The radiologic findings of neurofibromatosis
10.3348/jkrs.1986.22.4.461
- Author:
So Sun KIM
;
Yeon Won PARK
;
Ho Joon KIM
;
Byung Hee CHUN
;
Young Duk JOH
;
Soo Jhi SUH
- Publication Type:Original Article
- MeSH:
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
- From:Journal of the Korean Radiological Society
1986;22(4):461-468
- CountryRepublic of Korea
- Language:Korean
-
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.
