1. 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
- Author
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Tomaz de Jesus Maria Grezzana-Filho, Taís Burmann de Mendonça, Liane Golbspan, Cleber Rosito Pinto Kruel, Aljamir Duarte Chedid, and Cleber Dario Pinto Kruel
- Subjects
Neurofibromatose ,Tumor estromal gastrointestinal ,Abdome agudo ,Neurofibromatosis ,Gastrointestinal stromal tumors ,Acute abdomen ,Surgery ,RD1-811 ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Abstract
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 (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 (
- Published
- 2009
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