Tumor de GIST, presentación no habitual / GIST tumor: an unusual presentation

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

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

GIST gástrico complicado. Una forma de presentación inusual / Complicated gastric GIST. An unusual presentation

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

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

Condroma pulmonar y paraganglioma carotídeo como manifestaciones de la tríada de Carney: reporte de un caso / Pulmonary chondroma and carotid paraganglioma as expressions of Carney's triad: report of a case

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

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

Prognostic factors and management of colorectal gastrointestinal stromal tumors

Introduction: The gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. Even though it can be found in any location of the digestive tract, the colorectal GIST is rare. With this study, we aim to review the current knowledge regarding the prognosis and management of colorectal GIST. Methods: A literature search was conducted in PubMed, and 717 articles were collected. After analyzing these studies, 60 articles were selected to use in this review. Results: The mitotic index, as well as tumor size and location were identified as good discriminators of prognosis in various studies. Surgery remains the only curative therapy for potentially resectable tumors. However, even after surgical resection, some patients develop disease recurrence and metastasis, especially those with highrisk tumors. Therefore, surgical resection alone might be inadequate for the management of all colorectal GISTs. The discovery of GIST's molecular pathway led to a shift in its therapy, insofar as tyrosine kinase inhibitors became part of the treatment schemes for this tumor, revolutionizing the treatment's outcome and prognosis. Discussion/Conclusion: The controversy concerning colorectal GIST prognosis and treatment can be, in part, attributed to the limited number of studies in the literature. In this review, we gathered the most recent knowledge about the prognosis and management of GIST in this rare location and propose two algorithms for its approach. Lastly, we highlight the importance of an individualized approach in the setting of a multidisciplinary team. (AU)

Tumores del estroma gastrointestinal en un Hospital de México / Gastrointestinal stroma tumor in a hospital of Mexico

Introducción: Los tumores del estroma gastrointestinal son neoplasias de comportamiento benigno o maligno. Se originan de las células intersticiales de Cajal del tubo digestivo. Objetivo: Describir dos formas distintas de presentación clínica de los tumores del estroma gastrointestinal. Casos clínicos: El caso 1, paciente femenina de 65 años de edad que acudió por síntomas compresivos del tubo digestivo superior a causa de un gastrointestinal gástrico. El caso 2, paciente masculino de 56 años de edad que acudió por sangrado de tubo digestivo medio ocasionado por un gastrointestinal intestinal. Conclusiones: Los tumores del estroma gastrointestinal tienen distinta presentación clínica. Su tratamiento es esencialmente quirúrgico y en algunos casos complementados con terapia molecular dirigida(AU)

Introduction: Gastrointestinal stromal tumors are neoplasms of benign or malignant behavior. They originate from the interstitial cells of Cajal in the digestive tract. Objective: The objective of this work is to describe two different forms of clinical presentation. Case report: case 1: 65-year-old female patient who presented for compression symptoms of the upper digestive tract due to gastric GIST; case 2: 56-year-old male who presented with bleeding from the middle digestive tract caused by intestinal GIST. Conclusions: GISTs have different clinical presentation. Its treatment is essentially surgical and in some cases supplemented with targeted molecular therapy(AU)

Safety and feasibility of laparoscopic double-flap technique in digestive tract reconstruction after proximal gastrectomy for esophagogastric junction tumors larger than 5 cm / 中华胃肠外科杂志

Objective: To investigate the safety and feasibility of laparoscopic double-flap technique (Kamikawa) in digestive tract reconstruction after proximal gastrectomy for esophagogastric junction (EGJ) leiomyoma and gastrointestinal stromal tumor (GIST) with the maximum diameter >5 cm. Methods: A descriptive case-series study was used to retrospectively analyze the data of patients with EGJ leiomyoma and GIST undergoing laparoscopic-assisted proximal gastrectomy and double-flap technique (Kamikawa) at the Department of Gastrointestinal Surgery, Guangdong Hospital of Traditional Chinese Medicine from September 2017 to March 2019. All the tumors invaded the cardia dentate line, and the maximum diameter was >5 cm. After the exclusion of patients requiring emergency surgery and complicating with severe cardiopulmonary diseases, a total of 4 patients, including 3 males and 1 female with age of 29-49 years, were included in this study. After laparoscopic-assisted proximal gastrectomy, the residual stomach was pulled out of the abdominal cavity and marked with methylene blue at the proximal end 3~4 cm from the anterior wall of the residual stomach in the shape of "H". The gastric wall plasma muscular layer was cut along the "H" shape, and the space between the submucosa and the muscular layer was separated to both sides along the longitudinal incision line to make the seromuscular flap. The residual stomach was put back into the abdominal cavity. Under laparoscopy, 4 stitches were intermittently sutured at the upside of "H" shape and 4-5 cm from the posterior wall of the esophageal stump. The stump of the esophagus was cut open, and the submucosa and mucosa were cut under the "H" shape to enter the gastric cavity. The posterior wall of the esophageal stump was sutured continuously with the gastric stump mucosa and submucosa under laparoscopy. The anterior wall of the esophageal stump was sutured continuously with the whole layer of the residual stomach. The anterior wall of the stomach was sutured to cover the esophagus. The anterior gastric muscle flap was sutured and embedded in the esophagus to complete the reconstruction of digestive tract. The morbidity of intraoperative complications and postoperative reflux esophagitis and anastomosis-related complications were observed. Results: All the 4 patients completed the operation successfully, and there was no conversion to laparotomy. The median operative time was 239 (192-261) minutes, the median Kamikawa anastomosis time was 149 (102-163) minutes, and the median intraoperative blood loss was 35 (20-200) ml. The abdominal drainage tube and gastric tube were removed, and the fluid diet was resumed on the first day after surgery in all the 4 patients. The median postoperative hospitalization time was 6 (6-8) days. Postoperative pathology revealed 3 leiomyomas and 1 GIST. There were no postoperative complications such as anastomotic leakage or stenosis, and no reflux symptoms were observed. The median follow-up time was 22 (11-29) months after the operation, and no reflux esophagitis occurred in any of the 4 patients by gastroscopy. Conclusion: For >5 cm EGJ leiomyoma or GIST, double-flap technique (Kamikawa) used for digestive tract reconstruction after proximal gastrectomy is safe and feasible.

Endoscopic or laparoscopic resection for small gastrointestinal stromal tumors: a cumulative meta-analysis / 中华医学杂志(英文版)

BACKGROUND@#Despite the recent large number of studies comparing endoscopic and laparoscopic resection for small gastrointestinal stromal tumors (GISTs) (diameter ≤ 5 cm), the results remain conflicting. The objective of this work was to perform a cumulative meta-analysis to assess the advantages and disadvantages of endoscopic resection vs. laparoscopic resection.@*METHODS@#The meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. We searched medical databases up to January 2020. Meta-analytical random or fixed effects models were used in pooled analyses. Meta-regression, cumulative meta-analyses, and subgroup analyses were performed to improve the accuracy of the conclusion. Sensitivity analyses were applied to assess the robustness of the results.@*RESULTS@#A total of 12 cohort studies with 1383 participants comparing endoscopic resection and laparoscopic resection were identified, while three cohort studies with 167 participants comparing endoscopic resection and laparoscopic and endoscopic cooperative surgery were found. We found that endoscopic resection had shorter operation times (weighted mean difference [WMD] = -27.1 min, 95% confidence interval [CI]: -40.8 min to -13.4 min) and lengths of hospital stay (WMD = -1.43 d, 95% CI: -2.31 d to -0.56 d) than did laparoscopic resection. The results were stable and reliable. There were no significant differences in terms of blood loss, hospitalization costs, incidence of complications or recurrence rates. For tumor sizes 2 - 5 cm, endoscopic resection increased the risk of positive margins (relative risk [RR] = 5.78, 95% CI: 1.31 - 25.46). Although operation times for endoscopic resection were shorter than those of laparoscopic and endoscopic cooperative surgery (WMD = -41.03 min, 95% CI: -59.53 min to -22.54 min), there was a higher incidence of complications (RR = 4.03, 95% CI: 1.57 - 10.34).@*CONCLUSIONS@#In general, endoscopic resection is an alternative method for gastric GISTs ≤ 5 cm. Laparoscopic and endoscopic cooperative surgery may work well in combination. Further randomized controlled trials are recommended to validate or update these results.

Jejunal stromal tumor and neurofibromatosis / Tumor estromal de jejuno e neurofibromatose

Abstract Gastrointestinal stromal tumors, although rare, are the most common primary mesenchymal neoplasms of the gastrointestinal tract and originate from the interstitial cells of Cajal. They present slow growth and symptoms such as bleeding, abdominal pain or discomfort, and the presence of an abdominal mass. The most affected organs are the stomach and small intestine. Differential diagnoses for gastrointestinal stromal tumor include adenocarcinoma and small intestine lymphoma, metastasis, and carcinoid tumor. Gastrointestinal stromal tumors have been associated with familial syndromes such as type 1 neurofibromatosis, considered a predisposing factor for tumors in the small intestine. This study aimed to report a case of gastrointestinal stromal tumor in the jejunal region in a patient with type 1 neurofibromatosis, followed-up for two years, who underwent laparoscopic segmental enterectomy and diagnosis determined by histopathology and immunohistochemistry. The diagnosis of small intestine gastrointestinal stromal tumor is challenging because of its low incidence, nonspecific symptoms, relative inaccessibility of the small intestine to conventional endoscopic examination, broad spectrum of radiological appearances, and the fact that the nature of the mass is difficult to determine with imaging examinations of the abdomen alone. Thus, the small intestine gastrointestinal stromal tumor may be erroneously diagnosed as pancreatic, gynecological, or mesenteric tumors. The literature does not present many reports on the association of jejunal gastrointestinal stromal tumor with neurofibromatosis. Understanding the tumoral behavior of small intestine gastrointestinal stromal tumor in this subgroup of patients would allow better follow-up.

Resumo Os tumores estromais gastrointestinais, embora raros, são as neoplasias mesenquimais primárias mais comuns do trato gastrointestinal e originam-se das células intersticiais de Cajal. Apresentam crescimento lento e manifestam sintomas como sangramento, dor ou desconforto abdominal e presença de massa abdominal. Os órgãos mais acometidos são estômago e intestino delgado. Os diagnósticos diferenciais para tumores estromais gastrointestinais incluem adenocarcinoma e linfoma de intestino delgado, metástases e tumor carcinoide. Os tumores estromais gastrointestinais têm sido associados a síndromes familiares como a neurofibromatose tipo 1, considerada um fator predisponente para tumores no intestino delgado. O objetivo desse trabalho é relatar um caso de tumor estromal gastrointestinal em região jejunal em paciente portadora de neurofibromatose tipo 1, com 2 anos de seguimento, submetida a enterectomia segmentar laparoscópica e diagnóstico determinados pela histopatologia e imuno-histoquímica. O diagnóstico de tumor estromal gastrointestinal do intestino delgado é desafiador, devido a sua baixa incidência, sintomas inespecíficos, relativa inacessibilidade do intestino delgado ao exame endoscópico convencional, amplo espectro de aparências radiológicas e difícil determinação da natureza da massa apenas com exames de imagens do abdome. Assim, tumor estromal gastrointestinal no intestino delgado podem ser erroneamente diagnosticados como tumores pancreáticos, tumores ginecológicos, ou tumores do mesentério. A descrição científica da associação de tumor estromal gastrointestinal de jejuno com neurofibromatose é incomum. Tais descrições permitem melhor seguimento dos pacientes a partir do momento que se entende o comportamento tumoral do tumor estromal gastrointestinal de intestino delgado nesse subgrupo de pacientes.

Pre-operative gastric gist downsizing: the importance of neoadjuvant therapy / Redução tumoral pré-operatória do gist gástrico: a importância da terapia neoadjuvante

ABSTRACT Introduction: Gastric gastrointestinal tumors (GIST) are a rare and usually asymptomatic neoplasm that can present as abdominal mass in more advanced scenarios. Since surgical resection is the main aspect of the treatment, locally advanced tumors require multivisceral resection and, therefore, higher postoperative morbidity and mortality. Objective: To perform a review the literature on the topic, with emphasis on the neoadjuvant therapy. Methods: Literature review on the Medline database using the following descriptors: gastrointestinal stromal tumors, neoadjuvant therapy, imatinib mesylate and molecular targeted therapy. Results: Surgical resection remains the cornerstone for the treatment of GISTs; however, tyrosine kinase inhibitors have improved survival as an adjuvant therapy. More recently, neoadjuvant therapy have been described in the treatment of locally advanced tumors in order to avoid multivisceral resection. Conclusion: Despite surgical resection remains as the most important aspect of the treatment of GISTs, adjuvant and neoadjuvant therapy with tyrosine kinase inhibitors have shown to both improve survival and resectability, respectively.

RESUMO Introdução: O tumor estromal gastrintestinal (GIST) gástrico é neoplasia que cursa, por vezes, com apresentação assintomática, se manifestando como massa abdominal, relacionada a casos mais avançados. Esses, por sua vez, exigem tratamento com operações maiores e que cursam com mais alta morbimortalidade. Objetivo: Revisar e atualizar o tratamento do GIST gástrico, com enfoque na relevância do tratamento neoadjuvante. Método: Revisão da literatura utilizando a base de dados Medline/PubMed. Utilizaram-se os seguintes descritores: gastrointestinal stromal tumors, neoadjuvant therapy, imatinib mesylate e molecular targeted therapy. Dos artigos selecionados, 20 foram incluídos. Resultados: O tratamento cirúrgico é pilar fundamental para a cura do GIST. Entretanto, após a introdução do mesilato de imatinibe, classicamente utilizado como terapia adjuvante, houve mudança no manejo do GIST, permitindo aumento da sobrevida e diminuição da recorrência. A aplicação como terapia neoadjuvante é mais recente, e visa evitar procedimentos maiores sem, no entanto, prejudicar o resultado oncológico. Conclusão: A ressecção cirúrgica possui papel bem estabelecido no tratamento do GIST, inclusive com abordagem laparoscópica. O tratamento adjuvante com mesilato de imatinib, durante os primeiros três anos após a operação, mostra-se como opção segura para casos com elevado risco de recidiva. A terapia neoadjuvante é opção promissora para casos de tumor localmente avançado, permitindo ressecções menores e com menor morbimortalidade operatória.

Laparoscopic resection of gastrointestinal stromal tumors (GIST) of the small intestine: a case report

Although gastrointestinal stromal tumors (GISTs) are a rare type of cancer, they are the commonest mesenchymal tumors of the gastrointestinal tract (GIT). GISTs can affect any segment of the GIT, but the usual location is the stomach, followed by the small intestine. Surgical resection of the tumor is the gold standard treatment for localized GISTs, and in patients with inoperable and metastatic disease, imatinib mesylate is the standard treatment. Pathological diagnosis is based on morphology and immunohistochemical findings. We report the case of a 55-year-old man with jejunal GIST presenting with endophytic and exophytic growth, located in the proximal jejunum. He had history of melena, anemia and one episode of enterorrhagia, and was treated with surgical resection of the lesion. (AU)

Endoscopic full-thickness resection for gastric gastrointestinal stromal tumor originating from the muscularis propria

SUMMARY OBJECTIVE: This study retrospectively reviewed 46 cases of gastric gastrointestinal stromal tumors treated by endoluminal endoscopic full-thickness resection (EFR) microsurgery in our gastrointestinal endoscopy center. We aimed to evaluate the EFR for the treatment of gastric gastrointestinal stromal tumors originating from the muscularis propria. METHODS: A total of 46 patients with gastric gastrointestinal stromal tumors originated from the muscularis propria layer from January 2012 to June 2015 were treated with EFR. The patients were followed up with gastroscope and computed tomography (CT) for evaluation of therapeutic effect and safety. RESULTS: EFR was successfully accomplished to remove all tumors in 46 patients. The mean procedure time was 82.5±39.8min (56-188min). Except in 3 leiomyomas, pathological examination confirmed gastrointestinal stromal tumor (GIST) in 43 cases. None of the patients had occurred bleeding, peritonitis and other complications after EFR. Thereafter, all patients were followed up with gastro-scope after 1, 6,12 months. CONCLUSIONS: EFR is effective and safe for patients with gastric gastrointestinal stromal tumors originated from muscularis propria layer and has the advantage of less invasive treatment and higher tumor resection rate. It should be considered for further application.

RESUMO OBJETIVO: Este estudo revisou retrospectivamente 46 casos de tumores gástricos estromáticos gastrointestinais tratados por microcirurgia endoluminal endoscópica de ressecção completa (EFR) em nosso centro de endoscopia gastrointestinal. Pretendemos avaliar a EFR para o tratamento de tumores gastrointestinais estromáticos originários da muscularis própria. MÉTODOS: Um total de 46 pacientes com tumores gástricos estromáticos gastrointestinais originários da camada muscular própria, de janeiro de 2012 a junho de 2015, foi tratado com EFR. Os pacientes foram acompanhados com gastroscópio e tomografia computadorizada (TC) para avaliação de efeitos terapêuticos e segurança. RESULTADOS: A EFR foi realizada com sucesso para remover todos os tumores em 46 pacientes. O tempo médio de procedimento foi de 82,5±39,8 min (56-188 min). Exceto em três leiomiomas, exame patológico confirmou tumor estromal gastrointestinal (Gist) em 43 casos. Em nenhum paciente ocorreu sangramento, peritonite e outras complicações após EFR. Posteriormente, todos os pacientes foram acompanhados com gastroscópio após um, seis e 12 meses. CONCLUSÕES: A EFR é eficaz e segura para pacientes com tumores gastrointestinais originários da camada muscular própria e tem a vantagem de ser um tratamento menos invasivo e com maior taxa de ressecção tumoral. Deve ser considerada para posterior aplicação.

Modificación del abordaje quirúrgico en tumores estromales gástricos posterior a neoadyuvancia con Imatinib / Change of surgical approach in gastrointestinal stromal tumors after neoadyuvant therapy with Imatinib

Resumen Introducción: El tratamiento de los tumores estromales gastrointestinales (GIST) de alto riesgo es quirúrgico. Su resultado podría variar al usarse neoadyuvancia. Objetivo: Evaluar si el uso de la terapia neoadyuvante con imatinib puede cambiar el abordaje quirúrgico en los tumores estromales gastrointestinales de alto riesgo. Materiales y Métodos: Se realizó un análisis retrospectivo en el Hospital Clínic de Barcelona entre enero de 2002 y mayo de 2016. Resultados: Se obtuvo un total de 8 pacientes. La edad media fue 66,1 ± 13,3 años. La ubicación del tumor fue de 37,5% (3) en el tercio superior, el 50% (4) en el tercio medio y el 12,5% (1) en el tercio inferior. Debido a la clasificación de riesgo alto, la ubicación y/o la necesidad de resecciones multiviscerales, se indicó, previa evaluación comité oncológico, realizar terapia neoadyuvante. La mediana de tiempo de neoadyuvancia fue de 30 semanas. En el 100% (8) de los casos se logró un cambio de enfoque quirúrgico después de la utilización de imatinib. En todos los casos se realizó un resección local (7 laparoscópica y 1 endolaparoscópica) con márgenes negativos La biopsia posoperatoria mostró un promedio de 51,2% de reducción del tamaño tumoral inicial, lo que resultó en una diferencia estadística (p < 0,01) con el tamaño inicial de las lesiones. Durante el seguimiento, tanto la sobrevida relacionada al tumor como la global, fue de un 100%. Conclusión: La terapia neoadyuvante podría cambiar el abordaje quirúrgico de los pacientes con GIST gástrico de riesgo intermedio o alto mediante la reducción del tamaño tumoral, permitiendo realizar cirugías más económicas y logrando resultados oncológicos adecuados.

Introduction: The treatment of high-risk gastrointestinal stromal tumors (GIST) is surgical. Results may change when using neoadjuvant. Objetive: To evaluated if the use of neoadjuvant therapy with imatinib can change the surgical approach in high risk gastrointestinal stromal tumors (GIST). Materials and Methods: A retrospective analysis was performed from a prospective collected database in Hospital Clinic of Barcelona between January 2002 and May 2016. Results: A total of 8 patients were analyzed with a mean age of 66.1 ± 13.3 years. The tumor location was upper third 37.5% (3) cases, 50% (4) in the middle third and 12.5% (1) in lower third. Because of high risk classification, location and the need of multivisceral resections, neoadjuvant therapy was indicated. The median time of neoadjuvant therapy was 30 weeks. In 87.5% (7) cases a change of surgical approach was achieved after the use of imatinib. In 100% of our series laparoscopic wedge resection was performed achieving negative margins of resection. The postoperative biopsy showed 51.2% of reduction of initial tumor size, resulting in statistical difference (p < 0.01). All patients are alive and 100% of tumor related survival was achieved. Conclusion: Neoadjuvant therapy maybe can change the surgical approach of patients with high-intermediate risk gastric GIST by reducing tumor size. This response also eventually can achieve optimal oncological outcome.

Adenocarcinoma gástrico y tumor estromal del estómago sincrónico incidental / Gastric adenocarcinoma and incidental synchronous incidental gastrointestinal stromal tumor

Se presenta el caso de un paciente masculino de 73 años de edad operado por adenocarcinoma del antro gástrico. Se practicó gastrectomía subtotal distal más linfadenectomía D2. Durante el examen anatomopatológico de la pieza quirúrgica se encontró otro tumor pequeño en el espesor de la pared del antro cubierto por mucosa normal, separado del adenocarcinoma. El análisis histológico confirmó otra neoplasia incidental sincrónica, un tumor del estroma gastrointestinal de tipo fusiforme, positivo para el marcador tumoral CD117. El objetivo del trabajo es presentar esta asociación tumoral sincrónica, revisar sus características y las teorías etiopatogénicas actuales de esta condición sincrónica según la literatura especializada(AU)

A case is presented of a 73-year-old male patient who was operated for a gastric adenocarcinoma of the antrum. A distal subtotal gastrectomy plus D2 lymphadenectomy were practiced. During the anatomic and pathologic examination of the surgical piece, a second tiny tumor was found in the wall of the antrum and that was covered with a normal mucosa separated from the adenocarcinoma. The histologic examination confirmed another synchronous incidental neoplasm, a spindle-cell gastrointestinal stromal tumor, positive for the tumor marker CD117. This paper is aimed at presenting synchronous tumor entity, to review its characteristics and the current etiologic and pathogenic theories of this synchronic condition according to the specialized literature(AU)

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.

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 de estroma gastrointestinal en intestino delgado / Gastrointestinal stromal tumor in the small intestine

Aunque comprenden menos de 1 por ciento de todos los tumores gastrointestinales, los tumores de estroma gastrointestinal son los tumores mesenquimatosos más comunes del aparato digestivo. Son tumoraciones heterogéneas que varían en tamaño, morfología y conducta biológica. Se comportan virtualmente como tumores benignos hasta cánceres muy agresivos, muchas veces metastásicos. El tumor de estroma gastrointestinal puede producirse en cualquier punto del tracto gastrointestinal desde el esófago hasta el ano. Se presenta un caso de un hombre de 49 años que acude a consulta por molestias abdominales. Se realizan estudios que impresionan paquete ganglionar. En la videolaparoscopía se evidenció una tumoración a nivel de yeyuno que se reseca y extrae. El resultado de la biopsia informa un tumor fusocelular del estroma gastrointestinal tipo GIST de la pared del intestino delgado con diferenciación leiomatosa (leiomioma) de bajo grado, de posible comportamiento agresivo(AU)

Although they represent less than one percent of all the gastrointestinal tumors, the stromal tumors are the most common mesenchymal tumors in the digestive system. They are heterogeneous tumors that vary in size, morphology and biological behavior. Their virtual behavior ranges from benign tumors to very aggressive cancers, often metastatic. Gastrointestinal stromal tumor may appear in any point of the gastrointestinal tract from the esophagus to the anus. Here is a 49 years-old man who went to the doctor´s because of abdominal pains. The studies showed ganglionic package. Video-laparoscopy showed tumor at the jejunum that was excised and removed. The result of the biopsy revealed a fusocellular tumor of the gastrointestinal stroma of GIST type in the small intestine wall with low grade differentiation (leiomyoma) of possible aggressive behavior(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).

Tumores del estroma gastrointestinal / Gastrointestinal stromal tumors

Antecedentes: Los tumores del estroma gastrointestinal (TEG) son los tumores mesenquimales más comunes del tracto gastrointestinal (TGI), se considera que surgen de las células de Cajal, ocurren principalmente en adultos mayores (60-65 años) y se localizan en estómago (50%-70%), intestino delgado (25%-35%), colon-recto (5%-10%) y esófago (<5%). La mayoría se presenta de manera esporádica y hasta el 70% son clínicamente sintomáticos. El diagnóstico definitivo se realiza en el estudio anatomopatológico. El pronóstico de estos tumores se determina por el tamaño, recuento mitótico y localización del tumor, clasificandose: riesgo muy bajo, riesgo bajo, riesgo intermedio y riesgo alto. La cirugía es la opción terapéutica principal...

Background: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract (GI), areconsidered to arise from the Cajal cells. Theyoccur mainly in older adults, 60-65 years. Theypresent in the stomach (50%-70%), small intestine (25%-35%), colon and rectum (5%-10%)and esophagus (<5%). Most GISTs are sporadicand are clinically symptomatic. The definitivediagnosis is made through anatomic pathology study. To determine the prognosis of this type of tumors we use the size, mitotic count and location of the tumor, classified them in: very low risk, low risk, intermediate risk and high risk. Surgery is the main treatment...

Intususcepción gastroduodenal como presentación de un tumor del estroma gastrointestinal gástrico / Gastroduodenal intussusception as a presentation of gastric gastrointestinal stromal tumor

Objetivo Presentar un caso de GIST con presentación clínica poco usual. Caso clínico Presentamos una paciente de género femenino, de 70 años, con 5 días de dolor en hemiabdomen superior y vómitos; se le diagnóstica por TC de abdomen y pelvis con contraste una intususcepción gastroduodenal. Se ingresa a pabellón; confirmando diagnóstico, se realiza gastrectomía subtotal más Y de Roux. La biopsia diferida con inmunohistoquímica confirma un GIST gástrico con 2 mitosis cada 50 campos. Se decide manejo conservador y control anual con endoscopia digestiva alta. Buena evolución.

Aim To present a case of GIST with unusual clinical presentation. Case report We present a 70-year old female patient with 5 days of upper abdominal pain and vomiting, being diagnosed with a gastroduodenal intussusceptions by contrasted CT scan. She was admitted to the operating room, confirming the diagnosis and a subtotal gastrectomy with Roux-en-Y reconstruction was performed. A deferred biopsy with immunohistochemical confirmed GIST with 2 mitoses/50 high-powerfields. Conservative management was proposed and annual control with upper endoscopy decided. Satisfactory evolution.