GIST duodenal asociado a hemorragia digestiva alta: reporte de caso / Duodenal GIST associated with upper gastrointestinal bleeding: case report

Resumen Introducción Los tumores del estroma gastrointestinal (GIST) corresponden al 1% de todas las neoplasias gastrointestinales, sin embargo, sólo el 3-5% de estos se desarrollan en el duodeno. Objetivo Reportar el caso de un paciente masculino con localización atípica de un tumor de estroma gastrointestinal y su manejo. Caso clínico paciente masculino de 50 años con antecedente de traumatismo encefalo craneano (TEC) con daño orgánico cerebral secundario, tabaquismo, consumidor de alcohol ocasional y sometido a quistectomía branquial en la infancia, que consulta en el servicio de urgencias por cuadro de hemorragia digestiva alta con compromiso hemodinámico. Tras realizar endoscopia digestiva alta (EDA), resonancia nuclear magnética (RNM) y tomografía computada (TC) de abdomen, se pesquisa masa tumoral en segunda porción de duodenal. Discusión A pesar de que la presentación clínica de los GIST es variable, lo más frecuente es que sean pacientes asintomáticos. En algunas ocasiones, al igual que en este reporte, pueden presentarse con dolor abdominal y/o hemorragia digestiva alta. El diagnóstico preoperatorio fue difícil ya que el estudio con imágenes (TC, RNM, EDA) sólo permite establecer la sospecha; el diagnóstico definitivo se realizó con biopsia (no contamos con endosonografía en nuestro centro). Debido a los sitios de reparo anatómico, no existe una cirugía estandarizada; en este caso, debido a la localización, infiltración y características, se decidió realizar una pancreatoduodenectomía.

Introduction Gastrointestinal stromal tumors (GIST), corresponds to 1%, of all gastrointestinal neoplasms, however, only 3%-5% developed in duodenum. Aim To report a case of a male patient with atypical location of gastrointestinal stroma tumor and the treatment proposed. Case report 50-year-old male patient, with medical history of organic brain damage secondary a traumatic brain injury, smoker, occasional alcohol consumer and branquial cystomy during childhood. Consulted in the emergency department for a high digestive hemorrhage case with hemodynamic compromise. Upper digestive endoscopy, computed tomography and nuclear magnetic resonance were performed, which impresses tumor-like lesion in the second duodenal portion. Discussion Although the GIST clinical presentation is variable, most often they are asymptomatic patients. In some times, as in this report, they may present with abdominal pain and/or upper gastrointestinal bleeding. The preoperative diagnosis was difficult, because the imaging study (CT, RNM, EDA) only stablished the suspicion and the final diagnosis was made by biopsy (we don't have endosonography in our center). Due to the anatomic repair, there is not a standardized surgery, in this case, due tumor location, infiltration and characteristics, it was decided to perform a pancreatoduodenectomy.

Caracterización de paciente con tumores estromales gastrointestinales en Hospital Clínico-Quirúrgico "Arnaldo Milián Castro" / Characterization of Patients with Stromal Gastrointestinal Tumors in Arnaldo Milián Castro Clinical-Surgical Hospital

Introducción: El tumor estromal gastrointestinal es la neoplasia mesenquimal más frecuente en el tracto digestivo, su diagnóstico y tratamiento aun es controvertido por ser infrecuente. Objetivo: Caracterizar los pacientes con tumores estromales gastrointestinales atendidos en nuestro servicio. Métodos: Se realizó un estudio observacional descriptivo longitudinal en pacientes con tumores estromales gastrointestinales atendidos en el servicio de cirugía del Hospital Clínico-Quirúrgico "Arnaldo Milián Castro" desde 2015 hasta 2018. La muestra fue de 17 pacientes. Resultados: Predominaron los pacientes entre 60 y 69 años (35,3 por ciento) del sexo femenino (58,82 por ciento). El dolor abdominal como síntoma más frecuente (58,82 por ciento). El (47,1 por ciento) de los tumores midieron más de 10 cm, celularidad fusiforme (58,8 por ciento), índice mitótico menor de 5 (70,6 por ciento), sin patrón de crecimiento infiltrante (70,6 por ciento). La metástasis ausente en el (82,4 por ciento). Presente con igual frecuencia en estómago e intestino delgado (N = 8), fue igual la presencia de comportamiento agresivo bajo y alto (35,3 por ciento). Predominaron los marcadores CD.117 (41,2 por ciento) y CD.34 (35,3 por ciento). La recesión segmentaria de intestino delgado con anastomosis término-terminal fue la técnica quirúrgica más empleada (35,3 por ciento). Conclusiones: A pesar de ser mayormente grandes y encontrarse en porciones altas del tubo digestivo, muchos presentaron índice mitótico bajo, no obstante, un grupo considerable presentó comportamiento agresivo. Si bien es cierto que casi la totalidad de pacientes egresaron vivos, falta un seguimiento en el centro, lo cual resultaría interesante evaluar en futuros estudios(AU)

Introduction: Gastrointestinal stromal tumor is the most frequent mesenchymal neoplasm in the digestive tract, its diagnosis and treatment is still controversial because it is infrequent. Objective: To characterize the patients with gastrointestinal stromal tumors treated in our service. Methods: A longitudinal descriptive observational study was carried out in patients with gastrointestinal stromal tumors treated in the surgery service of the "Arnaldo Milián Castro" Clinical-Surgical Hospital from 2015 to 2018. The sample consisted of 17 patients. Results: Female patients between 60 and 69 years old (35.3 percent) predominated (58.82 percent). Abdominal pain as the most frequent symptom (58.82 percent). Tumors (47.1 percent) measured more than 10 cm, spindle cell cellularity (58.8 percent), mitotic index less than 5 (70.6 percent), and no infiltrative growth pattern (70.6 percent). Metastasis absent in (82.4 percent). Present with equal frequency in the stomach and small intestine (N = 8), the presence of low and high aggressive behavior (35.3 percent) was the same. The markers CD.117 (41.2 percent) and CD.34 (35.3 percent) predominated. Segmental recession of the small intestine with end-to-end anastomosis was the most widely used surgical technique (35.3 percent). Conclusions: Despite being mostly large and found in high portions of the digestive tract, many presented a low mitotic index, however, a considerable group presented aggressive behavior. Although it is true that almost all the patients were discharged alive, there is a lack of follow-up at the center, which would be interesting to evaluate in future studies(AU)

Identification and inner relation between gastrointestinal stromal tumor and intra-abdominal desmoid tumor / 中华胃肠外科杂志

Intra-abdominal desmoid tumor (IADT) and gastrointestinal stromal tumor (GIST) are both mesenchymal tumors mostly found in gastrointestinal tracts and easily misdiagnosed, which would directly damage the survival prognosis and quality of life of patients. With the advent of the era of precision medicine, the understanding of the above two diseases is more in-depth, and the requirements for accurate diagnosis and individualized precision treatment are more stringent. Moreover, there seems to be some internal relationship between IADT and GIST, and the lack of systematic research and discussion makes clinical decision-making and patient management easy to fall into traps and misunderstandings. Therefore, this paper reviews the clinical characteristics, pathogenesis and treatments of the two, and explore their differences and internal relations, so as to provide research and practical reference for promoting more precise and individualized diagnosis and treatment regimens.

Pay attention to misdiagnosis and differential diagnosis of gastric gastrointestinal stromal tumor / 中华胃肠外科杂志

Due to the lack of typical symptoms and imaging findings, gastric gastrointestinal stromal tumor (GIST) is easy to be misdiagnosed as other gastric tumors clinically. In clinical practice, clinicians should adopt the multidisciplinary team model, fully understand the characteristics of gastric GIST, grasp the key points of its differential diagnosis from other gastric tumors to achieve precise diagnosis and treatment. This paper analyzes the causes of misdiagnosis and elucidates the differential diagnosis of gastric GIST, aiming to improve the accuracy of preoperative diagnosis, reduce the misdiagnosis, and improve treatment outcomes.

Act following etiquette and change with time: analysis and discussion on the difference between the current guidelines and clinical practice of gastrointestinal stromal tumor / 中华胃肠外科杂志

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the gastrointestinal tract. At present, many professional academic groups and associations at home and abroad have released guidelines for the diagnosis and treatment of GIST. In 2020, Gastrointestinal Stromal Tumor Expert Committee of China Society of Clinical Oncology (CSCO) issued the first "CSCO gastrointestinal stromal tumor diagnosis and treatment guidelines" in China. In the same year, NCCN also released the first "NCCN guideline for the diagnosis and treatment of gastrointestinal stromal tumors (first edition in 2021)" by separating GIST-related content from the guideline for soft tissue sarcoma. However, there are many inconsistencies between the guidelines' recommendation and clinical practice in China. For example, in the aspect of diagnosis, the emphasis on and implementation of molecular detection are variable, the format of molecular detection report is not unified or standardized, the selection of detection methods is inappropriate, the diagnosis of wild-type GIST is not standardized, the risk assessment is improper, and the use of EUS-FNA is relatively seldom; in the aspect of drug therapy, there are some problems, such as the lack of standardization in dose and course of targeted drugs, and off-label medication such as cross line therapy; in terms of surgical treatment, there are also some problems, such as the clinical application scenarios of laparoscopic surgery exceed the recommendations of the guidelines, and the general description of surgical intervention of advanced GIST in the guidelines cannot well guide specific clinical practice. In this context, on the one hand, clinicians need to understand the academic frontier and standardize their medical behavior with the latest guidelines; on the other hand, clinicians need to consider the scientific rationale and accessibility of treatment on some clinical problems that are not clear in the guidelines, and take whether patients can benefit the most as the criteria.

Pancreatitis aguda posduodenopancreatectomía cefálica con complicaciones locales / Acute pancreatitis with local complications after cephalic pancreaticoduodenectomy

RESUMEN Antecedentes: la pancreatitis aguda posduodenopancreatectomía cefálica inmediata es una complica ción cuya frecuencia puede llegar al 55% y condiciona la aparición de fístula pancreática. Objetivo: describir el manejo de 3 pacientes que presentaron pancreatitis aguda posduodenopancrea tectomía con complicaciones locales y realizar una revisión de la literatura. Material y métodos: se revisó una base de datos prospectiva de resecciones pancreáticas. Se identifi caron los pacientes con diagnóstico de pancreatitis aguda con lesiones locales posterior a la realización de duodenopancreatectomía. Se definió fístula pancreática de acuerdo con la clasificación del ISGPF y pancreatitis como la elevación de la amilasa o lipasa tres veces por encima del máximo valor sérico normal en asociación con dolor abdominal o confirmación radiológica. Resultados: entre 2008 y 2019 los autores realizaron 260 duodenopancreatectomías. Tres pacientes presentaron pancreatitis posoperatoria con complicaciones locales. Conclusiones: la pancreatitis aguda posoperatoria es una complicación de una frecuencia elevada. La mayoría de ellas se resuelven en forma espontánea. Pocos pacientes presentan complicaciones locales que pueden requerir tratamiento percutáneo o quirúrgico, predisponiendo al desarrollo de fístulas a veces de difícil manejo. No hay forma de prevenir la aparición de la fístula pancreática. El tratamiento de las complicaciones locales se realizará de acuerdo con su aparición y repercusión, pudiendo requerir desde la colocación de un drenaje percutáneo hasta la pancreatectomía total.

ABSTRACT Background: The incidence of acute pancreatitis immediately after cephalic pancreaticoduodenec tomy is up to 55% and is associated with the development of pancreatic fistula. Objective: The aim of this study is to report three cases of acute pancreatitis after pancreaticoduode nectomy with local complications with a review of the literature. Material and methods: The information about pancreatic resections was retrieved from a prospective database. Patients with diagnosis acute pancreatitis with local lesions immediately after pancreatico duodenectomy were identified. Pancreatic fistula was defined according to the International Study Group on Pancreatic Fistula (ISGPF) Definition and pancreatitis was defined as serum amylase or lipase >3x upper limit of normal associated with abdominal pain or imaging criteria. Results: A total of 260 pancreaticoduodenectomies were performed between 2008 and 2019. Three patients developed postoperative acute pancreatitis with local complications. Conclusions: Postoperative acute pancreatitis is a common complication that solves spontaneously in most cases. Few patients present local complications that may require percutaneous or surgical treatment, which may predispose to the development of fistulas that are sometimes difficult to mana ge. There is no way to prevent pancreatic fistulas. Local complications will be treated according to their occurrence and impact, and may require a variety of procedures, ranging from percutaneous drainage to total pancreatectomy.

A rectal gastrointestinal stromal tumor - a plea for neoadjuvant imatinib and TAMIS / Tumor estromal gastrointestinal no reto - um caso de uso de imatinibe neoadjuvante e TAMIS

Abstract Here we describe an infrequent case of gastrointestinal stromal tumor of the rectum in a 57 year-old man with spindle cell neoplasm probably gastrointestinal stromal tumor and CT scan showed tumor from the anterior rectal wall and offered abdominoperineal resection for the same. The patient was started on imatinib and had a significant reduction in symptoms. The patient was reassessed with the CT scan, which showed a reduction in tumor size and Transanal minimally invasive surgery was planned for the patient. Use of imatinib prior to surgical resection to attain the reduced size of the tumor within the limit of resection is an attractive approach. Since tumor development can happen rapidly again after substantial tumor shrinkage, the best time to operate depending on resectability and the maximum therapeutic outcome remains divisive.

Resumo No presente estudo, os autores descrevem um caso raro de tumor estromal gastrointestinal no reto em um homem de 57 anos que se apresentou com neoplasia de células fusiformes, com provável tumor estromal gastrointestinal. A tomografia computadorizada demonstrou tumor na parede anterior do reto e foi sugerida sua ressecção abdominoperineal. O paciente iniciou tratamento com imatinibe e apresentou uma redução significativa nos sintomas. O paciente foi reavaliado por tomografia computadorizada, que evidenciou redução do tamanho do tumor; portanto, foi indicada cirurgia transanal minimamente invasiva. O tumor era ressecável e foi necessário um extenso acompanhamento para romper o órgão, de forma a alcançar a ressecção máxima; caso contrário, o tumor estromal gastrointestinal também seria irressecável. O uso de imatinibe antes da ressecção cirúrgica para reduzir o tamanho do tumor dentro do limite de ressecção é uma abordagem interessante. Como o tumor pode se crescer rapidamente após ser substancialmente reduzido, a literatura ainda apresenta controvérsias quanto ao melhor momento para operar e quanto ao melhor desfecho terapêutico.

Páncreas ectópico, diagnóstico diferencial de lesión gástrica subepitelial: reporte de caso y enfrentamiento inicial / Ectopic pancreas, differential diagnosis of subepithelial gastric lesion: case report and initial confrontation

Subepithelial lesions are generally an incidental diagnosis with an prevalence of 0.4%. These tumors represent a great diagnostic challenge, mainly when ruling out potentially malignant lesions, such as gastrointestinal stromal tumor (GIST), lymphomas and carcinomas. Among the many differential diagnosis, the ectopic pancreas arises with an prevalence of 1-2% in general population. The first diagnostic approach is performed using upper digestive endoscopy, computed tomography and endosonography. This last one has a diagnostic performance of less than 50%, which increases to 90% when it is associated with a histopathological examination. There is no current consensus regarding the management and monitoring of these lesions. Based on the imaging and histological characteristics, the possibilities range from observation to endoscopic or surgical resection. In this context, we will present a clinical case of ectopic pancreas as an incidental finding, and afterwards the diagnostic and therapeutic breakdown of subepithelial lesions.

Las lesiones subepiteliales son pesquisadas generalmente de manera incidental, con una prevalencia de 0,4%. Estos tumores suponen un gran desafío diagnóstico, principalmente al momento de descartar lesiones potencialmente malignas, como el tumor estromal gastrointestinal (GIST), linfomas y carcinomas. Dentro de los posibles diagnósticos, surge el páncreas ectópico, con una prevalencia de hasta 1-2% en la población general. La primera aproximación diagnóstica se realiza mediante endoscopia digestiva alta, tomografía computarizada y la endosonografía, ésta última con un rendimiento diagnóstico menor del 50%, que aumenta hasta el 90% al asociar el examen histopatológico. No existe consenso actual respecto al manejo y seguimiento de estas lesiones, que según sus características imagenológicas e histológicas, va desde la observación hasta la resección endoscópica o quirúrgica. En este contexto, se presenta un caso clínico de páncreas ectópico como hallazgo incidental y el desglose diagnóstico y terapéutico de las lesiones subepiteliales.

Valor del ultrasonido endoscópico en el diagnóstico de las lesiones subepiteliales del tracto gastrointestinal / Value of endoscopic ultrasound in diagnosis of subepithelial lesions of the gastrointestinal tract

Introducción: El ultrasonido endoscópico (USE) es el método de mayor valor y el que con más frecuencia se emplea en la evaluación de las lesiones subepiteliales del tracto digestivo, como lo han demostrado grandes estudios multicéntricos. Objetivo: Describir la experiencia con el uso del ultrasonido endoscópico en el diagnóstico de las lesiones subepiteliales del tracto gastrointestinal. Método: Se realizó un estudio descriptivo longitudinal prospectivo desde mayo 2016 hasta enero 2018 en el Centro de Investigaciones Médico Quirúrgicas (CIMEQ) de La Habana, Cuba. La muestra quedó conformada por un total de 26 pacientes en los que predominó el sexo femenino con edades comprendidas entre 43 y 80 años. Resultados: La dispepsia fue el síntoma más reportado, así como los tumores del estroma gastrointestinal, seguido de los lipomas el diagnóstico más frecuente. En la mayoría de los pacientes se decidió una conducta quirúrgica. Conclusiones: El ultrasonido endoscópico es un método seguro, de mínima invasión y nos permite decidir la conducta a seguir en la mayoría de los pacientes con lesiones subepiteliales(AU)

Introduction: Endoscopic ultrasonography is the method of highest value and the most frequently used in the evaluation of subepithelial lesions of the digestive tract, as it has been shown in large multicenter studies. Objective: To describe the experience gained in the use of endoscopic ultrasonography for the diagnosis of subepithelial lesions of the gastrointestinal tract. Method: A prospective, longitudinal and descriptive study was conducted in the Center of Medical and Surgical Research of Havana from May 2016 to January 2018. The final sample was made up of 26 patients, mainly women, aged 43 to 80 years. Results: Dyspepsia was the most reported symptom as well as the most frequent diagnosis was gastrointestinal stroma tumors followed by lipomas. Surgical treatment was decided to be applied in most of these patients. Conclusions: The endoscopic ultrasonography is a safe, minimally invasive method that allows making decisions on the behavior to be followed in the treatment of most of the patients with subepithelial lesions(AU)

Video Caso Clínico: Tumores Subepiteliales del Estómago / Case Report: Subepithelial Tumors of the Stomach

INTRODUCCIÓN: Las lesiones subepiteliales son aquellas estructuras ubicadas debajo de una mucosa normal que se ven como bultos o protuberancias, en cualquier parte de la luz del tracto digestivo. Los estudios informan que estas protuberancias ocurren en 1 de cada 300 endoscopias [1] o en 1 % de las endoscopias digestivas altas, con una incidencia de 0.76 % en Ecuador [2]. Uno de ellos es el pólipo fibroide inflamatorio, representa el tumor benigno menos frecuente entre el 1 al 4 %, con una incidencia de 0.1 % [3] y otro es el GIST, el más común de los tumores gastrointestinales, principalmente en el estómago con frecuencia entre el 60 al 70 % [4].Los tumores subepiteliales se encuentran principalmente en el estómago con un 60 a 70 %, seguido de intestino delgado con un 20 a 30 %, luego colon con 5 % y en esófago en menos del 5 % [5]. En el estómago los tumores más comunes son los GIST, se originan de las células intersticiales de Cajal con un CD117 positivo que detecta un componente celular llamado c-kit en el 95 % de estas. En el video se ilustra las técnicas usadas en estos dos pacientes para obtener una muestra y orientar la conducta.

BACKGROUND: Subepithelial lesions are those structures located below a normal mucosa that are seen as lumps or bulges located in any part of the lumen of the digestive tract. Studies report that these bulges occur in 1 in 300 endoscopies [1] or in 1 % of upper digestive endoscopies or with an incidence of 0.76 % in Ecuador [2]. One of them is the inflammatory fibroid poly; it represents the less frequent benign tumor between 1 to 4 %, with an incidence of 0.1 % [3] and another is the GIST which represents the most common subepithelial lesions, with a frequency between 60 to 70 % in the stomach [4]. Subepithelial tumors are found mainly in the stomach with 60 to 70 %, followed by small intestine with 20 to 30 %, then colon with 5 % and in the esophagus in less than 5 % [5]. In the stomach, the most common tumors are GIST, they originate from the interstitial cells of Cajal with a positive CD-117 that detects a cellular component called c-kit in 95 % of these. The video illustrates the techniques used in these two patients to obtain a sample and guide behavior.

Duodenectomía parcial distal con conservación del páncreas por un tumor del estroma gastrointestinal / Distal partial duodenoscopy with pancreas conservation for stromal gastrointestinal tumor

Los tumores del estroma gastrointestinal son los tumores mesenquimatosos más frecuentes del sistema digestivo. En el duodeno son raros, con menos de 5 por ciento. A diferencia de los carcinomas, los tumores del estroma gastrointestinal no infiltran la mucosa de manera extensa. La resección quirúrgica con bordes de sección negativos sin linfadenectomía es el principal tratamiento con intención curativa y las resecciones conservadoras se llevan a cabo siempre y cuando sean factibles desde el punto de vista técnico. Presentamos una paciente con un tumor del estroma gastrointestinal de duodeno cuya principal manifestación fue el sangrado digestivo alto. La lesión fue resecada con bordes de sección quirúrgicos negativos mediante una duodenectomía parcial distal de la tercera y cuarta porciones del duodeno con preservación del páncreas. El tránsito intestinal fue restituido mediante una duodenoyeyunostomía término-terminal en un plano de sutura. En este momento, la paciente recibe tratamiento con metisilato de imatinib(AU)

Gastrointestinal stromal tumors are the most frequent mesenchymal tumors of the digestive system. In the duodenum, their presentation is rare, with less than 5 persent. Unlike carcinomas, gastrointestinal stromal tumors do not extensively infiltrate the mucosa. Surgical resection with negative section borders without lymphadenectomy is the main treatment with curative intent, and conservative resections are carried out as long as they are feasible from the technical point of view. We present the case of a patient with a gastrointestinal stromal tumor of the duodenum and whose main manifestation was high digestive bleeding. The lesion was resected with negative surgical section borders through a distal partial duodenectomy of the third and fourth portions of the duodenum and with preservation of the pancreas. The intestinal transit was restored by a terminal duodenojejunostomy in a suture plane. At this time, the patient is treated with imatinib mesylate(AU)

Tumores de estroma gastrointestinal / Gastrointestinal stromal tumors

Objetivos: Nos últimos anos foram desenvolvidos diversos trabalhos visando entender o comportamento biológico e definir a melhor forma de abordagem para os Tumores de Estroma Gastrointestinal. Nessa revisão, são organizadas informações atualizadas sobre o assunto. Métodos: Foi realizado levantamento bibliográfico, sem restrição de período, nas bases de dados PubMed e LILACS. Foi utilizada a palavra- -chave "Tumores de Estroma Gastrointestinal", que deveria aparecer no título dos artigos. Foram encontrados 1619 artigos e, depois da leitura dos resumos, foram selecionados 20 artigos publicados entre 1998 e 2018. Resultados: Foram levantados dados relevantes e atualizados sobre a epidemiologia, histopatologia, apresentação clínica, diagnóstico, prognóstico e tratamento dos Tumores de Estroma Gastrointestinal. Conclusões: Apesar de ser considerada uma neoplasia rara, é um assunto que deve ser estudado e discutido pela definição recente do diagnóstico e da abordagem terapêutica.

Objectives: In recent years, several studies have been developed to understand the biological behavior and to define the best therapeutic approach for Gastrointestinal Stromal Tumor. In this review, we have organized up-to-date information on the subject. Methods: A bibliographic survey was carried out, without period restriction, in PubMed and LILACS databases. The key word "Gastrointestinal Stromal Tumors" was used, which should appear in the title of the articles. A total of 1619 articles were found and, after reading the abstracts, 20 papers published between 1998 and 2018 were selected. Results: We collected relevant and updated data on the epidemiology, histopathology, clinical presentation, diagnosis, prognosis and treatment of Gastrointestinal Stromal Tumors. Conclusions: Despite being considered a rare neoplasm, it is a subject that must be studied and discussed because of the recent definition of diagnosis and the therapeutic approach.

Primary cutaneous amelanotic melanoma and gastrointestinal stromal tumor in synchronous evolution

Abstract: Gastrointestinal stromal tumor is rare digestive tract mesenchymal tumor, most often in the wall of the stomach. It is a benign neoplasm, but it can become malignant if not treated. We report a case of gastrointestinal stromal tumor that was discovered after abdominal ultrasonography during staging of a patient with primary cutaneous amelanotic melanoma. Mutation in the tyrosine kinase receptor could explain the development of two types of tumors in the same patient.

Tumores del estroma gastrointestinal: Análisis de 40 casos / Gastrointestinal stromal tumors. Analysis of 40 cases

Los tumores del estroma gastrointestinal representan menos del 3% de los tumores digestivos. Se localizan principalmente en el estómago y el intestino delgado. El tratamiento radical es la resección quirúrgica. Cuando son inoperables o diseminados la administración de imatinib es el tratamiento de elección. La finalidad de este estudio retrospectivo fue describir las características de los pacientes con tumores del estroma gastrointestinal atendidos en nuestra institución en el período 2000-2015. Fueron analizados los casos de 40 pacientes consecutivos con diagnóstico de tumor del estroma gastrointestinal (edad media 58 años, rango 33-84). La supervivencia media a 5 años del total de pacientes fue 30.5%. Al diagnóstico, 30 (75%) tenían enfermedad localizada; de estos, 14 recibieron imatinib adyuvante y 15 seguimiento en observación. En este grupo, el intervalo libre de enfermedad fue 55 meses. En aquellos con enfermedad diseminada, el intervalo libre de progresión fue 30 meses.

Gastrointestinal stromal tumors represent less than 3% of all digestive tumors. They are primarily located in the stomach and the small intestine. The curative treatment is surgical resection. In the case of unresectable tumor or advanced disease, imatinib is the treatment of choice. The purpose of this retrospective study was to describe the characteristics of patients with gastrointestinal stromal tumors treated at our institution in the period 2000-2015. We analyzed 40 consecutive patients diagnosed with gastrointestinal stromal tumor (mean age 58-year old, range 33-84). The mean 5-year survival was 30.5%. At diagnosis, 30 patients had localized disease (75%); 14 of them received adjuvant imatinib and 15 follow-up on observation. In this group the disease-free interval was 55 months. In patients with advanced disease, the progression-free interval was 30 months.

Tumor del estroma gastrointestinal y trasplante renal / Gastrointestinal stromal tumor and renal transplant

El tumor estromal gastrointestinal (GIST) representa alrededor del 1% de todos los tumores digestivos y su aparición en pacientes trasplantados renales es infrecuente. Aproximadamente el 95% muestra tinción positiva para c-kit/CD117. DOG1 es un anticuerpo recientemente descrito que se sobre-expresa en los GIST, incluso en c-kit/ CD117 negativos. El diagnóstico preciso de GIST resulta imperativo, debido a la disponibilidad y la creciente eficacia de los inhibidores de la tirosina quinasa en estos tumores, incluso en el subgrupo c-kit/ CD117 negativo. Se presenta el caso de una mujer trasplantada renal inicialmente con GIST en intestino delgado y débil positividad para CD117 tratada con cirugía y recidiva tumoral a los tres años, pérdida de la expresión CD117 y tinción positiva para DOG1. Recibió tratamiento exitoso con imatimib sin presentar recaída tumoral durante un seguimiento de cinco años.

Gastrointestinal stromal tumor (GIST) accounts for nearly 1% of all gastrointestinal tumors. Its association with renal transplantation is not frequent. Approximately 95% of GIST show staining for CD177. DOG1 is a recently described monoclonal antibody that shows positivity even in the absence of CD177 staining. The diagnosis of GIST should be pursued because of the availability of very effective treatments with tyrosine-kinase inhibitors. Herein, we describe the case of a woman with renal transplant who presented a small bowel GIST and weak positivity for CD177, treated initially with surgery. Tumor recurrence was documented 3 years later and histopatology showed loss of CD177 staining and positivity for DOG1. She was treated with imatimib without further recurrence after five years of follow up.

Gastrectomía atípica laparoscópica en el tratamiento de GIST gástrico: Resultados a corto y mediano plazo / Laparoscopic atypical gastrectomy in the treatment of gastric GIST. Short-term and medium-term outcomes

Los tumores gastrointestinales del estroma son las neoplasias mesenquimatosas más frecuentes del tracto gastrointestinal. Numerosas investigaciones se han dirigido al tratamiento laparoscópico de los mismos. El propósito de este estudio es evaluar la seguridad y la eficacia de las gastrectomías atípicas en un hospital de alta complejidad de nuestro medio. En el período comprendido entre enero 2009 y diciembre 2015, 24 pacientes con sospecha de GIST gástrico fueron intervenidos quirúrgicamente vía laparoscópica, considerándose criterio de inclusión la ausencia de metástasis hepáticas y peritoneales a distancia, el tamaño tumoral < 5 cm de diámetro y la positividad del antígeno CD117 en los estudios inmunohistoquímicos. Se analizaron retrospectivamente las características clínico-patológicas, los resultados quirúrgicos, las complicaciones postoperatorias y los resultados oncológicos. Fueron incluidos en el estudio estadístico 14 pacientes. La edad promedio fue 60 años (10 mujeres y 4 hombres) y la hemorragia digestiva alta fue la sintomatología de diagnóstico más frecuente. El tiempo promedio de cirugía fue 113 minutos, con necesidad de conversión en un caso. No se registraron complicaciones mayores, reintervenciones ni reinternaciones. El tamaño tumoral promedio fue 41 mm, con márgenes quirúrgicos libres de enfermedad. El promedio de internación fue 3 días y el de seguimiento 44 meses, sin registro de recidivas tumorales. El abordaje laparoscópico a través de gastrectomías atípicas del GIST gástrico resultó una técnica segura y eficaz con buenos resultados a corto y mediano plazo en manos de cirujanos con experiencia.

Gastrointestinal stromal tumors are the most common mesenchymal neoplasms of the gastrointestinal tract. Recently, many studies have addressed the laparoscopic management of these tumors. The aim of this study was to evaluate the safety and efficacy of laparoscopic atypical gastrectomy in the resolution of these neoplasms. In the period between January 2009 and December 2015, 24 patients with suspected gastric GIST less than 5 cm in size underwent surgery under this approach. Abscense of peritoneal and liver metastases and immunohistochemistry expression of CD117 antigen were considered as inclusion criteria. We retrospectively analyzed clinical and histopathological characteristics, surgical outcomes, postoperative complications and oncological results. Statistical analysis included 14 patients. The mean age was 60 years (10 women and 4 men) and upper gastrointestinal bleeding was the chief complaint for consultation. Conversion to open surgery was necessary in one case and no major complications, readmissions or reoperations were recorded. The mean tumor size was 41 mm with negative resection margin in all samples. The mean hospital stay was 3 days and no tumor recurrences were recorded in 44 months of follow-up. Laparoscopic atypical gastrectomy for gastric GIST is a safe and effective technique with good outcomes in experienced hands.

Tumor del estroma gastrointestinal en mujer gestante joven / Gastrointestinal stromal tumor in young pregnant woman: case report

Paciente mujer de 28 años de edad, con gestación de 14 semanas, natural y procedente de Ilo, Moquegua. Acudió con tiempo de enfermedad de 4 días con hematemesis, melenas, epigastralgia y síncope. Antecedente de resección colónica por peritonitis debido a un traumatismo hace 20 años; cuadro de hemorragia digestiva alta hace 3 años; abuelos paternos fallecidos de cáncer gástrico; gastritis crónica diagnosticada desde los 19 años de edad. Dentro de los estudios laboratoriales hemoglobina de 9,2. En el estudio de endoscopia digestiva alta se evidencia ausencia de hemorragia digestiva alta activa, una gran tumoración en cuerpo proximal hacia curvatura mayor y pared posterior, de aproximadamente 8 cm de diámetro, de aspecto lobulado, con la mayor parte de la superficie mucosa indemne, áreas de ulceración de 2 a 3 mm cubiertas de fibrina (de las cuales se toma biopsias), de consistencia dura, signo de la carpa (positivo) y signo del cojín (negativo). No se pudo realizar estudios de imagen como resonancia magnética o tomografía debido a la gestación. En la ecografía abdominal completa sólo se reportó esteatosis hepática de I grado. Los resultados de biopsia endoscópica indicaron proliferación celular de características epitelioides con atipia. Se realizó inmunohistoquímica indicando CD 117 (positivo), CD 34 (negativo), Ki 67 (positivo 1%). El diagnóstico final fue un tumor del estroma gastrointestinal de tipo epitelioide con tasa mitótica menor de 1%. Finalmente se realizó resección quirúrgica del tumor, en la que además se encontró 3 implantes de hasta 1 cm de diámetro en peritoneo que cubrían la superficie pancreática. Presentamos el caso por ser esta una inusual presentación de este tipo de tumor

Female patient, 28 years old, with a gestation of 14 weeks, natural and from Ilo-Moquegua. Came with a time of disease 4 days with hematemesis, melena, epigastric pain and syncope. Background of colonic resection for peritonitis due to trauma 20 years ago; upper gastrointestinal bleeding three years ago; paternal grandparents died of gastric cancer; chronic gastritis diagnosed from 19 year old. In laboratory studies hemoglobin of 9.2. In the upper gastrointestinal endoscopy was found absence of active upper gastrointestinal bleeding, a large tumor in proximal body toward greater curvature and posterior wall, 8 cm in diameter approximately, lobed appearance, with most of the mucosal surface unscathed is evidence, areas of ulceration of 2-3 mm covered with fibrin (biopsies taken), hard consistency, tent sign (positive) and cushionsign (negative). Imaging studies such as MRI or CT were not possible due to the pregnancy. In the complete abdominal ultrasound only grade I hepatic steatosis was reported. The results of endoscopic biopsy showed cell proliferation ofepithelioid characteristics with atypia. Immunohistochemistry was performed indicating CD117 (positive), CD 34 (negative), Ki 67 (positive 1%). The final diagnosis was a gastrointestinal stromal tumor epithelioid type with mitotic rate lower of 1%. Finally, surgical resection of the tumor was performed in which addition of 3 implants was found up to 1 cm in diameter in pancreatic peritoneum covering surface. We present the case as this is an unusual presentation of this tumor

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).