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.

[ case report of metastatic choriocarcinoma to jejunum with severe lower GI bleeding]

A 33-year-old female presented with intermittent abdominal pain and severe attacks of lower gastrointestinal [GI] bleeding since 2 weeks. She has had history of irregular menses in last 3 months. In the labeled RBC-Scan study, the bleeding site was detected in left upper quadrant [LUQ]. In surgery, a tumor with the diameter of 2x1cm in a 65 cm distance of ligament of Trietz was detected. In pathological assessment jejunal metstatic choriocarcinoma was reported. Generally, choriocarcinoma is very rare among the intestinal metastatic tumors, including less than 5% of these tumors. Its presentation is acute lower GI bleeding and organ rupture, especially splenic rupture. These tumors will respond appropriately to the treatment in initial stages. Chemotherapy is the treatment of choice in these tumors