Computed tomography (CT) findings in 88 neurofibromatosis 1 (NF1) patients: Prevalence rates and correlations of thoracic findings. Issue 6 (June 2015)

• Record Type: Journal Article
• Authors: Ueda, Ken; Honda, Osamu; Satoh, Yukihisa; Kawai, Misa; Gyobu, Tomoko; Kanazawa, Toru; Hidaka, Shojiro; Yanagawa, Masahiro; Sumikawa, Hiromitsu; Tomiyama, Noriyuki
• Is Part Of: European journal of radiology. Volume 84:Issue 6(2015)
• Page Start: 1191
• Page End: 1195
• Publication Date: 2015-06
• Subjects: Neurofibromatosis 1 (NF1) -- CT -- Lung -- Mediastinum; Medical radiology -- Periodicals; Radiology -- Periodicals; Radiologie médicale -- Périodiques; Medical radiology; Periodicals; 616.075705
• DOI: 10.1016/j.ejrad.2015.02.024
• Languages: English
• ISSNs: 0720-048X

Abstract

Highlights: Various thoracic CT findings, including cysts, mediastinal masses, etc. were found. Cysts show upper and peripheral dominant distribution. The number, size, and distribution of the pulmonary cysts in NF-1 revealed significant correlation. It is suspected that thoracic CT findings in NF-1 occur independently. Abstract: Purpose: To evaluate the prevalence rates and the correlations of thoracic computed tomography (CT) findings of neurofibromatosis 1 (NF1) in 88 patients. Materials and methods: Chest CT images of 88 NF1 patients were independently reviewed by three observers, and the CT findings were evaluated. If abnormal findings were present, their number, size, and distribution were recorded. The prevalence rate of each CT finding was calculated, and the correlations between CT findings were analyzed. Results: Of the 88 cases, 13 were positive for cysts, 16 for emphysema, 8 for nodules, 8 for GGNs (ground glass nodules), 13 for mediastinal masses, 20 for scoliosis, 44 for subcutaneous nodules, and 34 for skin nodules. Cysts showed upper and peripheral dominant distributions. Regarding 13 mediastinal masses, 2 were diagnosed as malignant peripheral nerve sheath tumors (MPNSTs), 1 was diagnosed as primary lung cancer, 2 were diagnosed as lateral meningocele, 3 were diagnosed as neurofibromas, and the remaining 7 were considered neurofibromas. There was a significant correlation between the prevalence of subcutaneous nodules and that of skin nodules. Significant positive correlations were also seen between size and number, size and rate of central distribution, and number and rate of central distribution of cysts. Conclusion: Various CT findings were found in NF-1 patients, and the prevalence rates of subcutaneous and skin nodules were higher than other findings. Though the prevalence rates of subcutaneous nodules and skin nodules were significantly correlated, the other CT findings in NF-1 occurred independently. The number, size, and distribution of the cysts showed significant positive correlations with each other.