Characteristics and prognosis of jejunoileal gastrointestinal stromal tumours (GISTs) in the era of imatinib: a comparative study with gastric GISTs.

BACKGROUND: Gastrointestinal stromal tumours (GISTs) located in the jejunum or ileum (JI-GIST) are considered worse prognosis compared to those of gastric (G-GIST) location. It has been suggested that this dogma should be revised. The aim of this study was to describe the characteristics of jejunoileal GISTs and its prognosis and to compare them with G-GISTs in the era of imatinib. METHODS: We retrospectively reviewed the clinical histories of all the patients diagnosed with GISTs between January 2000 and November 2016: Clinical and pathological data, as recurrence, metastatic state, disease-free survival (DFS) as well as overall survival (OS) rates of patients were reviewed. RESULTS: JI-GIST patients comprise 29 cases (37.7%). Compared to G-GIST, JI-GIST patients had undergone emergency surgery more frequently (37.9% vs. 10.4%, p = 0.007). According to the NIH-Fletcher classification, the low or very-low risk group represents 17.2% of JI-GISTs as opposed to 37.6% of G-GISTs (p < 0.005). When the AFIP-Miettinen system was used the low or very-low group represented 17.2% of JI-GISTs vs. 58.4% in the G-GISTs group (p < 0.001). Both local recurrence (24.1% vs. 12.5%, p < 0.05) and metastatic rate (34.5% vs. 22.9%, p < 0.05) were higher in the JI-GIST group than in G-GIST. 5- and 10-year DFS and 10-year OS rate were lower for JI-GIST (54.5% and 39.6% vs. 77.2% and 60.8%, and 57.9% vs. 65%, respectively, p < 0.05). CONCLUSIONS: The observed differences between both groups in DFS and OS rates at long term could be attributed to the effect of imatinib.

Valor de la tomografía abdominal para el diagnóstico del fibroleiosarcoma yeyunal / Abdominal tomography importance on the Leiomyosarcoma of jejunum diagnosis

RESUMEN Se presentó el caso de un paciente de 49 años de edad, que acudió a la consulta de Medicina Interna del Hospital General Docente "Dr. Agostinho Neto" por perder unas 20 libras de peso en dos meses y presentar heces fecales con aspecto aparente a "borra de café". El examen físico reveló desnutrición ligera y mucosas hipocoloreas. El ultrasonido abdominal mostró en hipocondrio y flanco izquierdo una imagen hipoecogénica heterogénea polilobulada que midió 68x61 mm con aspecto de paquete adenomegálico. En la tomografía computarizada de abdomen se identificó una imagen con densidad entre 33 y 57 UH, de 100 x 80 mm, polilobulada en topografía del yeyuno, que con el estudio contrastado realzó hasta 67 UH con aspecto tumoral. Se realizó resección quirúrgica del tumor con anastomosis termino-terminal. La biopsia informó fibroleiomiosarcoma. Se concluye que el estudio de las imágenes resulta útil para el diagnóstico de esta enfermedad.

ABSTRACT A 49-year-old patient presented to the internal medicine consultation office at the "Dr. Agostinho Neto" General teaching Hospital because of weight loss (20 pounds) and black stools in a period of 2 months before the evaluation. The physical examination revealed mild malnutrition and hypochlorous mucous membranes. The abdominal ultrasound image in the left hypochondrium showed a heterogeneous - polylobate - hypoechogenic mass measuring 68 x 61 mm with appearance of an adenomelagia. Computed tomography (CT) scan of the abdomen revealed an image with a density of 33 and 57 Hounsfield units (HU), measured 100 mm by 80 mm, polylobulated in jejunum topography, which with the contrasted study enhanced up to 67 UH with a tumor appearance. Surgical resection of the tumor with termino-terminal anastomosis was performed. Tumor biopsy diagnosed leiomyosarcoma. It was concluded that the study of the images was useful for the diagnosis of this disease.

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

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

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

Adenocarcinoma de yeyuno: infrecuente cáncer gastrointestinal / Adenocarcinoma of jejunum: an infrequent type of intestinal cancer

Los tumores de intestino delgado son infrecuentes, generalmente se presentan con cuadros clínicos inespecíficos y la mayoría de los diagnósticos se hacen en etapas avanzadas de la enfermedad. Se presenta un paciente de sexo masculino de 58 años de edad, con dolor abdominal generalizado, intermitente, vómitos ocasionales, pérdida de peso y llenura posprandial, de dos meses de evolución. Ingresó con manifestaciones clínicas de obstrucción intestinal, y en una tomografía abdómino-pélvica se observó disminución abrupta del calibre en el yeyuno proximal. Se le practicó una laparotomía en la que se evidenció una lesión obstructiva del yeyuno que se resecó y se anastomosó el segmento. En la histopatología se reportó adenocarcinoma de yeyuno moderadamente diferenciado T3N0M0. Presentó una evolución favorable

Small bowel tumors are infrequent. Adenocarcinoma of the jejunum constitutes a rare diagnosis. It usually presents with nonspecific clinical symptoms and most patients are diagnosed in advanced stages of the disease. We present the case of a 58-year-old man with a diagnosis of jejunal adenocarcinoma, intermittent generalized abdominal pain, occasional vomiting, weight loss and postprandial fullness of two months of evolution. The patient was admitted with intestinal obstruction, with an abdominopelvic tomography showing abrupt caliber reduction at the level of the proximal jejunum. A laparotomy was performed, where an obstructive lesion was evidenced in the jejunum; resection was performed primary anastomosis. Histopathology reported moderately differentiated adenocarcinoma T3N0M0. The patient has had a favorable evolution

[Obscure gastrointestinal bleeding due to gastrointestinal stromal tumors]. / Hemorragia gastrointestinal de origen oscuro por tumores de estroma gastrointestinal.

BACKGROUND: The gastrointestinal stromal tumours (GIST) are the most common soft tissue sarcomas of the digestive tract. They are usually found in the stomach (60-70%) and small intestine (25-30%) and, less commonly, in the oesophagus, mesentery, colon, or rectum. The symptoms present at diagnosis are, gastrointestinal bleeding, abdominal pain, abdominal mass, or intestinal obstruction. The type of symptomatology will depend on the location and size of the tumour. The definitive diagnosis is histopathological, with 95% of the tumours being positive for CD117. CLINICAL CASES: This is an observational and descriptive study of 5cases of small intestinal GIST that presented with gastrointestinal bleeding as the main symptom. The period from the initial symptom to the diagnosis varied from 1 to 84 months. The endoscopy was inconclusive in all of the patients, and the diagnosis was made using computed tomography and angiography. Treatment included resection in all patients. The histopathological results are also described. CONCLUSION: GIST can have multiple clinical pictures and unusual symptoms, such as obscure gastrointestinal bleeding. The use of computed tomography and angiography has shown to be an important tool in the diagnosis with patients with small intestine GISTs.

[Jejunal perforation secondary to pulmonary mucoepidermoid carcinoma metastasis. Case report and review]. / Perforación yeyunal secundaria a metástasis de carcinoma mucoepidermoide pulmonar. Reporte de caso y revisión de la literatura.

BACKGROUND: The first reported case of intestinal perforation secondary to metastatic lung carcinoma was reported in 1957. Intestinal metastases are present in up to 1.8% of the cases, with small bowel obstruction as the most common clinical presentation. CLINICAL CASE: An 89 year-old male, who was diagnosed with a high-grade pulmonary mucoepidermoid tumour 2 months previously. The patient was admitted to the hospital for 3 days due to diffuse colic abdominal pain of moderate to severe intensity, accompanied by nausea and gastric vomiting, as well as 2 episodes of bloody bowel movements. On physical examination, the patient was noted to have tachycardia and tachypnoea, as well as clinical signs of acute abdomen. He had white cells of 24,900 per mm3, and 87% neutrophils. Exploratory laparotomy was performed, which showed a bowel perforation associated with a tumour mass 15cm beyond the angle of Treitz. Bowel resection and primary anastomosis were performed. The histopathological analysis reported the diagnosis of a high-grade mucoepidermoid tumour with small bowel and mesentery with disease-free surgical margins. Unfortunately the patient had a fatal outcome secondary to hospital-acquired pneumonia. CONCLUSION: The cases of metastases to small bowel are extremely rare, and to our knowledge this is first case reported in Mexico. The patient described went to the emergency room with gastrointestinal bleed and intestinal perforation that required urgent surgical intervention with small bowel resection and primary anastomosis. Unfortunately the patient died secondary to hospital acquired pneumonia.

Leiomioma intestinal em um equino: relato de caso / Intestinal leiomyoma in a horse: case report / Leiomioma intestinal en un caballo: estudio de caso

Elleiomioma es un tumor benigno originado a partir de células de músculo liso. La mayoría de tumores benignos dei intestino delgado no muestran síntomas y suelen ser hallazgos incidentales durante procedimientos como Ia laparotomía exploratoria o necropsia. Sin embargo, dependiendo dei tamaõo dei tudisminución de lumen asa intestinal puede conducir a signos clínicos tales como cólico y compresión. EI pestudio reporta un caso de un caballo pérdida de peso presentando cuatro anos de edad, Ia fatiga después ejercicio y calambres recurrentes. Los ensayos identificados fue una masa dura, redonda en Ia región dei fiizquierdo de aspecto ecográfico heterogéneo. Laparotomía exploratoria se realizó revelando una masa tuque invade ellumen dei yeyuno dei mango y el plexo vascular mesentérica. Esta masa se eliminó a través de enterectomía y se mantuvo citológico e histológico diagnosticar un leiomioma. Oespués de 19 días tue dadoalta el paciente y el propietario informó el aumento de peso y un mejor rendimiento.

Leiomyoma is a benign tumor originating from smooth muscle cells. Most small intestinetumors don't show clinical signs and usually are incidental findings during procedures like exploratoryor necropsy. However, depending on the size of the tumor, the reduced lumen of intestinal loop mayclinical signs of colic and impactation. This present paper reports a case of a four year old equine thatprogressive weight loss, post exercise fatigue and recurrent colic. Through examination, a rigid roundheterogeneous ultrassonographic appearance mass was found in the left flank. An exploratory celiomperformed revealing a mass tumor located in jejunum invading the lumen and the mesenteric vascularThis mass was removed through enterectomy and held cytological and histopathological exams dignleiomyoma. After 19 days the pacient was discharged and the owner reported weight gain and pertoimprovement.

Leiomioma é um tumor benigno originário de células do músculo liso. A maioria dos tumores benignos de intestino delgado não demonstram sintomas e geralmente são achados acidentais durante outros procedimentos como celiotomia exploratória ou necrópsia. Porém, dependendo do tamanho do tumor a diminuição do lúmen da alça intestinal pode levar a sinais de abdômen agudo compatíveis com afecções não estranguladas do intestino dos equinos. O presente trabalho relata o caso de um equino de quatro anos de idade que apresentava emagrecimento progressivo, fadiga pós-exercício e cólicas recidivantes. Após palpação retal e exame ultrassonográfico foi identificada uma massa rígida e arredondada em região do flanco esquerdo. Foi realizada celiotomia exploratória revelando uma massa tumoral em jejuno invadindo o lúmen da alça e o plexo vascular mesentérico. Essa massa foi removida através de uma enterectomia e realizou-se exame citológico e histopatológico diagnosticando um leiomioma. Após 19 dias o paciente recebeu alta e proprietário relatou ganho de peso e melhora na performance após a alta médica.

Leiomioma intestinal em um equino: relato de caso / Intestinal leiomyoma in a horse: case report / Leiomioma intestinal en un caballo: estudio de caso

Elleiomioma es un tumor benigno originado a partir de células de músculo liso. La mayoría de tumores benignos dei intestino delgado no muestran síntomas y suelen ser hallazgos incidentales durante procedimientos como Ia laparotomía exploratoria o necropsia. Sin embargo, dependiendo dei tamaõo dei tudisminución de lumen asa intestinal puede conducir a signos clínicos tales como cólico y compresión. EI pestudio reporta un caso de un caballo pérdida de peso presentando cuatro anos de edad, Ia fatiga después ejercicio y calambres recurrentes. Los ensayos identificados fue una masa dura, redonda en Ia región dei fiizquierdo de aspecto ecográfico heterogéneo. Laparotomía exploratoria se realizó revelando una masa tuque invade ellumen dei yeyuno dei mango y el plexo vascular mesentérica. Esta masa se eliminó a través de enterectomía y se mantuvo citológico e histológico diagnosticar un leiomioma. Oespués de 19 días tue dadoalta el paciente y el propietario informó el aumento de peso y un mejor rendimiento.(AU)

Leiomyoma is a benign tumor originating from smooth muscle cells. Most small intestinetumors don't show clinical signs and usually are incidental findings during procedures like exploratoryor necropsy. However, depending on the size of the tumor, the reduced lumen of intestinal loop mayclinical signs of colic and impactation. This present paper reports a case of a four year old equine thatprogressive weight loss, post exercise fatigue and recurrent colic. Through examination, a rigid roundheterogeneous ultrassonographic appearance mass was found in the left flank. An exploratory celiomperformed revealing a mass tumor located in jejunum invading the lumen and the mesenteric vascularThis mass was removed through enterectomy and held cytological and histopathological exams dignleiomyoma. After 19 days the pacient was discharged and the owner reported weight gain and pertoimprovement.(AU)

Leiomioma é um tumor benigno originário de células do músculo liso. A maioria dos tumores benignos de intestino delgado não demonstram sintomas e geralmente são achados acidentais durante outros procedimentos como celiotomia exploratória ou necrópsia. Porém, dependendo do tamanho do tumor a diminuição do lúmen da alça intestinal pode levar a sinais de abdômen agudo compatíveis com afecções não estranguladas do intestino dos equinos. O presente trabalho relata o caso de um equino de quatro anos de idade que apresentava emagrecimento progressivo, fadiga pós-exercício e cólicas recidivantes. Após palpação retal e exame ultrassonográfico foi identificada uma massa rígida e arredondada em região do flanco esquerdo. Foi realizada celiotomia exploratória revelando uma massa tumoral em jejuno invadindo o lúmen da alça e o plexo vascular mesentérico. Essa massa foi removida através de uma enterectomia e realizou-se exame citológico e histopatológico diagnosticando um leiomioma. Após 19 dias o paciente recebeu alta e proprietário relatou ganho de peso e melhora na performance após a alta médica.(AU)

Most small bowel cancers are revealed by a complication.

OBJECTIVE: To characterize the pattern of primary small bowel cancers in a tertiary East-European hospital. METHODS: A retrospective study of patients with small bowel cancers admitted to a tertiary emergency center, over the past 15 years. RESULTS: There were 57 patients with small bowel cancer, representing 0.039% of admissions and 0.059% of laparotomies. There were 37 (64.9%) men, mean age of 58 years; and 72 years for females. Out of 57 patients, 48 (84.2%) were admitted due to an emergency situation: obstruction in 21 (38.9%), perforation in 17 (31.5%), upper gastrointestinal bleeding in 8 (14.8%), and lower gastrointestinal bleeding in 2 (3.7%). There were 10 (17.5%) duodenal tumors, 21 (36.8%) jejunal tumors and 26 (45.6%) ileal tumors. The most frequent neoplasms were gastrointestinal stromal tumor in 24 patients (42.1%), adenocarcinoma in 19 (33.3%), lymphoma in 8 (14%), and carcinoids in 2 (3.5%). The prevalence of duodenal adenocarcinoma was 14.55 times greater than that of the small bowel, and the prevalence of duodenal stromal tumors was 1.818 time greater than that of the small bowel. Obstruction was the complication in adenocarcinoma in 57.9% of cases, and perforation was the major local complication (47.8%) in stromal tumors. CONCLUSION: Primary small bowel cancers are usually diagnosed at advanced stages, and revealed by a local complication of the tumor. Their surgical management in emergency setting is associated to significant morbidity and mortality rates.

Most small bowel cancers are revealed by a complication / A maioria dos cânceres de intestino delgado são revelados por uma complicação

ABSTRACT Objective To characterize the pattern of primary small bowel cancers in a tertiary East-European hospital. Methods A retrospective study of patients with small bowel cancers admitted to a tertiary emergency center, over the past 15 years. Results There were 57 patients with small bowel cancer, representing 0.039% of admissions and 0.059% of laparotomies. There were 37 (64.9%) men, mean age of 58 years; and 72 years for females. Out of 57 patients, 48 (84.2%) were admitted due to an emergency situation: obstruction in 21 (38.9%), perforation in 17 (31.5%), upper gastrointestinal bleeding in 8 (14.8%), and lower gastrointestinal bleeding in 2 (3.7%). There were 10 (17.5%) duodenal tumors, 21 (36.8%) jejunal tumors and 26 (45.6%) ileal tumors. The most frequent neoplasms were gastrointestinal stromal tumor in 24 patients (42.1%), adenocarcinoma in 19 (33.3%), lymphoma in 8 (14%), and carcinoids in 2 (3.5%). The prevalence of duodenal adenocarcinoma was 14.55 times greater than that of the small bowel, and the prevalence of duodenal stromal tumors was 1.818 time greater than that of the small bowel. Obstruction was the complication in adenocarcinoma in 57.9% of cases, and perforation was the major local complication (47.8%) in stromal tumors. Conclusion Primary small bowel cancers are usually diagnosed at advanced stages, and revealed by a local complication of the tumor. Their surgical management in emergency setting is associated to significant morbidity and mortality rates.

RESUMO Objetivo Caracterizar o padrão de neoplasias malignas primárias do intestino delgado em um hospital terciário de Leste Europeu. Métodos Estudo retrospectivo de pacientes com câncer de intestino delgado, internados em um hospital terciário e de emergência, ao longo dos últimos 15 anos. Resultados Foram avaliados 57 pacientes com neoplasias malignas gastrintestinais, o que representou 0,039% das admissões e 0,059% das laparotomias realizadas. Total de 37 (64,9%) pacientes masculinos, média de idade de 58 anos, e de 72 anos para mulheres. Dentre os 57 pacientes, 48 (84,2%) foram internados em situação de emergência: obstrução intestinal em 21 (38,9%), perfuração em 17 (31,5%), hemorragia digestiva alta em 8 (14,8%), e hemorragia digestiva baixa em 2 (3,7%). Houve 10 (17,5%) tumores duodenais, 21 (36,8%) jejunais e 26 (45,6%) ileais. As neoplasias mais frequentes foram tumor estromal gastrintestinal, em 24 (42,1%) pacientes, adenocarcinoma em 19 (33,3%), linfoma em 8 (14%) e carcinoides em 2 (3,5%). A prevalência de adenocarcinoma duodenal foi 14,55 vezes maior do que a do intestino delgado, e a prevalência de tumores estromais duodenais foi 1,818 vez maior do que a do intestino delgado. A obstrução intestinal foi complicação do adenocarcinoma em 57,9% dos casos, e a perfuração foi a principal complicação local (47,8%) dos tumores estromais. Conclusão As neoplasias malignas primárias do intestino delgado foram geralmente diagnosticadas em estado avançado e reveladas por uma complicação local do tumor. O tratamento cirúrgico em situação de emergência está associado à significativa morbimortalidade.

[Primary non-polipoid intestinal folicular lymphoma: case report and review of the literature]. / Linfoma folicular primario intestinal no polipósico: reporte de un caso y revisión de la literatura.

The primary intestinal follicular lymphoma is a rare disease described in the last classification of lymphomas from WHO. It is a localized disease with excellent prognosis. We describe in this article ,a 64 year-old Peruvian female with abdominal pain and delayed vomiting for the last two years, has undergone a partial intestinal resection due to bowel obstruction. There was a well-circumscribed annular tumor. A diagnosis of non-polypoid primary intestinal follicular lymphoma was made. We report the case and review the literature in this article.

[Laparoscopic removal of primary intestinal fibroid]. / Extirpación laparoscópica de mioma intestinal primario.

Uterine fibroids are the most frequent solid benign tumors of the female pelvis. The most frequent location is the uterine corpus; however, it has been also reported in cervical placement and broad ligament. Rarely it has been reported a case of intestinal fibroid after previous history of myomectomy. We report the case of a patient, with no surgical history of interest, who underwent a diagnostic laparoscopy due to suspicion of malignant abdominal mass. Ajejunal fibroid was found, which was removed via laparoscopy without complications. Laparoscopy may be considered in case of intestinal fibriod despite its rarity, due to the better postoperative recovery and cosmetic outcomes compared to laparotomy.

[Jejunal metastasis from choriocarcinoma. A three-case report]. / Coriocarcinoma metastásico a yeyuno. Reporte de tres casos.

Five per cent of patients presenting with choriocarcinoma develop small bowel metastasis. Tumors of the small bowel are rare and the metastases are generally from lung and breast carcinoma or from melanoma. Clinical presentation is vague and the majority of cases are autopsy findings. The main symptoms are related to the presence of abdominal tumor or hemorrhage, or bowel obstruction or perforation. We present the cases of three patients with small bowel metastasis from choriocarcinoma. A 24-year-old woman with bowel obstruction secondary to intussusception caused by a metastatic choriocarcinoma polypoid mass and two men, one 18 years old and the other 24 years old, with a history of testicular tumor, who presented with gastrointestinal bleeding due to small bowel metastasis from choriocarcinoma, 2 and 10 months after orchiectomy, respectively. Management was endoscopic in one case and surgical in the other two. Two patients died in the early postoperative period and one patient died during the first year of follow-up. Choriocarcinoma metastases are very rare and their main clinical manifestations are hemorrhage and bowel obstruction. Management can be either medical or surgical. The majority of patients with choriocarcinoma respond to chemotherapy but prognosis is worse for those patients presenting with small bowel metastasis.