Nevoid basal cell carcinoma syndrome:
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Date
1994Author
Ochola, Tom J Onyango
Type
ArticleLanguage
enMetadata
Show full item recordAbstract
Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by the development of multiple jaw keratocysts, frequently beginning in the second decade of life, and/or basal cell carcinomas (BCCs) usually from the third decade onward. Approximately 60% of individuals have a recognizable appearance with macrocephaly, bossing of the forehead, coarse facial features, and facial milia. Most individuals have skeletal anomalies (e.g., bifid ribs, wedge-shaped vertebrae). Ectopic calcification, particularly in the falx, is present in more than 90% of affected individuals by age 20 years. Cardiac and ovarian fibromas occur in approximately 2% and 20% of individuals respectively. Approximately 5% of children with NBCCS develop medulloblastoma (primitive neuroectodermal tumor [PNET]), generally the desmoplastic subtype. Peak incidence is at age two years. Life expectancy in NBCCS is not significantly different from average
URI
http://www.ncbi.nlm.nih.gov/books/NBK1151/http://erepository.uonbi.ac.ke:8080/xmlui/handle/123456789/47345
Citation
East African Medical Journal Vol. 7 No.11 1994 – 1994Publisher
University of Nairobi School of Medicine
Collections
- Faculty of Health Sciences (FHS) [10377]