Neurofibromatosis type 1 associated to peripheral nerve sheath and colon malignant tumor

• Autores: Valle, Rubén, Valencia, Víctor, Huamán-Muñante, José
• Formato: artículo
• Fecha de Publicación: 2009
• Institución: Universidad Nacional Mayor de San Marcos
• Repositorio: Revistas - Universidad Nacional Mayor de San Marcos
• Lenguaje: español
• OAI Identifier: oai:ojs.csi.unmsm:article/942
• Enlace del recurso: https://revistasinvestigacion.unmsm.edu.pe/index.php/anales/article/view/942
• Nivel de acceso: acceso abierto
• Materia: Neurofibromatosis 1; neurofibroma; sarcoma; neoplasias; colon.; neoplasms

Neurofibromatosis type 1 is a dominant autosomal disease caused by mutation of the neurofibrin gene located in chromosome 17q11.2. This disease presents a variety of clinical manifestations and predisposition to develop tumors, leading to high mortality. We report the case of a patient with neurofibromatosis type 1 diagnosis who developed a malignant tumor of peripheral nerve sheath and colon cancer. The authors review the literature as well as the clinical factors, imaging and histological features associated to neurofibromas malignant transformation.