Neurofibromatose plexiforme retroperitoneal e pélvica: aspectos na tomografia computadorizada e ressonância magnética - relato de caso e revisão da literatura

Abstract

Os autores apresentam um caso de neurofibromatose tipo I, caracterizado por múltiplos neurofibromas plexiformes no retroperitônio e pelve. A tomografia computadorizada mostrou lesões marcadamente hipoatenuantes, acometendo principalmente psoas e regiões paravertebrais, aspectos que mimetizavam outras doenças, como abscessos e linfonodomegalias. A ressonância magnética foi útil para a melhor caracterização da extensão dos neurofibromas, bem como de sua estrutura interna.

Repercussões clínicas e radiológicas do neurofibroma plexiforme na região pélvica

“…Clinical signs raising suspicion for malignization include onset of persistent pain, neurological deficit and growth of tumor mass. MRI is currently the best imaging method, allowing the identification of neurofibromas and other lesions associated with the disease and distinguishing PNF from malignant peripheral nerve sheath tumor, since it can better characterize the nature and extent of neurofibromas ( 6 - 9 ) . The relevance of contrast-enhanced MRI and fat saturation techniques is highlighted to differentiate between malignant and benign lesions.…”

“…Additionally, at contrast-enhanced MRI, asymmetrical growth and heterogeneous pattern of a lesion (either due to necrotic areas, hemorrhage, or heterogeneous contrast uptake) indicate malignancy ( 6 , 7 ) . At MRI, PNFs show intermediate to slightly hyperintense signal in relation to muscle on T1-weighted sequences; and on T2-weighted sequences, the lesions are hyperintense and may present hypointense septations ( 9 ) . The presence of findings suggestive of PNF at imaging studies (particularly contrast-enhanced MRI) in patients with NF1 makes biopsy unnecessary to confirm the diagnosis ( 9 ) .…”

“…At MRI, PNFs show intermediate to slightly hyperintense signal in relation to muscle on T1-weighted sequences; and on T2-weighted sequences, the lesions are hyperintense and may present hypointense septations ( 9 ) . The presence of findings suggestive of PNF at imaging studies (particularly contrast-enhanced MRI) in patients with NF1 makes biopsy unnecessary to confirm the diagnosis ( 9 ) . Until the present moment, no significant sign of increase in size of the PNF was observed in the present case.…”

Neurofibroma Plexiforme Vesical em Portador de Neurofibromatose: Relato de caso

“…Ante a suspeita de NFP, a combinação de métodos de imagem (ultrassonografia, tomografia computadorizada e ressonância magnética) é capaz de demonstrá-los [16][17][18] . A ressonância magnética é o método de escolha para o diagnóstico de neurofibromas plexiformes, assim como para distinção da natureza e extensão tumoral 19,20 .…”