We describe a girl, one of monozygotic (MZ) twins, with endocrine dysfunction with precocious puberty, cafe-au-lait nevi and polyostotic fibrous dysplasia (PFD), McCune-Albright syndrome (MAS). After treatment with cyproterone acetate for 7 years the precocious puberty and excess growth improved but
Whole-body MR imaging for the evaluation of mcCune-albright syndrome
✍ Scribed by Elisa C. Ferreira; Cecília C.B. Brito; Roberto C. Domingues; Marcio Bernardes; Edson Marchiori; Emerson L. Gasparetto
- Publisher
- John Wiley and Sons
- Year
- 2010
- Tongue
- English
- Weight
- 185 KB
- Volume
- 31
- Category
- Article
- ISSN
- 1053-1807
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✦ Synopsis
Abstract
This paper is a detailed case study of a seven‐year‐old girl who developed precocious puberty. The young girl experienced bilateral breast enlargement, vaginal discharge, and multiple areas of “cafe‐au‐lait” pigmentation on the skin. The skeletal radiographs showed multiple bone lesions which were suggestive of polyostotic fibrous dysplasia. These findings were sufficient to establish the diagnosis of McCune‐Albright syndrome. Whole‐body MRI was used to assess the extent of the osseous lesions throughout the body. The MRI showed approximately the same number of osseous lesions compared to the skeletal radiographs. In addition, the MRI detected a cystic lesion localized in the pelvis that raised the possibility of an ovarian‐follicular cyst. This case suggests that whole‐body MRI might be a promising method for the evaluation and follow‐up of polyostotic fibrous dysplasia, a disease with diffuse involvement of the musculoskeletal system. J. Magn. Reson. Imaging 2010;31:706–710. © 2010 Wiley‐Liss, Inc.
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