Document Detail


Cardiac tumors and the nevoid basal cell carcinoma syndrome.
MedLine Citation:
PMID:  2020522     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Nevoid basal cell carcinoma syndrome is a multisystem disease with a wide range of initial symptoms that can be seen at any age. The most characteristic features are vertebral or rib anomalies, intracranial falx calcification, multiple basal cell carcinomas, odontogenic keratocysts of the jaw, and palmar and/or plantar pits. Pediatricians need to be aware that if any one of these major anomalies is seen, this diagnosis should be considered. There now appears to be an established association between cardiac tumors and nevoid basal cell carcinoma syndrome. Primary cardiac tumors have been associated with cerebral tuberous sclerosis and neurofibromatosis, and evaluation of cardiac status is recommended when these genetically determined syndromes are diagnosed. This communication should serve to alert pediatricians to the need for complete cardiac evaluation and genetic counseling when a diagnosis of nevoid basal cell carcinoma is made.
Authors:
J L Cotton; R E Kavey; C E Palmier; W W Tunnessen
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatrics     Volume:  87     ISSN:  0031-4005     ISO Abbreviation:  Pediatrics     Publication Date:  1991 May 
Date Detail:
Created Date:  1991-05-29     Completed Date:  1991-05-29     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  725-8     Citation Subset:  AIM; IM    
Affiliation:
Dept. of Pediatrics, State University of New York, Syracuse.
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MeSH Terms
Descriptor/Qualifier:
Basal Cell Nevus Syndrome / diagnosis*
Child, Preschool
Fibroma / diagnosis*
Heart Neoplasms / diagnosis*
Heart Ventricles
Humans
Male
Neoplasms, Multiple Primary / diagnosis*
Skin Neoplasms / diagnosis*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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