CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions

Neurology. 1998 Jun;50(6):1755-60. doi: 10.1212/wnl.50.6.1755.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abdominal Neoplasms / diagnostic imaging
  • Abdominal Neoplasms / epidemiology
  • Adult
  • Cohort Studies
  • Humans
  • Incidence
  • Middle Aged
  • Neoplasms, Multiple Primary / diagnostic imaging
  • Nerve Sheath Neoplasms / diagnostic imaging
  • Nerve Sheath Neoplasms / epidemiology
  • Neurofibromatosis 1 / diagnostic imaging*
  • Neurofibromatosis 1 / epidemiology
  • Pelvic Neoplasms / diagnostic imaging
  • Pelvic Neoplasms / epidemiology
  • Radiography, Thoracic*
  • Thoracic Neoplasms / diagnostic imaging
  • Thoracic Neoplasms / epidemiology
  • Tomography, X-Ray Computed*