RESUMO
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.
Assuntos
Hospitais Universitários , Neoplasias Intestinais , Intestino Delgado , Humanos , Pessoa de Meia-Idade , Masculino , Feminino , Estudos Retrospectivos , Chile/epidemiologia , Hospitais Universitários/estatística & dados numéricos , Prognóstico , Idoso , Neoplasias Intestinais/epidemiologia , Neoplasias Intestinais/patologia , Neoplasias Intestinais/diagnóstico , Intestino Delgado/patologia , Adulto , Tumores do Estroma Gastrointestinal/epidemiologia , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/diagnóstico , Idoso de 80 Anos ou mais , Taxa de Sobrevida , Tumores Neuroendócrinos/epidemiologia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/patologia , Adenocarcinoma/epidemiologia , Adenocarcinoma/diagnóstico , Adenocarcinoma/patologia , Adulto Jovem , Linfoma/epidemiologia , Linfoma/diagnóstico , Linfoma/patologiaRESUMO
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).
Assuntos
Tumores do Estroma Gastrointestinal , Laparoscopia , Neurofibromatose 1 , Masculino , Humanos , Adulto , Neurofibromatose 1/complicações , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/cirurgia , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/diagnóstico , Jejuno , Endoscopia Gastrointestinal/métodos , Tumores do Estroma Gastrointestinal/complicações , Tumores do Estroma Gastrointestinal/diagnósticoRESUMO
Gastrointestinal stromal tumor (GIST) is the most common malignant neoplasm of mesenchymal origin, and a paradigmatic model for a successful rational development of targeted therapies in cancer. The introduction of tyrosine kinase inhibitors with activity against KIT/PDGFRA in both localized and advanced stages has remarkably improved the survival in a disease formerly deemed resistant to all systemic therapies. These guidelines are elaborated by the conjoint effort of the Spanish Society of Medical Oncology (SEOM) and the Spanish Sarcoma Research Group (GEIS) and provide a multidisciplinary and updated consensus for the diagnosis and treatment of GIST patients. We strongly encourage that the managing of these patients should be performed within multidisciplinary teams in reference centers.
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Tumores do Estroma Gastrointestinal , Sarcoma , Humanos , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/terapia , Oncologia , Consenso , Receptores Proteína Tirosina QuinasesRESUMO
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.
Assuntos
Fibroma , Tumores do Estroma Gastrointestinal , Neoplasias Gástricas , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/patologia , Biomarcadores Tumorais , Tumores do Estroma Gastrointestinal/diagnóstico , Fibroma/diagnóstico , Fibroma/patologiaRESUMO
Resumen Introducción Los tumores del estroma gastrointestinal (GIST) corresponden al 1% de todas las neoplasias gastrointestinales, sin embargo, sólo el 3-5% de estos se desarrollan en el duodeno. Objetivo Reportar el caso de un paciente masculino con localización atípica de un tumor de estroma gastrointestinal y su manejo. Caso clínico paciente masculino de 50 años con antecedente de traumatismo encefalo craneano (TEC) con daño orgánico cerebral secundario, tabaquismo, consumidor de alcohol ocasional y sometido a quistectomía branquial en la infancia, que consulta en el servicio de urgencias por cuadro de hemorragia digestiva alta con compromiso hemodinámico. Tras realizar endoscopia digestiva alta (EDA), resonancia nuclear magnética (RNM) y tomografía computada (TC) de abdomen, se pesquisa masa tumoral en segunda porción de duodenal. Discusión A pesar de que la presentación clínica de los GIST es variable, lo más frecuente es que sean pacientes asintomáticos. En algunas ocasiones, al igual que en este reporte, pueden presentarse con dolor abdominal y/o hemorragia digestiva alta. El diagnóstico preoperatorio fue difícil ya que el estudio con imágenes (TC, RNM, EDA) sólo permite establecer la sospecha; el diagnóstico definitivo se realizó con biopsia (no contamos con endosonografía en nuestro centro). Debido a los sitios de reparo anatómico, no existe una cirugía estandarizada; en este caso, debido a la localización, infiltración y características, se decidió realizar una pancreatoduodenectomía.
Introduction Gastrointestinal stromal tumors (GIST), corresponds to 1%, of all gastrointestinal neoplasms, however, only 3%-5% developed in duodenum. Aim To report a case of a male patient with atypical location of gastrointestinal stroma tumor and the treatment proposed. Case report 50-year-old male patient, with medical history of organic brain damage secondary a traumatic brain injury, smoker, occasional alcohol consumer and branquial cystomy during childhood. Consulted in the emergency department for a high digestive hemorrhage case with hemodynamic compromise. Upper digestive endoscopy, computed tomography and nuclear magnetic resonance were performed, which impresses tumor-like lesion in the second duodenal portion. Discussion Although the GIST clinical presentation is variable, most often they are asymptomatic patients. In some times, as in this report, they may present with abdominal pain and/or upper gastrointestinal bleeding. The preoperative diagnosis was difficult, because the imaging study (CT, RNM, EDA) only stablished the suspicion and the final diagnosis was made by biopsy (we don't have endosonography in our center). Due to the anatomic repair, there is not a standardized surgery, in this case, due tumor location, infiltration and characteristics, it was decided to perform a pancreatoduodenectomy.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Tumores do Estroma Gastrointestinal/diagnóstico , Neoplasias Gastrointestinais/patologia , Hemorragia Gastrointestinal/etiologia , Tomografia Computadorizada por Raios X , Tumores do Estroma Gastrointestinal/complicações , Tumores do Estroma Gastrointestinal/patologia , Hemorragia Gastrointestinal/complicaçõesRESUMO
Introducción: El tumor estromal gastrointestinal es la neoplasia mesenquimal más frecuente en el tracto digestivo, su diagnóstico y tratamiento aun es controvertido por ser infrecuente. Objetivo: Caracterizar los pacientes con tumores estromales gastrointestinales atendidos en nuestro servicio. Métodos: Se realizó un estudio observacional descriptivo longitudinal en pacientes con tumores estromales gastrointestinales atendidos en el servicio de cirugía del Hospital Clínico-Quirúrgico "Arnaldo Milián Castro" desde 2015 hasta 2018. La muestra fue de 17 pacientes. Resultados: Predominaron los pacientes entre 60 y 69 años (35,3 por ciento) del sexo femenino (58,82 por ciento). El dolor abdominal como síntoma más frecuente (58,82 por ciento). El (47,1 por ciento) de los tumores midieron más de 10 cm, celularidad fusiforme (58,8 por ciento), índice mitótico menor de 5 (70,6 por ciento), sin patrón de crecimiento infiltrante (70,6 por ciento). La metástasis ausente en el (82,4 por ciento). Presente con igual frecuencia en estómago e intestino delgado (N = 8), fue igual la presencia de comportamiento agresivo bajo y alto (35,3 por ciento). Predominaron los marcadores CD.117 (41,2 por ciento) y CD.34 (35,3 por ciento). La recesión segmentaria de intestino delgado con anastomosis término-terminal fue la técnica quirúrgica más empleada (35,3 por ciento). Conclusiones: A pesar de ser mayormente grandes y encontrarse en porciones altas del tubo digestivo, muchos presentaron índice mitótico bajo, no obstante, un grupo considerable presentó comportamiento agresivo. Si bien es cierto que casi la totalidad de pacientes egresaron vivos, falta un seguimiento en el centro, lo cual resultaría interesante evaluar en futuros estudios(AU)
Introduction: Gastrointestinal stromal tumor is the most frequent mesenchymal neoplasm in the digestive tract, its diagnosis and treatment is still controversial because it is infrequent. Objective: To characterize the patients with gastrointestinal stromal tumors treated in our service. Methods: A longitudinal descriptive observational study was carried out in patients with gastrointestinal stromal tumors treated in the surgery service of the "Arnaldo Milián Castro" Clinical-Surgical Hospital from 2015 to 2018. The sample consisted of 17 patients. Results: Female patients between 60 and 69 years old (35.3 percent) predominated (58.82 percent). Abdominal pain as the most frequent symptom (58.82 percent). Tumors (47.1 percent) measured more than 10 cm, spindle cell cellularity (58.8 percent), mitotic index less than 5 (70.6 percent), and no infiltrative growth pattern (70.6 percent). Metastasis absent in (82.4 percent). Present with equal frequency in the stomach and small intestine (N = 8), the presence of low and high aggressive behavior (35.3 percent) was the same. The markers CD.117 (41.2 percent) and CD.34 (35.3 percent) predominated. Segmental recession of the small intestine with end-to-end anastomosis was the most widely used surgical technique (35.3 percent). Conclusions: Despite being mostly large and found in high portions of the digestive tract, many presented a low mitotic index, however, a considerable group presented aggressive behavior. Although it is true that almost all the patients were discharged alive, there is a lack of follow-up at the center, which would be interesting to evaluate in future studies(AU)
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Dor Abdominal , Assistência ao Convalescente , Tumores do Estroma Gastrointestinal/diagnóstico , Neoplasias Gastrointestinais/terapia , Epidemiologia Descritiva , Estudos Longitudinais , Estudos Observacionais como AssuntoRESUMO
INTRODUCTION: Primary tumors of the small intestine (PTID) represent approximately 5% of all primary gastrointestinal neoplasms; the latter include benign and malignant lesions, with different histological subtypes. OBJECTIVE: To describe the clinical-pathological characteristics and the management of tumors located in the jejunum-ileum. MATERIALS AND METHODS: A descriptive, retrospective study was carried out in a single center. RESULTS: 45 patients were included, the average age at diagnosis was 54.2 ± 8.2 years. 27 were male (60%). In the diagnostic algorithm, computed tomography was used in all patients, double-balloon enteroscopy in 41 (91.1%) and video capsule endoscopy in 32 (71.1%). Endoscopic procedures such as: biopsies, tattoos, resection and dilation were performed in 40 (88.9%), 39 (86.7%), 4 (8.9%) and 1 (2.2%) patients, respectively. The most frequent location was the jejunum in 39 (86%). GIST was confirmed in 18 (40%), followed by lymphoma in 16 (35.6%) and adenocarcinoma in 5 (11%) cases. All GIST, adenocarcinoma, and neuroendocrine tumors underwent surgical treatment and chemotherapy; treatment of lymphomas consisted mainly of combined treatment; three harmartomas and one fibroangiolipoma were resected endoscopically. CONCLUSIONS: The most frequent jejunoileal small intestine tumors were GISTs, followed by lymphomas and adenocarcinomas. Double-balloon enteroscopy was the main diagnostic and therapeutic tool.