Acromegaly with fibrous dysplasia: McCune-Albright Syndrome -- clinical studies in 3 cases and brief review of literature--

Endocr J. 2003 Dec;50(6):793-9. doi: 10.1507/endocrj.50.793.

Abstract

The McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies including growth hormone (GH) excess. Polyostotic bone lesions and café-au-lait macules are common while monostotic bone lesions are rare. Similarly, acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon and in most of the instances somatotropinoma has not been documented. We report 3 patients, two of them had monostotic lesion, none had café-au-lait macules and all had GH secreting pituitary macroadenoma. All of them underwent transfrontal pituitary adenomectomy and had histopathological confirmation of GH secreting pituitary adenoma. A brief review of literature is also presented.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Acromegaly / complications*
  • Adenoma / complications*
  • Adenoma / diagnosis
  • Adenoma / metabolism
  • Adenoma / surgery
  • Adult
  • Fibrous Dysplasia, Monostotic / complications*
  • Fibrous Dysplasia, Polyostotic / complications*
  • Human Growth Hormone / metabolism*
  • Humans
  • Male
  • Pituitary Neoplasms / complications*
  • Pituitary Neoplasms / diagnosis
  • Pituitary Neoplasms / metabolism
  • Pituitary Neoplasms / surgery
  • Tomography, X-Ray Computed

Substances

  • Human Growth Hormone