J Korean Radiol Soc.  1986 Aug;22(4):469-473. 10.3348/jkrs.1986.22.4.469.

The radiologic findings of neurofibromatosis

• Chung CK
• Park KI
• Yoon Y
• Kim SY

• KMID: 2462304
• DOI: http://doi.org/10.3348/jkrs.1986.22.4.469

Abstract

Neurofibromatosis, or von Recklinghausen's disease, is a hereditary, harmartomatous disorder that primarilyinvolves neuroectoderm and mesoderm. The estimated incidence is 1 in 2,500 to 3,000 births. The clinical featuresare skin manifestations such as cafe-au-lait spots, skeletal manifestations primarily in volving vertebrae,central and peripheral nervous manifestations, and other associated abnormalities with increased risk ofmalignancy. The authors analysed the radiologic findings of 18 cases of patients with neurofibromatosis whovisited Pusan Kosin Medical Center and Taegu Dongsan Medical Center during the last five years. All were proven bysurgery, biopsy and other diagnostic criteria. The results obtained were as follows: 1. The male ot female ratiowas 11:7 and the age ranged from 11 months to 51 years. 2. All the cases fulfilled the diagnotic criteria of Croweand associates. 3. Bone manifestations were present in 44% of the cases. The other radiologic findings wereintrathoracic meningocele, bilateral acoustic neurinomas, mediastinal or chest wall mass shadows, and peripheralsoft tissue masses. 4. One of the soft tissue masses was proved to be malignant.

MeSH Terms

Biopsy
Busan
Cafe-au-Lait Spots
Daegu
Female
Humans
Incidence
Male
Meningocele
Mesoderm
Neural Plate
Neurofibromatoses*
Neurofibromatosis 1
Neurofibromatosis 2
Parturition
Skin Manifestations
Thoracic Wall