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Open Access GISTs múltiplos em neurofibromatose tipo 1: diagnóstico incidental em paciente com abdome agudo Multiple GISTs in neurofibromatosis type 1: incidental diagnosis in a patient with acute abdomen

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INTRODUÇÃO: Tem sido descrito na literatura incidência aumentada de tumores estromais gastrointestinais (GISTs) em portadores de neurofibromatose tipo 1. Estes tumores tipicamente ocorrem no intestino delgado e, frequentemente, são múltiplos. RELATO DO CASO: Diagnóstico incidental de GIST em um paciente portador de neurofibromatose tipo 1 com abdome agudo. No trans-operatório foi identificada apendicite retrocecal perfurada e massa neoplásica no jejuno proximal. A referida lesão ocupava aproximadamente 70% da circunferência do órgão e não apresentava invasão de estruturas adjacentes. Além disso, observaram-se dezenas de pequenos nódulos disseminados por toda a extensão do jejuno e íleo. O anatomopatológico revelou neoplasia compatível com GIST, com grau moderado de atipias, baixo índice mitótico (<5/50) e ausência de necrose. Na análise imunoistoquímica, as células neoplásicas foram positivas para CD-34 e CD-117 (c-KIT), e negativas para a desmina. Depois da operação, o uso do quimioterápico Imatinib foi indicado. Após período de acompanhamento de 12 meses, o paciente não demonstrou sinais de recidiva. CONCLUSÃO: A ocorrência de GISTs deve ser sempre lembrada na avaliação de pacientes com massas abdominais e neurofibromatose tipo 1, principalmente em intestino delgado. Muitas vezes o comportamento da neoplasia é favorável e o uso de Imatinib deve ser individualizado.
BACKGROUND: The literature described an increased incidence of gastrointestinal stromal tumors (GISTs) in patients with neurofibromatosis type 1. These tumors typically occur in the small intestine, and frequently are multiple. Often the behavior of the tumor in this association is more favorable than in sporadic cases. CASE REPORT: Incidental diagnosis of GIST was done in a patient with neurofibromatosis type 1 during treatment for acute abdomen. Trans-operatively was identified a retrocecal perforated appendicitis and a neoplastic mass in the proximal jejunum. The lesion occupied approximately 70% of the circumference of the organ and had no invasion of adjacent structures. Moreover, there were dozens of small nodules scattered throughout the length of the jejunum and ileum. The pathology revealed malignancy consistent with GIST, with moderate degree of atypia, low mitotic index (<5 / 50) and absence of necrosis. In immunohistochemical analysis, the neoplastic cells were positive for CD-34 and CD-117 (c-KIT), and negative for desmin. After surgery, the use of Imatinib chemotherapy was indicated. After a follow-up period of 12 months, the patient showed no signs of recurrence. CONCLUSION: GISTs should be considered in the presence of abdominal mass and neurofibromatosis type 1, affecting mainly small bowel. Sometimes Imatinib can be administered with good results in the control of the disease.
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Document Type: Research Article

Publication date: January 1, 2009

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