Linked Life Data

7000334

Source:http://linkedlifedata.com/resource/pubmed/id/7000334

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
1981-1-26
pubmed:abstractText
We report on the cases of two sisters with carotid body tumor (CBT) and present a literature review that assembles epidemiologic information on 88 familial and 835 nonfamilial CBT patients. The sex ratio (males/females) of 1.0 for familial CBT (0.7 for nonfamilial) and CBT reports with complete sibship information suggest autosomal dominant genetic transmission. As in other familial cancers, bilateral disease is significantly more frequent in familial (31.8% of cases) than in nonfamilial CBT (4.4%). However, there is no difference in age at diagnosis between familial and nonfamilial CBR. Thus, this adult-onset familial cancer does not completely fit the Knudson "two-step mutation" model of carcinogenesis. We also found that 6% of reported CBT patients developed second primary tumors, mostly other paragangliomas. This feature suggests that CBT may be part of a larger neurocristopathy syndrome of multiple tumors of cells of neural crest origin.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:status
MEDLINE
pubmed:month
Nov
pubmed:issn
0008-543X
pubmed:author
pubmed:issnType
Print
pubmed:day
1
pubmed:volume
46
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
2116-22
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1980
pubmed:articleTitle
Familial carotid body tumors: case report and epidemiologic review.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Review, Case Reports