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

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

Antral plexiform fibromyxoma: case report of a rare mesenchymal neoplasm

Plexiform fibromyxoma (PF) is a rare mesenchymal neoplasm of the stomach usually arising in the gastric antrum, and its main differential diagnosis is gastrointestinal stromal tumor. Most common symptoms are hematemesis, anemia. Immunohistochemically, positivity for smooth muscle actin (SMA) and vimentin suggests the diagnosis of PF. We report the case of a 56-year-old female patient with a 30- day history of nausea at presentation 4 years ago. Gastroscopy at that time revealed a subepithelial lesion (SEL) in the gastric antrum, measuring approximately 20 mm in diameter, with leakage of serous fluid after biopsy. Histopathology showed only an inflammatory process. Follow-up gastroscopies were performed 24, 36, and 48 months later, with surveillance biopsy at each follow-up. The last gastroscopies showed changes in lesion appearance, reduction in size, and absence of fluid leakage. Histopathology showed bland spindle cell proliferation, with a vaguely plexiform/multinodular pattern, in a fibromyxoid stroma with an arborizing capillary network without mitoses. The tumor cells were positive for SMA and negative for DOG1, CD117, CD34, S100, desmin, EMA, CD10, calponin, and beta-catenin. The choice of treatment and follow-up depends on the SEL features, but because no cases of malignancy or metastatic disease have previously been reported, the patient chose a conservative approach.

El fibromixoma plexiforme (FP) es una rara neoplasia mesenquimatosa del estómago que generalmente surge en el antro gástrico. Su principal diagnóstico diferencial es el tumor del estroma gastrointestinal. Los síntomas más comunes de los FP son hematemesis y anemia. Inmunohistoquímicamente, la positividad para actina del músculo liso (SMA) y vimentina sugieren el diagnóstico de FP. Presentamos el caso de una paciente de 56 años de edad que inicia su enfermedad hace 4 años con náuseas de 30 días de evolución. La primera gastroscopia reveló una lesión subepitelial (SEL) en el antro gástrico, de aproximadamente 20 mm de diámetro, con fuga de líquido seroso después de la biopsia. La histopatología mostró sólo un proceso inflamatorio. Se realizaron gastroscopias de seguimiento a los 24, 36 y 48 meses con biopsia de vigilancia en cada seguimiento. Las gastroscopias siguientes mostraron cambios en la apariencia de la lesión, reducción de tamaño y ausencia de fuga de líquido. La última histopatología mostró una proliferación blanda de células fusiformes, con un patrón vagamente plexiforme/multinodular, en un estroma fibromixoide con una red de capilares arborizantes sin mitosis. Las células tumorales fueron positivas para SMA y negativas para DOG1, CD117, CD34, S100, desmina, EMA, CD10, calponina y beta-catenina. La elección del tratamiento y el seguimiento depende de las características del SEL, sin embargo, por ser una enfermedad que no presentaba rasgos de enfermedad maligna o metastásica, el paciente eligió un mantener un enfoque conservador.

Estrategia quirúrgica en un paciente con tumor agresivo del tracto gastrointestinal en cuarta porción de duodeno

Los tumores del estroma gastrointestinal son tumores infrecuentes y se corresponden con un 1 % de todas las neoplasias gastrointestinales; la localización duodenal solo representa entre 3-5 %. Se realizó este reporte de caso con el propósito de divulgar la estrategia quirúrgica seguida con un paciente portador de tumor invasivo del estroma gastrointestinal (>10 cm) de localización atípica, en cuarta porción del duodeno. El internamiento fue por tumor abdominal y anemia. El ejercicio clínico consistió en identificar una masa abdominal izquierda con contacto lumbar. Las pruebas diagnósticas realizadas fueron: pruebas de química sanguínea, ultrasonido abdominal, tomografía axial computadorizada y endoscopia digestiva con biopsia que confirmó el diagnóstico. El procedimiento quirúrgico fue resección de la cuarta porción de duodeno y primeras asas yeyunales, con restablecimiento de la funcionabilidad intestinal mediante duodeno (segunda porción)-yeyunostomía latero-lateral. La cirugía fue interrumpida por inestabilidad hemodinámica del paciente y cuatro días después fue llevado otra vez al salón de operaciones por presentar peritonitis, con salida de pus por los drenajes abdominales, que fue solucionada con lavado de la cavidad. La morbilidad estuvo acompañada por una fístula pancreática. En el tercer tiempo quirúrgico se realizó resección del tumor residual, nefrectomía izquierda y control de la fístula pancreática. Después de un año el paciente se encuentra libre de enfermedad tumoral. Se puede concluir que la estrategia de manejo en pacientes con tumores del estroma gastrointestinal de localización atípica representa un reto para el cirujano como miembro del grupo multidisciplinar y depende de la extensión del tumor, el estado del paciente y el manejo oportuno del equipo quirúrgico.

Gastrointestinal stromal tumors are rare tumors and correspond to 1% of all gastrointestinal neoplasms; duodenal location only represents between 3-5%. This case report was made for disclosing the surgical strategy followed in a patient with invasive gastrointestinal stromal tumors (>10 cm) of atypical location in the duodenum fourth portion. Hospitalization was due to abdominal tumor and anemia. The clinical exercise consisted of identifying a left abdominal mass with lumbar contact. The diagnostic tests performed were: blood chemistry tests, abdominal ultrasound, computerized axial tomography, and digestive endoscopy with biopsy that confirmed the diagnosis. The surgical procedure was resection of the duodenum fourth portion and the first jejunal loops, with restoration of intestinal function through duodenum (second portion) lateral jejunostomy. The surgery was interrupted due to the patient's hemodynamic instability, and four days later he was taken back to the operating room due to peritonitis, with pus coming out of the abdominal drains, which was resolved by washing the cavity. Morbidity was accompanied by a pancreatic fistula. In the third surgical time, resection of the residual tumor, left nephrectomy, and control of the pancreatic fistula were performed. After one year the patient is free of tumor disease. The management strategy in patients with atypically located gastrointestinal stromal tumors represents a challenge for the surgeon as a member of the multidisciplinary group and depends on the extent of the tumor, the patient's condition, and the surgical team timely management.

Hemorragia de intestino delgado en un paciente con Neurofibromatosis tipo 1

Presentamos el caso de un paciente masculino de 32 años con antecedente de Neurofibromatosis tipo 1, que se presenta por hemorragia de intestino delgado activo, diagnosticada inicialmente al observar sangrado en ileoscopía, al cursar con inestabilidad hemodinámica se realiza angiotomografía abdominal identificando a nivel de yeyuno medio una masa con captación de contraste y sangrado activo por lo cual se realiza una angiografía con embolización arterial de la rama que irriga dicha zona. Con el paciente estable, se realizó una enteroscopía anterógrada de doble balón, observando una lesión subepitelial, ulcerada, se realiza tatuaje endoscópico y finalmente se envía a cirugía para resección mediante laparoscopia. El estudio anatomopatológico fue compatible con un tumor estromal gastrointestinal (GIST) yeyunal.

We present the case of a 32-year-old male patient with a history of Neurofibromatosis type 1, who presented with active small bowel bleeding, initially diagnosed by observing bleeding in ileoscopy, presenting with hemodynamic instability, abdominal angiotomography was performed, identifying a mass with contrast enhancement and active bleeding at the middle jejunum level, for which an angiography with arterial embolization of the branch that supplies said area is performed. With the patient stable, a double-balloon antegrade enteroscopy was performed, observing a subepithelial, ulcerated lesion, endoscopic tattooing was performed and finally surgery was sent for resection by laparoscopy. The pathology study was compatible with a jejunal gastrointestinal stromal tumor (GIST).

Hallazgo Incidental de Tumor Estromal Gastrointestinal en Divertículo de Meckel. Caso Clínico

Introducción: El divertículo de Meckel es un defecto sacular ubicado en el íleon distal, con una tasa de complicaciones de 4%. La resección quirúrgica está indicada en estas complicaciones, siendo controversial en casos asintomáticos o incidentales. Dentro de las complicaciones existen tumores como los estromales gastrointestinales (GIST). Caso Clínico: Hombre de 57 años, postrado por múltiples factores, presenta úlcera por presión sacra grado IV sobreinfectada y con evolución tórpida. Se decide realizar colostomía terminal, evidenciando un divertículo de Meckel en el intraoperatorio con aspecto sospechoso, por lo cual se decide resecar, resultando en un GIST de bajo riesgo. Se mantiene en seguimiento por 6 meses, sin recidiva tumoral. Discusión: Mientras que la cirugía pareciera ser imperativa en complicaciones, aún existe debate respecto a las indicaciones de resección de divertículo de Meckel hallado incidentalmente en el intraoperatorio por cirugía de otra causa. Si bien puede aumentar la morbimortalidad, la resección estaría indicada según ciertas características como edad, sexo, tamaño y aspecto, dado el riesgo de desarrollo de complicaciones y neoplasias.

Introduction: Meckel's diverticulum is a saccular defect located in the distal ileum, with a complication rate of 4%. Surgical resection is indicated when these complications occur, yet resection in asymptomatic or incidental cases remains controversial. Among these complications, the appearance of tumors such as gastrointestinal stromal (GIST), may occur. Case Report: A 57-year-old male, bedridden due to multi- ple factors, presents with a complicated grade IV sacral pressure ulcer with a torpid evolution. An end colostomy is performed, evidencing a suspicious-looking Meckel's diverticulum intraoperatively, which was resected and resulted to be a low-risk GIST. At a 6-month follow-up, the patient showed no evidence of tumor recurrence. Discussion: While surgery seems to be imperative in complications, there is still a debate regarding the indications for resection of Meckel's diverticulum found incidentally intraoperatively due to surgery for another cause. Although it could increase morbidity and mortality, resection could be indicated according to certain characteristics such as age, sex, size and appearance, given the risk of developing complications and neoplasm.

Calcifying fibrous tumor.

Calcifying fibrous tumor is a rare benign mesenchymal neoplasm. The etiology and pathogenesis of this tumor are uncertain. It has wide anatomical distribution. The tumor is most commonly found in the soft tissues of the extremities in younger individuals. However, in middle-aged patients, it tends to affect the visceral locations more commonly. In visceral location, it can mimic aggressive lesions clinically. The purpose of this report is to describe a case of calcifying fibrous tumor in a 71-year-old female with a history of breast carcinoma who was found to have an incidental small bowel mass on her follow-up. Clinically and radiologically, the mass was suspicious for either metastatic disease or gastrointestinal stromal tumor. The patient underwent open small bowel resection, and a 6.5 cm segment of the small bowel was sent to pathology. Grossly, a 2.0 cm tan-pink smooth round submucosal polyploid mass protruding into the lumen, mimicking a gastrointestinal stromal tumor, was identified. The tumor was hard and serially sectioned to reveal a white, calcified cut surface. Microscopically, the tumor appeared hypocellular and composed of scant spindle cells embedded in a dense, hyalinized and calcified collagenous stroma. Immunohistochemical stains for pan-cytokeratin, DOG1, desmin, S100, CD34, and MUC4 were negative, and a diagnosis of the calcifying fibrous tumor was rendered. This case provides a rare gross specimen image of calcifying fibrous tumor and highlights the importance of knowledge of rare entities in providing an accurate diagnosis for entities that can mimic other lesions.

Tumor de ovario de los cordones sexuales con túbulos anulares: reporte de caso y revisión de la literatura / Ovarian sex cord tumor with annular tubules: case report and review of the literature

RESUMEN Objetivos: Reportar un caso de Tumor de Ovario de los Cordones Sexuales con Túbulos Anulares (TCSTA), hacer una revisión de la literatura acerca del diagnóstico, tratamiento y pronóstico de esta condición. Materiales y métodos: Se informa el caso de una mujer que consultó al Instituto Nacional de Cancerología, Bogotá (Colombia), con diagnóstico final de TCSTA avanzado, quien recibió tratamiento quirúrgico y quimioterapia con evolución satisfactoria a los 6 meses. Se realizó una búsqueda de la literatura en las bases de datos Medline vía PubMed, LILACS y Scielo, que incluía reportes y series de caso de mujeres con diagnóstico de TCSTA, publicados desde 1990, sin incluir rango de edad. Se extrajo información sobre el diagnóstico, tratamiento y pronóstico reportado. Se realizó resumen narrativo de los hallazgos. Resultados: Se incluyeron 14 publicaciones que incluían 26 pacientes. La edad media al diagnóstico fue de 22,5 años. Los síntomas principales fueron alteraciones de la menstruación y dolor pélvico. La tomografía computarizada fue la tecnología de imágenes más frecuentemente utilizada. El tratamiento fue quirúrgico, siempre usado, acompañado de quimioterapia (29 %); 2 casos recibieron radioterapia. Hubo recaída en el 20 % de los casos. La mortalidad fue del 12,5 %, toda en el primer año. Conclusiones: La información sobre la utilidad diagnóstica de las imágenes, marcadores tumorales y estudios de histoquímica es escasa, como también los datos sobre el pronóstico de la entidad. El tratamiento quirúrgico es el de elección, teniendo en cuenta el deseo de fertilidad de la paciente y el estadio tumoral. Se requieren más estudios que documenten de manera más detallada el manejo de esta condición.

ABSTRACT Objectives: To report a case of ovarian sex cord tumor with annular tubules (SCTAT) and conduct a literature review on diagnosis, treatment and prognosis of this condition. Material and methods: Case report of a woman with a final diagnosis of advanced SCTAT seen at the National Cancer Institute in Bogota (Colombia) who received surgical treatment and chemotherapy with a satisfactory course after 6 months. A literature search was conducted in the Medline via PubMed, LILACS and Scielo databases, including case reports and series of women diagnosed with SCTAT published since 1990, not using age ranges. Information about diagnosis, treatment and reported prognosis was retrieved. A narrative summary of the findings was prepared. Results: Fourteen publications with 26 patients were included. Mean age at diagnosis was 22.5 years. The main symptoms were menstruation abnormalities and pelvic pain. Computed tomography (CT) was the imaging technology most frequently used. Surgical treatment was used in all cases, together with chemotherapy in 29 %; 2 patients received radiotherapy. Recurrence occurred in 20 % of cases. Mortality was 12.5 %, with all deaths occurring within the first year. Conclusions: There is a paucity of information about the diagnostic utility of imaging, tumor markers and histochemical studies, as well as prognosis of this disease condition. Surgery is the treatment of choice, taking into consideration the patient's wishes regarding fertility, as well as the stage of the tumor. Further studies are needed to provide more detailed information about this condition.

Gist sincrónico con adenomioma gástrico: Reporte de caso y revisión de la literatura / Synchronous gist with gastric adenomyoma: Case report and literature review

Introducción: La colisión de dos tumores de diferente estirpe celular en un mismo órgano es infrecuente; a pesar de las asociaciones descritas en la literatura, el hallazgo de GIST con adenomioma en sincronismo, llama aún más la atención debido a sus distintos orígenes celulares. Reporte de caso: Presentamos el caso de una paciente mujer de 57 años de edad, quien es sometida a cirugía de resección doble en cuña, y distintos exámenes incluido el anátomo-patológico. Conclusión: Se demuestra la presencia de tumores sincrónicos, GIST gástrico y adenomioma gástrico, a pesar de la infrecuencia de este hallazgo.

Background:The collision of two tumors of different cell lines in the same organ is infrequent; even though, the associations described in the literature, the finding of synchronous GISTwith adenomyoma draws even more attention due to its different cellular origins. We present the case of a 57-year-Case report:old female patient who underwent double wedge resection surgery and various examinations, including pathology. Conclusion:The presence of synchronous tumors, gastric GIST and gastric adenomyoma is demonstrated,despite the infrequency of this finding.

Avances en tumores del estroma gastrointestinal: ¿hacia dónde vamos?

Gastrointestinal Stromal Sarcomas (GIST) are mesenchymal neoplasms whose incidence accounts for 1-2% of digestive tumors, being located in the stomach (55-60%) and small intestine (30%). The advances in its knowledge and management succeeded in the last years have being spectacular. This review aims to summarize the most important of them for surgeons. We identified four areas of interest: molecular oncology, laparoscopic approach, management of GIST located at unusual locations, and management of advanced GIST. Advances in the field of molecular oncology lead to the discovery of new oncogenic mutations making the term Wil Type GIST obsolete. Moreover, these advances allow for the development of 2 new drugs: Avapritinib and Ripretinib, that added to the previous 3 commercially available drugs (imatinib, sunitinib and regorafenib) make possible the management of GIST with resistant mutations. The principles of the surgical management of primary GIST are well stablished which laparoscopic approach must accomplish. This approach is limited by 2 main factors: location and size. The diagnosis of GIST in unusual locations as esophagus, duodenum, rectum of out of the gastrointestinal tract (EGIST), implies an extraordinary therapeutic challenge, being imperative to manage them by surgeons and oncologist among others in the setting of a multidisciplinary team. The management of advanced/metastatic GIST has changed in a revolutionary fashion because surgery is now part of its treatment as adjuvant to tyrosine kinase inhibitors.

Los tumores del estroma gastrointestinal (GIST) suponen el 1-2% de los tumores digestivos, siendo su localización más frecuente el estómago (55-60%) y el intestino delgado (30%). Los avances más importantes sucedidos en los últimos años se centran en cuatro áreas: biología molecular, abordaje quirúrgico laparoscópico, manejo técnico del GIST en localizaciones inusuales y tratamiento e integración de la cirugía en el manejo del GIST avanzado. Los avances en el conocimiento de la biología molecular del GIST han dado lugar a la progresiva identificación de nueva mutaciones oncogénicas que hacen del concepto wild type obsoleto. Estos avances han permitido el desarrollo de dos nuevos fármacos, avapritinib y ripretinib, lo que permite el tratamiento de pacientes con mutaciones resistentes a las tres líneas terapéuticas clásicas. El tratamiento quirúrgico del GIST se rige por unos principios técnicos bien establecidos que el abordaje laparoscópico debe cumplir, abordaje que queda limitado por dos factores clave: localización y tamaño. El GIST de localización infrecuente (esófago, duodeno o recto, o extradigestivo) supone un reto terapéutico. Estos pacientes deben ser manejados en un contexto multidisciplinario. La cirugía queda integrada en el manejo del GIST avanzado, considerándose como adyuvante a los inhibidores de la tirosina cinasa.

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)

Psychological Distress, Fatigue and Quality of Life in Patients with Gastrointestinal Stromal Tumors.

Background: Gastrointestinal stromal tumors (GIST) represent 1% of all gastrointestinal tumors and are included in the list of rare diseases. Objective: 1) To evaluate levels of psychological distress, fatigue, and quality of life. 2) To identify the variables that most influence distress among Mexican patients with GIST. Design: A cross-sectional study was conducted with a consecutive sample of 100 patients with GIST, who completed the following questionnaires online: Hospital Anxiety and Depression Scale (HADS) as a measure of distress, Multidimensional Fatigue Inventory (MFI), and Quality of Life Questionnaire (QLQ C30). Results: Distress was present in 31% of patients. No association was found between distress and sociodemographic/clinical variables. The patients with distress demonstrated higher scores in all fatigue dimensions and, regarding quality of life, had more symptoms and were lower functioning. Distress was positively associated with all fatigue dimensions and with QLQ C30 symptoms. Negative associations were found between distress and QLQ C30 functioning dimensions. The predictors of psychological distress were general fatigue, reduced motivation, and emotional functioning. Conclusion: The percentage of patients with distress was akin to the levels found in patients with the most common types of cancer. Fatigue in patients with GIST should be evaluated and managed to improve distress levels.

Internet-Delivered Cognitive Behavioral Therapy and Psychoeducation Program for Patients with Gastrointestinal Stromal Tumors.

Gastrointestinal stromal tumors (GIST) account for 0.2-3% of all cancer diagnoses and are thus considered a rare type of cancer. Severe levels of fatigue occur in 33% of patients on imatinib, but besides treatment, fatigue is also associated with psychological distress. As far as we know, only one previous study has reported on the design of a psychoeducational intervention for caregivers and patients with GIST. The current study was conducted to compare the preliminary effectiveness of an Internet-delivered cognitive behavioral stress management intervention and psychoeducation program for anxiety, depression, fatigue, and quality of life in patients with gastrointestinal stromal tumors (GIST). This study had an experimental pre-test post-test design with a control group. Ninety-nine patients were randomly allocated to an Internet-delivered cognitive program (n = 50) or psychoeducation control group (n = 49). Participants completed the questionnaires at three time points (pre-test, post-test, and 3-month follow-up). Twenty-seven participants completed the intervention. A significant interaction effect between groups and time was found for general fatigue [F(2, 28) = 8.2, p < 0.001, ƞ2p = 0.25], reduced motivation [F(2, 28) = 3.5, p < 0.05, ƞ2p = 0.12], distress [F(2, 28) = 3.4, p < 0.05, ƞ2p = 0.12], and global health status [F(2, 28) = 3.8, p < 0.05, ƞ2p = 0.13]. Both intervention programs showed reductions in the dimensions of fatigue and improvements in distress and dimensions of quality of life. Additional research on a larger sample size is needed to confirm the effects obtained in this study.

Calcifying fibrous tumor

ABSTRACT Calcifying fibrous tumor is a rare benign mesenchymal neoplasm. The etiology and pathogenesis of this tumor are uncertain. It has wide anatomical distribution. The tumor is most commonly found in the soft tissues of the extremities in younger individuals. However, in middle-aged patients, it tends to affect the visceral locations more commonly. In visceral location, it can mimic aggressive lesions clinically. The purpose of this report is to describe a case of calcifying fibrous tumor in a 71-year-old female with a history of breast carcinoma who was found to have an incidental small bowel mass on her follow-up. Clinically and radiologically, the mass was suspicious for either metastatic disease or gastrointestinal stromal tumor. The patient underwent open small bowel resection, and a 6.5 cm segment of the small bowel was sent to pathology. Grossly, a 2.0 cm tan-pink smooth round submucosal polyploid mass protruding into the lumen, mimicking a gastrointestinal stromal tumor, was identified. The tumor was hard and serially sectioned to reveal a white, calcified cut surface. Microscopically, the tumor appeared hypocellular and composed of scant spindle cells embedded in a dense, hyalinized and calcified collagenous stroma. Immunohistochemical stains for pan-cytokeratin, DOG1, desmin, S100, CD34, and MUC4 were negative, and a diagnosis of the calcifying fibrous tumor was rendered. This case provides a rare gross specimen image of calcifying fibrous tumor and highlights the importance of knowledge of rare entities in providing an accurate diagnosis for entities that can mimic other lesions.

Gastrointestinal stromal tumor: outcomes of the past decade in a reference institution in southern brazil / Tumor estromal gastrointestinal (gist): resultados da última década em uma instituição de referência no sul do brasil

ABSTRACT - BACKGROUND: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the digestive tract and has a wide variation in biological behavior; surgical resection remains the main form of treatment. AIM: This study aimed to analyze clinicopathological characteristics and survival of patients with GIST in a reference institution for oncological diseases. METHODS: An observational, longitudinal, and retrospective study of patients diagnosed with GIST from January 2011 to January 2020 was carried out by analyzing epidemiological and clinical variables, staging, surgical resection, recurrence, use of imatinib, and curves of overall survival (OS) and disease-free survival (DFS). RESULTS: A total of 38 patients were included. The majority (58%) of patients were males and the median age was 62 years. The primary organs that were affected by this tumor were stomach (63%) and small intestine (17%). Notably, 24% of patients had metastatic disease at diagnosis; 76% of patients received surgical treatment and 13% received neoadjuvant treatment; and 47% of patients received imatinib as adjuvant or palliative therapy. Tumor recurrence was 13%, being more common in the liver. The 5-year OS was 72.5% and DFS was 47.1%. The operated ones had better OS (87.1% vs. 18.5%) and DFS (57.1% vs. 14.3%) in 5 years. Tumor size ≥5 cm had no difference in OS at 5 years, but DFS was 24.6%, when compared with 92.3% of smaller tumors. Patients who were undergoing neoadjuvant therapy and/or using imatinib did not show any significant differences. CONCLUSIONS: Surgical treatment with adequate margins allows the best gain in survival, and the use of imatinib in more advanced cases has prognostic equity with less advanced-stage tumors. Treatment of metastatic tumors seems promising, requiring further studies.

RESUMO - RACIONAL: O Tumor estromal gastrointestinal (Gastrointestinal stromal tumor - GIST) é a neoplasia mesenquimal mais comum do trato digestivo, possui comportamento biológico variado e a principal forma de tratamento é a ressecção cirúrgica. OBJETIVO: analisar as características clínico-patológicas e a sobrevida de pacientes com GIST em uma instituição de referência para doenças oncológicas. MÉTODOS: Foi realizado um estudo observacional, longitudinal e retrospectivo de pacientes com diagnóstico de GIST de janeiro de 2011 a janeiro de 2020, analisando variáveis epidemiológicas e clínicas, estadiamento, ressecção cirúrgica, recidiva, uso de imatinibe e curvas de sobrevida global (SG) e sobrevida livre de doença (SLD). RESULTADOS: foram incluídos 38 pacientes, a maioria (58%) do sexo masculino, idade mediana de 62 anos. Os principais órgãos primários foram estômago (63%) e intestino delgado (17%). 24% tinham doença metastática ao diagnóstico. 76% receberam tratamento cirúrgico e 13% tratamento neoadjuvante. 47% dos pacientes receberam Imatinib como terapia adjuvante ou paliativa. A recorrência tumoral foi de 13%, mais comum no fígado. SG de 5 anos foi de 72,5% e SLD 47,1%. Os operados tiveram melhor SG (87,1% vs. 18,5%) e SLD (57,1% vs. 14,3%) em 5 anos. O tamanho do tumor igual ou maior que 5 cm não teve diferença na SG em 5 anos, mas SLD foi de 24,6%, em comparação com 92,3% dos tumores menores. Pacientes em terapia neoadjuvante e/ou em uso de imatinibe não apresentaram diferenças significativas. CONCLUSÕES: O tratamento cirúrgico com margens adequadas permite o melhor ganho de sobrevida, e o uso de Imatinibe em casos mais avançados tem equidade prognóstica com tumores em estágio menos avançado. O tratamento de tumores metastáticos parece promissor, necessitando de mais estudos.

Laparoscopic versus open surgery in gastric gastrointestinal stromal tumors larger than 5 cm: a systematic review and meta-analysis / Abordagem laparoscópica versus cirurgia aberta em tumores estromais gástricos superiores a 5 cm: revisão sistemática e meta-análise

ABSTRACT BACKGROUND: Surgical resection represents the main treatment for resectable nonmetastatic gastric gastrointestinal stromal tumors. Despite the feasibility and safety of laparoscopic resection, its standard use in gastric tumors larger than 5 cm is yet to be established. AIMS: This study aimed to compare the current evidence on laparoscopic resection with the classical open surgical approach in terms of perioperative, postoperative, and oncological outcomes. METHODS: The PubMed, Scopus, and Web of Science databases were consulted. Articles comparing the approach to gastric gastric gastrointestinal stromal tumors larger than 5 cm by open and laparoscopic surgery were eligible. A post hoc subgroup analysis based on the extent of the surgery was performed to evaluate the operative time, blood loss, and length of hospital stay. RESULTS: A total of nine studies met the eligibility criteria. In the study, 246 patients undergoing laparoscopic surgery and 301 patients undergoing open surgery were included. The laparoscopic approach had statistically significant lower intraoperative blood loss (p=0.01) and time to oral intake (p<0.01), time to first flatus (p<0.01), and length of hospital stay (0.01), compared to the open surgery approach. No significant differences were found when operative time (0.25), postoperative complications (0.08), R0 resection (0.76), and recurrence rate (0.09) were evaluated. The comparative subgroup analysis between studies could not explain the substantial heterogeneity obtained in the respective outcomes. CONCLUSION: The laparoscopic approach in gastric gastrointestinal stromal tumors larger than 5 cm compared to the open surgical approach is a technically safe and feasible surgical method with similar oncological results.

RESUMO RACIONAL: A resseção cirúrgica representa a principal forma de tratamento de tumores estromais gástricos não metastáticos ressecáveis. Apesar da viabilidade e segurança da ressecção laparoscópica, a sua utilização generalizada em tumores gástricos com mais de 5 centímetros ainda não foi estabelecida. OBJETIVOS: Comparar as evidências atuais sobre a resseção por via laparoscópica com a cirurgia aberta, em termos de resultados peri-, pós-operatórios e oncológicos. MÉTODOS: Foram consultadas as bases de dados PubMed, Scopus e Web of Science. Artigos que comparassem a abordagem de tumores estromais gástricos gástricos com tamanho superior a 5 centímetros por cirurgia aberta e por via laparoscópica foram incluídos. Uma análise de subgrupos post-hoc, com base na extensão da cirurgia, foi realizada para os outcomes tempo intra-operatório, perdas de sangue e tempo de hospitalização. RESULTADOS: Nove estudos cumpriram os critérios de elegibilidade, tendo sido incluídos 246 pacientes submetidos a cirurgia por via laparoscópica e 301 pacientes submetidos a cirurgia aberta. A abordagem laparoscópica apresentou perdas de sangue intra-operatórias (p=0,01) e tempos até alimentação oral (p<0,01), para primeiro flato (p<0,01) e de hospitalização (0,01) estatisticamente inferiores relativamente à abordagem por cirurgia aberta. Não foram encontradas diferenças significativas quando avaliados o tempo operatório (0,25), complicações pós-operatórias (0,08), resseção R0 (0,76) e taxa de recorrência (0,09). A análise de subgrupos comparativa não permitiu explicar a heterogeneidade substancial obtida nos respetivos outcomes. CONCLUSÕES: A via laparoscópica em tumores estromais gástricos superiores a 5 centímetros comparativamente com a abordagem por cirurgia aberta, constitui um método cirúrgico tecnicamente seguro e viável, com resultados oncológicos semelhantes.

Nódulo del estroma endometrial: Reporte de un caso infrecuente / Endometrial stromal nodule: A rare case report

Introducción: Los tumores del estroma endometrial representan menos del 2% de los tumores uterinos, estando dentro de las neoplasias menos comunes del cuerpo uterino. Se pueden dividir en cuatro categorías principales: nódulo del estroma endometrial, sarcoma del estroma endometrial de bajo grado, sarcoma del estroma endometrial de alto grado y sarcoma uterino indiferenciado. En el presente trabajo se describe el caso de un paciente con diagnóstico de nódulo del estroma endometrial. Caso clínico : Paciente femenino de 50 años de edad, quien refiere inicio de enfermedad en marzo de 2022, caracterizado por presentar sangrado uterino anormal anemizante y aumento de volumen abdominal, por lo que acude a facultativo foráneo, donde indican paraclínicos. Para el día 12 de julio de 2022, presentó dolor abdominal de aparición brusca de moderada a severa intensidad. Motivo por el cual acudió a nuestro centro. Se determina anemia y leucocitosis. Estudios de imagen reportan masa voluminosa, densidad mixta, bien delimitada. Otra lesión hiperecogénica, que corresponde a quiste unicameral de ovario derecho. Se decide resolución quirúrgica, mediante la realización de laparotomía exploradora más protocolo de endometrio, con evolución satisfactoria de la paciente. Conclusión : La histerectomía es el tratamiento de elección. El estudio anatomopatológico es fundamental para su diagnóstico final y diferenciación de los sarcomas estromales, ya que su pronóstico, tratamiento y seguimiento es diferente(AU)

Introduction: Endometrial stromal tumors represent less than 2% of uterine tumors, being among the least common neoplasms of the uterine body. They can be divided into four main categories: endometrial stromal nodule, low-grade endometrial stromal sarcoma, high-grade endometrial stromal sarcoma and undifferentiated uterine sarcoma. This paper describes the case of a patient with a diagnosis of endometrial stromal nodule.Clinical case : A 50-year-old female patient, who reported the onset of the disease in March 2022, characterized by abnormal uterine bleeding with anemia and increased abdominal volume, for which she went to a foreign physician, where they indicated paraclinical tests. On July 12, 2022, he presented abdominal pain of sudden onset of moderate to severe intensity. Which is why she came to our center. Anemia and leukocytosis are determined. Imaging studies report a voluminous mass, mixed density, well delimited. Another hyperechoic lesion, which corresponds to a unicameral cyst of the right ovary. Surgical resolution was decided by performing an exploratory laparotomy and endometrial protocol, with satisfactory evolution of the patient.Conclusion : Hysterectomy is the treatment of choice. The anatomopathological study is fundamental for its final diagnosis and differentiation of stromal sarcomas, since its prognosis, treatment and follow-up are different(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)

Acute abdomen secondary to massive bleeding due to rupture of liver metastases of a gastrointestinal stroma tumor.

Hemoperitoneum secondary to non-traumatic liver rupture is a rare cause of an acute abdomen. We present the case of a patient with a hepatic rupture secondary to metastasis of a gastrointestinal stromal tumor (GIST) who required an emergent laparotomy due to hemodynamic instability. Intraoperatively, a lesion of more than 20cm dependent on the posterior gastric wall and a hemoperitoneum secondary to rupture of liver metastases and decapsulation of the Glisson capsule was evidenced. A frequent complication of GIST tumors is its rupture causing gastrointestinal bleeding or hemoperitoneum, as occurred in our case.

El hemoperitoneo secundario a estallido hepático no traumático es una causa poco frecuente de abdomen agudo. Se presenta el caso de una paciente con estallido hepático secundario a metástasis de un tumor del estroma gastrointestinal (GIST) que requirió una laparotomía emergente por inestabilidad hemodinámica. Se evidenció intraoperatoriamente una lesión de más de 20 cm dependiente de la pared posterior gástrica y un hemoperitoneo secundario a estallido de metástasis hepáticas y descapsulación de la cápsula de Glisson. Una complicación frecuente de los GIST es la rotura que ocasiona hemorragia digestiva o hemoperitoneo, como ocurrió en nuestro caso.

Resección laparoendoscópica de tumor de la unión gastroesofágica / Laparoendoscopic resection of a tumor of the gastroesophageal junction

RESUMEN La resección gástrica atípica ha demostrado ser beneficiosa para tumores submucosos. La técnica pre senta mayor riesgo cuando estos se desarrollan próximos a la unión esófago-gástrica (UEG). Para esta limitación se propuso la resección intragástrica mediante una técnica mixta combinando laparoscopia y endoscopia. En nuestro medio no existen publicaciones al respecto. Se trata de una mujer de 42 años, con lesión subepitelial-subcardial de 2 cm, evaluada mediante videoendoscopia alta (VEDA), compa tible con tumor del estroma gastrointestinal (GIST) evaluado mediante ecoendoscopia. La lesión fue resecada mediante abordaje combinado laparoendoscópico. Bajo visión laparoscópica se introdujeron en cavidad abdominal trocares con balón, y bajo visión endoscópica intragástrica se introdujeron estos en el estómago y se fijó la pared gástrica a la pared abdominal insuflando dichos balones. Posterior mente se realizó la resección de la lesión con sutura mecánica. El abordaje combinado es seguro y eficaz, simple en manos entrenadas, pero constituye una opción reproducible en casos seleccionados.

ABSTRACT Atypical gastric resection has proved to be beneficial to treat submucosal tumors. The technique is more difficult when these tumors develop next to the gastroesophageal junction (GEJ). Intragastric resection combining endoscopic and laparoscopic approach was proposed to solve this limitation. There are no publications about this technique in our environment. A 42-year-old female patients with a 2-mm subepithelial tumor below the cardia evaluated by upper gastrointestinal (UGI) videoendoscopy and endoscopic ultrasound suggestive of a gastrointestinal stroma tumor (GIST) underwent resection using the combined laparo-endoscopic approach. Under laparoscopic guidance, balloon-tipped trocars were introduced in the abdominal cavity and then into the stomach using endoscopic view. The balloons were inflated to fix the gastirc wall to the abdominal wall. The lesion was resected using mechanical stapler. The combined approach is safe and efficient, and simple to perform for trained professionals, constituting a reproducible option in selected cases.