Acromegaly with fibrous dysplasia: McCune-Albright Syndrome -- clinical studies in 3 cases and brief review of literature--.
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.
Bhansali
Anil
A
Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Sharma
Bhawani S
BS
Sreenivasulu
Polupoina
P
Singh
Paramjit
P
Vashisth
Rakesh K
RK
Dash
Radharaman J
RJ
D000172:Acromegaly / Q000150:complications*; D000236:Adenoma / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D000328:Adult; D005358:Fibrous Dysplasia, Monostotic / Q000150:complications*; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications*; D019382:Human Growth Hormone / Q000378:metabolism*; D006801:Humans; D008297:Male; D010911:Pituitary Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D014057:Tomography, X-Ray Computed
D019382:Human Growth Hormone