Linked Life Data

9719504

Source:http://linkedlifedata.com/resource/pubmed/id/9719504

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
1998-9-9
pubmed:abstractText
The association between of fibrous dysplasia and intramuscular myxomas is extremely rare. The authors summarize the characteristics of Mazabraud's syndrome, as well as its clinical course on the basis of 24 cases reported in the literature and 3 cases of their own. The syndrome is quite uniform, and mostly occurs in women. Usually the presentation of poliostotic fibrous dysplasia bilaterally in the lower limbs and pelvic bones is followed by the appearance of multifocal intramuscular myxomas in the adjacent muscles, mostly decades later. These hamartomas tend to recur locally or symmetrically in the ipsilateral muscle groups and may reach enormous size without treatment. Malignant transformation (osteosarcoma, fibrosarcoma) on the basis of fibrous dysplasia in Mazabraud's syndrome has been reported in the literature. In our cases, however, in spite of the many recurrences, and the enormous size of the tumours, no malignant transformation was noted in either the fibrous dysplasias or the intramuscular myxomas during the long follow-up time (31 years for fibrous dysplasia and 16 years for myxomas). Early wide surgical excision and a careful long-term follow-up for the often very late recurrences of the myxomas is suggested in Mazabraud's syndrome.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:issn
0171-5216
pubmed:author
pubmed:issnType
Print
pubmed:volume
124
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
401-6
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1998
pubmed:articleTitle
Fibrous dysplasia associated with intramuscular myxoma (Mazabraud's syndrome): a long-term follow-up of three cases.
pubmed:affiliation
Orthopaedic Department of the Semmelweis University of Medicine, Budapest Karolina út 27, Hungary.
pubmed:publicationType
Journal Article, Case Reports