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.

[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.

Hodgkin's disease of the gastrointestinal tract.

Hodgkin's disease (HD) is rarely considered in the differential diagnosis of gastrointestinal (GI) disorders. We report eight cases of GI HD. The patients, ages 16-64, all with stage IV disease of varied histologic subtypes and GI symptoms including pain, upper or lower GI bleeding and/or obstruction. There was diffuse gastric fold thickening in one case, a large submucosal mass along the proximal greater curvature in another case, and a large ulcerating mass along the lesser curvature in the third case. Two cases of jejunal disease manifested as either a short or long segment of luminal narrowing with prominent mucosal nodularity. Another case exhibited a short area of complete mucosal destruction, irregular cavitation and intraluminal filling defect. In one additional case there was diffuse jejunal nodularity. Colonic involvement (two cases) presented as a solitary polypoid mass in transverse colon in one patient and severe narrowing of the descending colon with adjacent mesenteric mass on CT in another. We conclude that although rare in Hodgkin's disease, GI involvement should be considered, particularly in patients with stage IV disease.

Linfomas del yeyuno-ileon / Jejunal-ileum lymphomas

Se presentaron 20 linfomas de intestino delgado, experiencia del período comprendido entre los años 1956 y 1990 de la práctica hospitalaria y privada. Se hacen consideraciones clínico-diagnósticas, destacándose su tardía forma de presentación. Se describen las formas comunes del diagnóstico en esta patología a tavés de sus complicaciones, obstrucción, hemorragía, perforación. Se destacan dos épocas: la primera, patrimonio exclusivo de la cirugía y la actual con el complemento de la terapía adyuvante, las que permiten mejorar xonsiderablemente los resultados. El tratamiento quirúrgico quedó polarizado entre la resección y la hemicolestomía derecha. Se describen las formas anatomopatológicas halladas, las que variaron desde las más benignas (linfocítico bien diferenciado) hasta las de pronóstico más reservado (histiocíticodifuso)

[Leiomyosarcoma of the small intestine. Contribution of computerized tomography to its diagnosis]. / Leiomiosarcoma del intestino delbado. Contribudión de la tomografía computada para su diagnóstico.

The case of a patient with a small bowel leiomyosarcoma in whom a correct preoperative diagnosis was done due to important clues provided by computed tomography is presented. We present these findings, and suggest that this new exploratory method may be useful for the study of patients with small bowel intramural tumors, in whom other studies are often non-conclusive.