Document Detail

Gorlin-Goltz syndrome and neoplasms: a case study.
MedLine Citation:
PMID:  21417126     Owner:  NLM     Status:  In-Process    
Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome. Objective: To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias.
Nilza N F Lopes; Eliana M Caran; Maria Lucia Lee; Nasjla Saba Silva; André Caroli Rocha; Carla R D Macedo
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of clinical pediatric dentistry     Volume:  35     ISSN:  1053-4628     ISO Abbreviation:  J Clin Pediatr Dent     Publication Date:  2010  
Date Detail:
Created Date:  2011-03-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9100079     Medline TA:  J Clin Pediatr Dent     Country:  United States    
Other Details:
Languages:  eng     Pagination:  203-6     Citation Subset:  D    
Pediatric Oncology Institute--GRAACC, Medical School of São Paulo, Federal University of São Paulo, SP, Brazil.
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