years (95% CI 45.5 - 56.3) in the high risk and 44.8 years (95% CI 28.6 - 60.9) in the intermediate risk category. CONCLUSIONS: Cases of GIST referred to us were more frequently from men, most commonly from stomach or small bowel, mostly in the high risk category, and presented a decade earlier than in other reported series.

Tumor de estroma gastrointestinal (gist) / Gastrointestinal stromal tumor (gist)

Os autores fazem uma revisão bibliográfica dos tumores do estroma gastrointestinal (gist), abordando a incidência, a fisiopatologia, prognóstico e o tratamento.

Synchronous gastric stromal tumor and cecal adenocarcinoma.

Gastrointestinal stromal tumors are rare intestinal tumors. There have been reports of this tumor occurring with other conditions and tumors. We report a 55-year-old man who presented with a gastric stromal tumor and cecal adenocarcinoma, necessitating right hemicolectomy and partial gastrectomy at the same sitting.

Gastrointestinal Stromal Tumors in Koreans: It's Incidence and the Clinical, Pathologic and Immunohistochemical Findings

Seven hundred forty seven cases of gastrointestinal stromal tumors (GISTs) in Koreans who were diagnosed between 2001 and 2002 were analyzed to evaluate their occurrence and their clinical, pathologic and immunohistochemical findings. The most frequent location of tumor was in the stomach (63%), followed by the small intestine (30%), the colorectum (5%), and the esophagus (2%). c-kit expression was found in 93.6% of the cases, while CD34, SMA and S-100 protein was positive in 80.1%, 28.2%, and 20.2%, respectively. c-kit positivity was high in the stomach (94.2%) and small intestine (94.6%), while it was relatively low in the colorectum (85.0%), and esophagus (81.2%). The positivity for CD34 was correlated with the higher risk of GISTs (p=0.04). Follow up of the patients showed that 58 primary GISTs patients died and 20 of these patients were recurrent or metastatic at the time of diagnosis. The pathologic diagnosis to predict the risk of aggressive behavior of GISTs was correlated with the numbers of tumor, clinical stage, epithelioid histologic type, cellularity, cellular atypia, necrosis, and mucosal invasion (p= 0.00). GISTs with a poor prognosis were closely related to the clinical stage at presentation, the locations of the tumor, and the ages of the patients.

Câncer gástrico: experiência de um hospital geral / Gastric cancer: experience of a general hospital

Foram revisados 115 prontuários de pacientes internados no Hospital Nossa Senhora da Conceição, em um período de 2 anos, com diagnóstico de câncer gástrico confirmado histolopatologicamente. A incidência foi maior entre homens (2:1), sendo identificada relação entre câncer gástrico e tabagismo, presente em 58,2% dos pacientes. Não foi possível estabelecer relação etiológica com alcoolismo e com história pessoal ou familiar de outras neoplasias. A apresentação endoscópica mais freqüente foi tumor ulcerado (44,7% dos casos), sendo adenocarcinoma o tipo histopatológico mais prevalente. No estadiamento, 67% dos pacientes que realizaram ecografia ou tomografia abdominal apresentaram alterações que sugeriram a presença do tumor ou sua disseminação e 56,5% dos pacientes apresentavam neoplasia gástrica irressecável no momento do diagnóstico. Novos ensaios clínicos randomizados são necessários para o reconhecimento dos fatores de risco para esta neoplasia e possibilitar o estabelecimento de medidas de prevenção e detecção precoce do câncer gástrico em pacientes de alto risco (AU)

The registers of 115 patients with diagnosis of gastric cancer (histopathologically confirmed) from Hospital Nossa Senhora da Conceição in a 2 year period were studied. The incidence was larger among men (2:1), and a relationship between gastric cancer and tobacco smoking, present in 58,2% of the patients, was identified. It was not possible to establish etiological relationship with alcoholism and personal or family history of another neoplasm. The more frequent endoscopic presentation was ulcerated tumor (44,7% of the cases), being adenocarcinoma the more prevalent histopathologic type. Sixty seven percent of the patients that were submitted to abdominal ecography or tomography presented alterations that suggested the presence of the tumor or its dissemination, and 56,5% of the patients presented irresecable gastric cancer in the moment of the diagnosis. New randomized clinical trials are necessary for the recognition of the risk factors for this neoplasm and then establish prevention measures and early detection of gastric cancer in high risk patients (AU)