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

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.

• Authors: Anil Bhansali, Bhawani S Sharma, Polupoina Sreenivasulu, Paramjit Singh, Rakesh K Vashisth, Radharaman J Dash
• Journal: Endocrine journal (Endocr J) Vol. 50 Issue 6 Pg. 793-9 (Dec 2003) ISSN: 0918-8959 [Print] Japan
• PMID: 14709853 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Human Growth Hormone

Topics

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