CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions.
Neurology
; 50(6): 1755-60, 1998 Jun.
Article
em En
| MEDLINE
| ID: mdl-9633723
ABSTRACT
OBJECTIVE:
The authors examined the incidence and radiologic characteristics of plexiform neurofibromas in neurofibromatosis-1 (NF-1) to define a cohort at greatest risk for malignant nerve-sheath tumors.BACKGROUND:
Plexiform neurofibromas are a frequent complication of NF-1. They can impair function, produce disfigurement, and be the site for the development of malignant nerve-sheath tumors. The incidence and natural history of plexiform neurofibromas is unknown.METHODS:
CT imaging of the chest, abdomen, and pelvis was performed in 91 of 125 consecutive adults (age, > or = 16 years) with NF-1.RESULTS:
Twenty percent of patients had plexiform neurofibromas of the chest in the paraspinal, mediastinal, or supraclavicular area. Approximately 40% of patients had abnormal abdominal/pelvic scans. The paraspinal, sacral plexus, sciatic notch, and perirectal regions were the most common sites. Most plexiform neurofibromas were asymptomatic. Imaging also revealed a number of tumors, including malignant nerve-sheath tumors, adrenal tumors, carcinoids, and schwannomas.CONCLUSIONS:
The frequency of plexiform lesions and other tumors in NF-1 indicates that clinicians should monitor young adults carefully; however, imaging characteristics alone cannot reliably distinguish benign from malignant lesions.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Radiografia Torácica
/
Tomografia Computadorizada por Raios X
/
Neurofibromatose 1
Tipo de estudo:
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Prognostic_studies
Limite:
Adult
/
Humans
/
Middle aged
Idioma:
En
Ano de publicação:
1998
Tipo de documento:
Article