RESUMO
Wilkie syndrome is a rare pathology that generates intestinal obstruction due to a decrease of the aortomesenteric angle compromising the third portion of the duodenum. We describe a case of an 18-year-old female patient, diagnosed with Wilkie syndrome, with clinical symptoms of intestinal obstruction and weight loss. The diagnosis was made with abdominal CT. Wilkie syndrome is a rare pathology, which becomes a diagnostic challenge because it presents a similar picture to other more common pathologies. We recommend that it should be suspected in the presence of duodenal obstruction.
Assuntos
Síndrome da Artéria Mesentérica Superior , Humanos , Feminino , Adolescente , Síndrome da Artéria Mesentérica Superior/diagnóstico , Síndrome da Artéria Mesentérica Superior/complicações , Obstrução Intestinal/etiologia , Obstrução Intestinal/diagnóstico , Obstrução Duodenal/etiologia , Obstrução Duodenal/diagnóstico , Tomografia Computadorizada por Raios XRESUMO
RESUMEN El síndrome de Wilkie es una patología poco frecuente que genera un cuadro de obstrucción intestinal, debido a la disminución del ángulo aortomesentérico comprometiendo la tercera porción del duodeno. Describimos el caso de una paciente de 18 años de edad, diagnosticada con síndrome de Wilkie, con cuadro clínico de obstrucción intestinal y pérdida de peso. El diagnóstico se realizó con tomografía abdominal. El síndrome de Wilkie es una patología rara, que se vuelve un reto diagnóstico, por presentar un cuadro similar a otras patologías más comunes. Recomendamos que se sospeche ante un cuadro de obstrucción duodenal.
ABSTRACT Wilkie syndrome is a rare pathology that generates intestinal obstruction due to a decrease of the aortomesenteric angle compromising the third portion of the duodenum. We describe a case of an 18-year-old female patient, diagnosed with Wilkie syndrome, with clinical symptoms of intestinal obstruction and weight loss. The diagnosis was made with abdominal CT. Wilkie syndrome is a rare pathology, which becomes a diagnostic challenge because it presents a similar picture to other more common pathologies. We recommend that it should be suspected in the presence of duodenal obstruction.
RESUMO
RESUMEN El carcinoide atípico (CA) de timo es la neoplasia más agresiva y rara que surge en el mediastino anterior y que pertenece a los tumores primarios neuroendocrinos de timo. La mayoría de los pacientes son asintomáticos y según la extensión de la enfermedad pueden presentar desde tos, disnea, dolor torácico hasta síndrome de vena cava superior. Esta presentación clínica inespecífica disminuye la probabilidad del diagnóstico temprano que, sumado con el estadio avanzado al debut y la imposibilidad de resección quirúrgica reduce la tasa de supervivencia. El objetivo es dar a conocer la presentación clínica, imagenológica y patológica en un varón de 39 años con CA de timo cuyo diagnóstico definitivo se basó en el estudio histopatológico (morfología carcinoide, mitosis 0-1/2 mm2, necrosis, Ki 67 = 12%) y marcadores inmunofenotípicos del tumor (CD 56 (+), Panqueratina (+), Sinaptofisina (+), TTF -1 (-)).
ABSTRACT The atypical carcinoid (AC) of thymus is the most aggressive and uncommon neoplasm in the anterior mediastinum, that belongs to the neuroendocrine primary tumors of thymus. Most patients are asymptomatic and according to the disease extension they may present with cough, dyspnea, chest pain, and superior cava vein syndrome. This non-specific clinical presentation reduces the likelihood for making an early diagnosis; and this, together with disease stage and lack of surgical resection reduces the survival rate. The aim of this paper is to describe the clinical presentation in a 39-year old male with thymus carcinoid whose diagnosis was based on histopathological studies (carcinoid morphology, 0-1/2 mitoses/mm2), necrosis, Ki67, 12%), and immunophenotypic tumor markers (CD56(+), Pankeratin (+), Synaptophysin (+), and TTF-1 (-)).