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The Journal of Bone and Joint Surgery 79:1545-51 (1997)
© 1997 The Journal of Bone and Joint Surgery, Inc.

Case Report

Dedifferentiated Chondrosarcoma in Albright Syndrome. A Case Report and Review of the Literature*

TOSHIFUMI OZAKI, M.D.{dagger}, NORBERT LINDNER, M.D.{ddagger} and SEBASTIAN BLASIUS, M.D.{ddagger}, MÜNSTER, GERMANY

Investigation performed at the Department of Orthopaedics, University of Münster, Münster


   Introduction
 
Fibrous dysplasia is a developmental condition in which the skeleton fails to mature normally. Albright syndrome, which was first reported in 1937 by McCune and Bruch23 and by Albright1, is characterized by polyostotic fibrous dysplasia, endocrine disorders, and brown patches on the skin (café-au-lait spots)3,17,25. It is seen more often in female than in male patients35. Malignant transformation in fibrous dysplasia was noted, in 1945, by Coley and Stewart5, and since then it has been reported in more than 100 patients12,19,33,43. The most common secondary malignant lesion in fibrous dysplasia is osteosarcoma, followed by fibrosarcoma and chondrosarcoma6,9,15,18,33,42. The prevalence of malignant transformation in fibrous dysplasia is only 0.4 per cent (six of 1517 patients)36. However, the prevalence of malignant transformation in Albright syndrome is 4 per cent (four of 100 patients36), which is higher than that in other forms of fibrous . . . [Full Text of this Article]


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