CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions.
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.
Tonsgard
J H
JH
Department of Pediatrics, The University of Chicago Pritzker School of Medicine, Illinois 60637, USA.
Kwak
S M
SM
Short
M P
MP
Dachman
A H
AH
D000008:Abdominal Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology; D000328:Adult; D015331:Cohort Studies; D006801:Humans; D015994:Incidence; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging; D018317:Nerve Sheath Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging* / Q000453:epidemiology; D010386:Pelvic Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology; D013902:Radiography, Thoracic; D013899:Thoracic Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology; D014057:Tomography, X-Ray Computed