[Management of gastrointestinal stromal tumors]. / Prise en charge des tumeurs stromales gastro-intestinales.

MANAGEMENT OF GASTROINTESTINAL STROMAL TUMORS. Gastrointestinal stromal tumors (GIST) are the most frequent sarcoma subtype. More than 80% of GIST are characterized by activating mutations in KIT or PDGFRA genes, but rare molecular subtypes exist. Localized GIST can be cured by surgery. Adjuvant treatment with imatinib is the gold standard in high-risk GIST presenting mutations sensitive to this tyrosine kinase inhibitor. The development of tyrosine kinase inhibitors targeting KIT and PDGFRA has revolutionized the prognosis of metastatic GIST, by increasing the median overall survival: from less than 18 months to more than 70 months within 20 years. Similary to other histological subtypes, the diagnostic and therapeutic management of GIST must be referred to sarcoma reference centers.

PRISE EN CHARGE DES TUMEURS STROMALES GASTRO-INTESTINALES. Les tumeurs stromales gastro-intestinales (GIST) représentent le sous-type de sarcomes le plus fréquent. Plus de 80 % des GIST sont caractérisées par des mutations activatrices des gènes KIT ou PDGFRA, mais des soustypes moléculaires plus rares existent. Au stade localisé, les GIST sont des maladies curables par exérèse chirurgicale. Le traitement adjuvant par imatinib est un standard thérapeutique dans les GIST associées à un haut risque de récidive et présentant des mutations sensibles au traitement. Au stade métastatique, le développement des inhibiteurs de tyrosine kinase ciblant KIT et PDGFRA a bouleversé la prise en charge et le pronostic des patients, en augmentant la survie globale : de moins de dix-huit mois il y a une vingtaine d'années à plus de soixante-dix mois aujourd'hui. Comme pour les autres sous-types histologiques, la prise en charge diagnostique et thérapeutique des GIST doit être réalisée dans des centres experts pour la prise en charge des sarcomes.

Discontinuation of imatinib in patients with oligometastatic gastrointestinal stromal tumour who are in complete radiological remission: a prospective multicentre phase II study.

INTRODUCTION: Metastatic gastrointestinal stromal tumour (GIST) is considered incurable, and life-long treatment with tyrosine kinase inhibitors is recommended. We investigated whether selected patients with metastatic GIST may remain in durable remission despite imatinib discontinuation. PATIENTS: In this 1-group, prospective, multicentre phase II trial selected patients with oligometastatic (≤3 metastases) GIST discontinued imatinib treatment. Eligible patients had been treated with imatinib >5 years without progression and had no radiologically detectable metastases after metastasectomy, radiofrequency ablation (RFA) or complete response to imatinib. The primary endpoint was progression-free survival (PFS) 3-years after stopping imatinib. Overall survival (OS) and quality of life (QoL) were secondary endpoints. RESULTS: The trial closed prematurely due to slow accrual. Between January 5, 2017, and June 5, 2019, 13 patients were enrolled, of whom 12 discontinued imatinib. The median follow-up time was 55 months (range, 36 to 69) after study entry. Five (42%) of the 12 eligible patients remained progression free, and seven (58%) progressed with a median time to progression 10 months. Median PFS was 23 months and the estimated 3-year PFS 41%. Six of the seven patients who progressed restarted imatinib, and all six responded. Three-year OS was 100%, and all patients were alive at the time of the study analysis. QoL measured 5 and 11 months after discontinuation of imatinib demonstrated improvement compared to the baseline. INTERPRETATION: A substantial proportion of selected patients with oligometastatic GIST treated with imatinib and metastasis surgery/RFA may remain disease-free for ≥3 years with improved QoL after stopping of imatinib.

[Gastrointestinal stromal tumors : Where do we stand?] / Gastrointestinale Stromatumoren : Wo stehen wir?

For more than 20 years gastrointestinal stromal tumors (GIST) have been a paradigm for a targeted treatment with tyrosine kinase inhibitors. A fundamental prerequisite for a neoadjuvant or adjuvant treatment of localized GIST or an additive treatment of metastatic GIST is the molecular typing of tumors, ideally at the initial diagnosis. In addition, the possibility of a hereditary or syndromic predisposition must be considered because this results in consequences for the treatment and a different follow-up strategy.

English version of Japanese Clinical Practice Guidelines 2022 for gastrointestinal stromal tumor (GIST) issued by the Japan Society of Clinical Oncology.

The Japan Society of Clinical Oncology Clinical Practice Guidelines 2022 for gastrointestinal stromal tumor (GIST) have been published in accordance with the Minds Manual for Guideline Development 2014 and 2017. A specialized team independent of the working group for the revision performed a systematic review. Since GIST is a rare type of tumor, clinical evidence is not sufficient to answer several clinical and background questions. Thus, in these guidelines, we considered that consensus among the experts who manage GIST, the balance between benefits and harms, patients' wishes, medical economic perspective, etc. are important considerations in addition to the evidence. Although guidelines for the treatment of GIST have also been published by the National Comprehensive Cancer Network (NCCN) and the European Society for Medical Oncology (ESMO), there are some differences between the treatments proposed in those guidelines and the treatments in the present guidelines because of the differences in health insurance systems among countries.

The long-term efficacy of imatinib with hepatic resection or other local treatment for gastrointestinal stromal tumours liver metastases: a retrospective cohort study.

BACKGROUND: The liver is the most common site of metastasis from gastrointestinal stromal tumors (GISTs). The authors aimed to evaluate imatinib (IM) combined with hepatic resection (HR) or other local treatments such as radiofrequency ablation (RFA) and transarterial chemoembolization (TACE), compared to IM monotherapy in long-term survival benefits in patients suffering from GIST liver metastases. METHODS: Our research encompassed 238 patients diagnosed with liver metastases of GISTs from January 2002 to April 2022 at the First Affiliated Hospital of Sun Yat-Sen University. The oncological outcomes of concern included overall survival (OS), progression-free survival (PFS), and liver-specific PFS. RESULTS: Of all 238 patients, 126 were treated with IM alone (IM group), 81 with IM combined with HR (IM+HR group), and 31 with IM combined with RFA/TACE (IM+RFA/TACE group). The median follow-up time was 44.83 months. The median OS in the IM group was 132.60 months and was not reached in either the IM+HR group or the IM+RFA/TACE group. The 10-year OS rate in the IM+HR group was significantly superior to the IM group and the IM+RFA/TACE group (91.9% vs. 61.1% vs. 55.2%, respectively, P =0.015), and the liver-specific PFS ( P =0.642) and PFS ( P =0.369) in the three groups showed a beneficial trend in the combined treatment group. Multivariate analyses showed that age less than or equal to 60 years (HR 0.280, P< 0.001) and IM+HR (HR 0.361, P =0.047) were independently associated with better OS. Achieving no evidence of disease through surgical intervention was independently correlated with enhanced OS (HR 0.099, P =0.034), liver-specific PFS (HR 0.388, P =0.014), and PFS (HR 0.402, P =0.004). CONCLUSIONS: In patients with GIST liver metastases, IM combined with HR might improve OS in selected patients compared with IM alone and IM combined with RFA/TACE. Achieving no evidence of disease status with surgical treatment of patients results in significant prolonging of OS, liver-specific PFS, and PFS.

Referral patterns of GIST patients: data from a nationwide study.

BACKGROUND: This study compares the characteristics, referral and treatment patterns and overall survival (OS) of gastrointestinal stromal tumor (GIST) patients treated in reference and non-reference centers in the Netherlands. PATIENTS AND METHODS: This retrospective cohort study on patients diagnosed between 2016 and 2019, utilises data from the Netherlands Cancer Registry and the Dutch Nationwide Pathology Database. Patients were categorized into two groups: patients diagnosed in or referred to reference centers and patients diagnosed in non-reference centers without referral. RESULTS: This study included 1,550 GIST patients with a median age of 67.0 in reference and 68.0 years in non-reference centers. Eighty-seven per cent of patients were diagnosed in non-reference centers, of which 36.5% (493/1,352) were referred to a reference center. Referral rates were higher for high-risk (62.2% [74/119]) and metastatic patients (67.2% [90/134]). Mutation analysis was performed in 96.9% and 87.6% of these cases in reference and in non-reference centers (p < 0.01), respectively. Systemic therapy was given in reference centers versus non-reference in 89.5% versus 82.0% (p < 0.01) of high-risk and in 94.1% versus 65.9% (p < 0.01) of metastatic patients, respectively. The proportion of positive resection margins and tumor rupture did not differ between reference and non-reference centers. Median OS was not reached. CONCLUSION: A substantial amount of metastatic GIST patients in non-reference centers did not receive systemic treatment. This might be due to valid reasons. However, optimisation of the referral strategy of GIST patients in the Netherlands could benefit patients. Further research is needed to explore reasons for not starting systemic treatment in metastatic GIST patients.

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)

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.

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.

Gastrointestinal stromal tumors treated at a Brazilian Cancer Center: evaluation of clinical, epidemiological, and therapeutic profiles

Background: Gastrointestinal stromal tumors (GISTs) originate as precursor cells of the interstitial cells of Cajal in the myenteric plexus and generally have a mutation in the tyrosine kinase receptor, C-KIT (CD117). The objective is to evaluate the clinical, epidemiological, and therapeutic profiles of GIST cases available from a hospital specializing in cancer treatment. Methods: A retrospective, longitudinal study of 85 GIST cases in a Cancer Center (São Paulo, Brazil) was conducted. Results: The cases identified involved 40 men and 45 women and the average age at diagnosis was 55.7 ± 14. 8 years (median, 57). The symptoms present at diagnosis depended on the location and dimensions of each lesion. In 49 cases (57.6%), the tumors had a gastric location and the mean dimensions were 7.2 ± 2.3 cm (median, 3.4 cm). Recurrent metastatic disease presented in 27 cases (with the liver mainly affected). Locoregional recurrence was detected in 16 cases. C-KIT was positive in 79/81 (97.5%) of the cases examined. Most of the tumors were initially treated with surgery, while clinical treatment was applied to the recurrent cases. The overall survival rate was 76.4% 162 months after diagnosis. Conclusions: The GISTs examined most commonly originated in the stomach, while the liver was the main site affected by metastatic lesions. Most of the lesions appeared to be slow-growing neoplasms that were positive for C-KIT (CD117). Complications, as well as death, mostly affected the elderly patients that had comorbidities or more aggressive forms of the disease (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).

Profile of patients with gastrointestinal stromal tumors (GIST) / Perfil de pacientes portadores de tumores estromais gastrointestinais (GIST)

BACKGROUND: There is an improvement on the GIST treatment in last decade due to biomolecular research and adjuvant therapy with tyrosine kinases inibitors. However, both modalities of treatment rarely are available in Brazilian public hospital. AIM: Evaluate GIST patients profile in public oncologic hospital. METHODS: A retrospective study was made on patients with GIST diagnosed and treated between 2001 and 2013. RESULTS: Sixty-nine patients were included, mean age 59 years with slight predominance in females (51%). The main symptom was abdominal pain associated with incidental imaging finding. The occurrence of other associated neoplasm was in 28.8% of cases. The positivity of CD117 was 97.1%. The most frequent location was the stomach in 55.1% of cases. The R0 resection was possible in 63.8% and the recurrence rate was 20.3 %, with liver and peritoneum the main affected sites. Overall survival in the whole sample was 71%. Free survival rate of disease was 64%. The use of imatinib was limited to patients with residual disease (unresectable disease, R2 and R1 resection), metastatic disease or recurrence. CONCLUSION: In order to improve GIST treatment is necessary to add the biomolecular analysis to risk stratification. However, for this to occur, incentive in biomolecular research is required, to increase the possibility of patient survival. .

RACIONAL: O tratamento do GIST tem se aprimorado muito na última década através das pesquisas biomoleculares e o uso adjuvante dos inibidores das tirosinas quinases. Entretanto, nos hospitais públicos brasileiros nem sempre são disponíveis tais ferramentas. OBJETIVO: Avaliar o perfil dos pacientes portadores de GIST em hospital público oncológico. MÉTODOS: Análise retrospectiva de todos os casos de GIST tratados no período de 2001 a 2013. RESULTADOS: Analisaram-se 69 pacientes, com média de idade de 59 anos e com discreto predomínio no sexo feminino (51%). A principal forma de apresentação clínica foi dor abdominal associada com achado de exame de imagem. A ocorrência de outra neoplasia associada foi de 28,8%. A positividade do CD117 foi de 97,1%. A localização mais frequente foi o estômago em 55,1%. A ressecção R0 foi possível em 63,8% dos casos e a taxa de recidiva foi de 20,3%, sendo fígado e peritôneo os sítios principais acometidos. A sobrevida global na amostra toda foi de 71%. A taxa de sobrevida livre de doença foi de 64%. A utilização do imatinibe ficou restrita aos pacientes com doença residual (ressecção R2, R1 ou metastáticos), irressecáveis ou com recidiva. CONCLUSÃO: Afim de aprimorar o tratamento do GIST é necessário acrescentar a análise biomolecular à estratificação de risco. Porém, para que isto ocorra, políticas de incentivo e fomento na pesquisa biomolecular são necessárias, ampliando a possibilidade de sobrevida dos pacientes. .

Tumores del estroma gastrointestinal (GIST) del duodeno: presentación clínica, estudio diagnóstico y tratamiento actual / Duodenal gastrointestinal stromal tumors (GIST): Clinical presentation, diagnostic studies and current treatment

Duodenal gastrointestinal stromal tumors (GIST) constitute the most challenging location for the treatment of this neoplasm. Duodenal GIST are relatively uncommon tumors, their prevalence is very low accounting for 5 percent to 7 percent or less of all surgically resected GIST. Most published reports on duodenal GIST are case reports or case series. Consequently, the clinical manifestations, radiologic diagnosis, appropriate surgical treatment, and prognostic factors constitute a subject of current controversy. Most articles concerning duodenal GISTs state that unlike tumors involving other sites of the gastrointestinal tract, the optimal procedure for duodenal GISTs has not been well characterized. However, when carefully reviewing the published literature on the subject, it was found that surgical approaches to duodenal GISTs are fairly standard among different authors. All take into account the location of GIST in the duodenum and its anatomic relationships to decide whether local resection or Whipple operation should be performed. Using this common sense knowledge, defined surgical options for duodenal GISTs according to their localization within the duodenal frame are proposed.

Los tumores del estroma gastrointestinal (GIST) localizados en el duodeno, constituyen la localización más compleja para el tratamiento de esta neoplasia. Los GIST duodenales son relativamente infrecuentes con una prevalencia de 5 por ciento a 7 por ciento de todos los GIST tratados quirúrgicamente. La mayoría de los reportes publicados sobre GIST duodenal son reportes de caso o series de casos. Consecuentemente, las manifestaciones clínicas, el diagnóstico radiológico, el tratamiento quirúrgico y los factores pronósticos constituyen materia de controversia. La mayoría de los artículos sobre GIST duodenales mencionan que a diferencia de otros tumores localizados en el tracto gastrointestinal, el procedimiento óptimo para el tratamiento del GIST duodenal no se encuentra bien caracterizado en la literatura. Sin embargo, la revisión de las publicaciones sobre el tema demuestra que el abordaje quirúrgico descrito por diferentes autores es bastante estándar. Todos toman en cuenta la localización del GIST en el duodeno y sus relaciones anatómicas para decidir entre la resección local o la pancreatoduodenectomía. Utilizando este conocimiento de sentido común, se proponen opciones quirúrgicas definidas para GIST duodenales basadas en su localización en el duodeno.

Tumores estromales gastrointestinales: evaluación clinicopatológica y sobrevida en el Hospital Rebagliati / Gastrointestinal stromal tumors: clinicopathologic and survival evaluation in Rebagliati Hospital

OBJETIVO: Determinar las manifestaciones clínicas, radiológicas, histopatológicas y sobrevida de los pacientes con Tumor Estromal Gastrointestinal (GIST) en el Hospital Nacional Edgardo Rebagliati Martins (HNERM), de Lima, Perú. MATERIAL Y MÉTODOS: El presente es un estudio descriptivo, retrospectivo. El cual se realizó a partir de las historias clínicas de 103 pacientes con GIST confirmado por inmunohistoquímica que fueron evaluados y tratados en el HNERM, desde Enero del 2002 hasta Diciembre de 2010. RESULTADOS: En los 103 pacientes el promedio de edad fue 64 años (entre 30 y 88 años). Predominó en mujeres (52%). El tiempo de enfermedad promedio fue 7 meses. La forma de presentación más frecuente fue sangrado digestivo (48.3%). El diagnóstico se hizo más por endoscopía (50.5%). La prevalencia por órganos fue más frecuente en estómago 56.3%. El promedio de tamaño fue 98mm, (49% entre 50mm y 100mm), el tumor de mayor tamaño alcanzaba 260 mm. El estadio tumoral más frecuente fue localizado (70.9%). Presentaron otro cáncer asociado el 9.7% de pacientes El patrón histológico predominante fue fusiforme 73.8%. El 84.5% tuvo bajo índice mitótico. La inmunohistoquímica mostró la expresión de KIT (CD 117) 94.17%, CD 34 77.5%, Vimentina 96.6%, NSE 84.9%, Alfa actina 52.7%, CD56 44.4%, S-100 32.3% y Actina 20.0%. La característica tomográfica más frecuente fue tumor heterogéneo (43.6%). La distribución según clasificación de riesgo fue: muy bajo riesgo 3.9%, bajo riesgo 28.2%, riesgo intermedio 37.7% y alto riesgo 30.1%. La resección quirúrgica completa se realizó en 87.4% de pacientes, 4.9% de pacientes recibió Imatinib. La sobrevida global acumulada a 5 años fue 31.07%. En el análisis bivariado se encontró asociación estadística entre el haber sobrevivido con: ausencia de cáncer asociado p= 0.004, CD 34 p=0.01, índice mitótico bajo p=0.00 y tratamiento quirúrgico recibido p= 0.000. En el análisis multivariado se encontró asociación estadística de mayor sobrevida con: los de menor tamaño del tumor p=0.015 (IC -3.67, -0.41), estadio tumoral localizado p=0.036 (IC -5.83, -0.19), menor índice mitótico p=0.038 (IC -0.86, 0.02), paciente asintomático p=0.009 (IC 1.25, 8.62), no recidiva del tumor p=0.01 (IC -8.49, -1.17) y el no presentar metástasis p=0.001 (IC 2.66, 10.62). CONCLUSIONES: Los resultados de nuestro estudio fueron similares a lo que reporta la literatura internacional. Los factores que se asociaron a mayor sobrevida fueron: haber recibido tratamiento quirúrgico, pacientes con menor tamaño tumoral, estadio tumoral localizado, índice mitótico bajo, paciente asintomático, no recidiva del tumor, no metástasis y no cáncer asociado.

OBJECTIVE: To determine the clinical, radiological, histopathological manifestations and survival of patients with gastrointestinal stromal tumor (GIST)in the National Hospital Edgardo Rebagliati Martins (HNERM) from Lima, Perú. MATERIAL AND METHODS: This is a descriptive and retrospective study, which was based on the medical records of 103 patients with confirmed GIST with immunohistochemical. All the patients were evaluated and treated at the HNERM, from January 2002 until December 2010. RESULTS: In 103 patients between 30 and 88 years the average age was 64 years. The tumor was more frequent in females (52%). The mean disease duration was 7 months. The most frequent form of presentation was gastrointestinal bleeding (48.3%). The diagnosis was made more by endoscopy (50.5%). The prevalence of GISTs by organs was more frequent in stomach (56.3%). The average size of the tumors was 98mm, 49% had a size between 50mm and 100mm, the largest tumor was 260 mm. Tumor stage more frequent was localized (70:9%). GIST associated with another cancer was 9.7% of patients. The predominant histologic pattern was fusiform (73.8%). The 84.5% had low mitotic index. Immunohistochemistry showed expression KIT (CD 117) was 94.17%, CD34 77.5%, Vimentin 96.6%, NSE 84.9%, alpha actin 52.7%, CD56 44.4%, S-100 32.3% and Actin 20%. The tomographic characteristic more frequent was heterogeneous tumor (43.6%).The distribution according to risk classification was: very low risk 3.9%, low risk 28.2, intermediate risk 37.7% and high risk 30.1%. Complete surgical resection was performed in 87.4% of patients and 4.9% of patients received imatinib. The cumulative overall survival at 5 years was 31.07%. In bivariate analysis statistical association was found between surviving with: no presence of cancer associated p = 0.004, CD 34 p = 0.01, low mitotic index p = 0.00 and received surgical treatment p = 0.000. In multivariate analysis one found statistical association of longer survival with smaller tumor size p = 0.015 (CI -3.67, -0.41), localized tumor stage p = 0.036 (CI -5.83, -0.19), lower mitotic index p = 0.038 (CI -0.86, 0.02), asymptomatic patient p=0.009 (CI 1.25, 8.62), no tumor recurrence p = 0.01 (CI -8.49, -1.17), and no metastasis p = 0.001 (CI 2.66, 10.62). CONCLUSIONS: The results of our study were similar to what was reported in international literature. Factors that were associated with longer survival were receiving surgical treatment, patients with smaller tumor size, tumor stage localized, low mitotic index, asymptomatic patient, not tumor recurrence, not metastasis and no cancer associated.

Tumores del estroma gastrointestinal (GIST) / Gastrointestinal stromal tumors (GIST)

Gastrointestinal stromal tumors are rare but are the most common mesenchymal tumors of the gastrointestinal tract. They originate from the interstitial cells of Cajal and mostly expressing positive c-kit (CD117). These are an heterogeneous group of injuries ranging from benign to aggressive. To date, the tumor size and number of mitosis are the most useful tool for predicting the risk of malignancy. Endosonography is an excellent imaging method for characterizing lesions and it allows obtaining material for pathological diagnosis. Surgical resection is the therapy of choice for localized disease and good prognosis. The use of imatinib in advanced disease has improved survival.

Los tumores del estroma gastrointestinal son poco frecuentes, pero son los tumores del mesénquima más frecuentes del tubo digestivo. Se originan de las células intersticiales de Cajal y expresan en su gran mayoría c-kit (CD117) positivo. Son un grupo heterogéneo de lesiones que van desde un comportamiento benigno a uno altamente agresivo. Hasta la fecha el tamaño tumoral y el número de mitosis son los elementos más útiles para predecir el riesgo de malignidad. La endosonografía es un excelente método de imagen para caracterizar las lesiones y, además, permite la obtención de material para diagnóstico anatomopatológico. La resección quirúrgica es la terapia de elección para la enfermedad localizada y con buen pronóstico. El uso de Imatinib en la enfermedad avanzada ha mejorado la sobrevida en los pacientes.

Tumor estromal gastrointestinal: experiência no tratamento da doença localizada e avançada no Hospital de Clínicas da Universidade Federal do Paraná / Gastrointestinal stromal tumor: experience in the treatment of localized and advanced disease at the Clinical Hospital of Parana Federal University

Introdução: Tumores estromais gastrointestinais (GIST) são neoplasias raras que se originam das células intersticiais de Cajal. Objetivo: Descrever a experiência do Hospital de Clínicas de Curitiba no tratamento do GIST localizado e avançado, com análise das características clínicas e anatomopatológicas e uso do imatinibe. Método: Estudo retrospectivo com 32 pacientes com diagnóstico por imuno-histoquímica, c-Kit positivo, no período de 2003 a 2008. Resultados: Idade mediana: 66 anos; tamanho mediano do tumor de 8,4 cm; e as localizações mais frequentes foram estômago em 46,9 por cento e intestino delgado em 40,9 por cento. Pacientes com alto risco de agressividade: 37,5 por cento; apresentavam doença localizada no diagnóstico 23 pacientes: 39,1 por cento recaíram e 9 com doença avançada. O seguimento mediano foi de 43,7 meses, com sobrevida global em 5 anos no grupo total de 56,2 por cento. Na doença localizada, a sobrevida global em 5 anos foi de 73,8 por cento e na avançada de 37,5 por cento (p=0,03). Não ocorreu impacto dos fatores prognósticos na sobrevida. Utilizou-se omesilato de imatinibe em 16 pacientes: 43,8 por cento por metástase inicial, 37,5 por cento recaída a distância, 12,5 por cento recaída local e 6,2 por cento margem comprometida. A sobrevida global com uso do imatinibe mediana foi de 53 meses e a sobrevida livre de primeira progressão de 32,9 meses. Houve boa tolerabilidade ao imatinibe e apenas dois pacientes utilizaram osunitinibe. Conclusão: A maioria dos tumores era grande, de localização gástrica e de alto risco de agressividade. A taxa de recaída na doença localizada foi alta. E a sobrevida global dos pacientes de doença localizada e que utilizaram o imatinibe foi considerada satisfatória.

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.

Resección laparoscópica mano asistida: de tumor del estroma gastrointestinal de Yeyuno / Laparoscopic hand resection assisted: gastrointestinal stromal tumor of ileum

Los tumores del intestino delgado son relativamente raros con una incidencia de 5 por ciento. Entre estos se encuentran los tumores del estroma gastrointestinal que son un grupo de neoplasias de origen mesenquimal y constituyen el 0,1 por ciento al 3 por ciento de los tumores del tracto digestivo. La presentación clínica es inespecífica, generalmente los pacientes consultan por hemorragia digestiva superior, dolor o tumor palpable. La determinación de marcadores es fundamental para la confirmación patológica y se sustenta con los estudios inmunohistoquímicos basados en la expresión de CD117 (receptor c-kit), CD34, desmina y proteína S100, para diferenciarlos de otros tumores mesenquimales, neurales y neuroendocrinos. El comportamiento clínico de este tipo de tumores es difícil de predecir. La resección quirúrgica con márgenes libres de lesión es el tratamiento de elección para las lesiones localizadas.

Tumors of the small bowel are relative rare with an incidence of 5 %. These kinds lesions we find in the stroma gastrointestinal tumors, there are a group of neoplasm mesenquimal original. They are constitutive 0,1 % to 3 % of the digestive tract tumors. The clinical presentation is no specific generally the patients consult for superior digestive bleeding, pain or palpable mass. The market determination is important to the pathological confirmation and are sustention with immunohistochemestry studies based in the expression of CD117 (c-kit receptor), CD34, desmine and S100 protein, to be differential of another tumors how mesenchyme, neural, and neuroendocrines. The biological clinical courses of these kinds of tumors are difficult to predict. The surgical resection with free lesion margins is the treatment of choice for the localized lesions.

Anatomía patológica de los tumores estromales gastrointestinales (GIST) / Histopathology of gastrointestinal stromal tumors (GIST)

En los últimos años se han publicado numerosos artículos acerca de los tumores estromales gastrointestinales (GlST), los que nos han permitido entender mejor la biología y aspectos genéticos de este tipo de tumores. El presente artículo está enfocado en revisar el conocimiento actual acerca del diagnóstico anatomopatológico de los GIST, ofreciendo una guía práctica para su diagnóstico, diagnóstico diferencial y describiendo brevemente sus aspectos biológicos, epidemiológicos y moleculares.

Numerous papers about gastrointestinal stromal tumors (GIST) have been published in recent years, allowing us a better understanding about the biology and genetic aspects of this kind of tumors. The present artic/e reviews the actual knowledge about the pathological diagnosis of GIST, its differentials and briefly describing its biological, epidemiological and molecular aspects.

Tumores del estroma gastrointestinal (GIST), un particular tipo de neoplasia / Gastrointestinal stromal tumors: an update

Gastrointestinal stromal tumors (GIS) are a heterogeneous group of tumors that express CD 117 molecule in their sur face. They may behave as benign tumors or be highly aggressive. A better survival of patients with these tumors has been achieved using the new molecular therapies such as imatinib mesylate, sunitinib and others. This review analyzes the prognostic factors of these tumors, their clinical features and the criteria for malignant behavior. The value of therapeutic alternatives such as radiotherapy chemotherapy and the new molecular therapies are also discussed.