Treatment protocols for growth hormone‑secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune‑Albright syndrome

Xu,Jia; Li,Xi; Lv,Chang‑Sheng; Chen,Ying; Wang,Meng; Liu,Jian‑Feng; Gui,Lai

doi:10.3892/etm.2014.1792

Treatment protocols for growth hormone‑secreting pituitary adenomas combined with craniofacial fibrous dysplasia: A case report of atypical McCune‑Albright syndrome

Authors:
- Jia Xu
- Xi Li
- Chang‑Sheng Lv
- Ying Chen
- Meng Wang
- Jian‑Feng Liu
- Lai Gui
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Affiliations: Department of Maxillofacial Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing 100144, P.R. China
Published online on: June 19, 2014 https://doi.org/10.3892/etm.2014.1792
Pages: 877-880

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Abstract

McCune‑Albright syndrome (MAS) is a rare, post‑zygotic (non‑germline) disorder, characterized by hypersecretory endocrinopathies, fibrous dysplasia of the bone and café‑au‑lait macules. The most common endocrine dysfunction is gonadal hyperfunction; thus, hypersecretion of growth hormones (GHs) as a manifestation of endocrine hyperfunction in MAS is rarely reported. MAS affects both genders, although the majority of cases have been reported in young females. Atypical presentations of MAS, with only one or two of the classic symptoms, have been previously described, but remain particularly challenging due to the lack of a diagnostic phenotype. In patients with atypical MAS, analysis of mutations in the gene of the α‑subunit of the stimulatory G‑protein is limited; thus, diagnosis is based on clinical judgment. In the present study, a male with polyostotic fibrous dysplasia and GH‑secreting pituitary adenomas, diagnosed with atypical MAS, was reported. The pituitary adenoma was effectively treated with radiotherapy and the patient underwent surgery for the polyostotic fibrous dysplasia, with marked improvements observed in appearance.

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1	Dumitrescu CE and Collins MT: McCune-Albright syndrome. Orphanet J Rare Dis. 3:122008. View Article : Google Scholar : PubMed/NCBI
2	Zhou J, Sun LH, Cui B, et al: Genetic diagnosis of multiple affected tissues in a patient with McCune-Albright syndrome. Endocrine. 31:212–217. 2007. View Article : Google Scholar : PubMed/NCBI
3	Akintoye SO, Chebli C, Booher S, et al: Characterization of gsp-mediated growth hormone excess in the context of McCune-Albright syndrome. J Clin Endocrinol Metab. 87:5104–5112. 2002. View Article : Google Scholar : PubMed/NCBI
4	Weinstein LS, Shenker A, Gejman PV, et al: Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J Med. 325:1688–1695. 1991. View Article : Google Scholar : PubMed/NCBI
5	Obuobie K, Mullik V, Jones C, et al: McCune-Albright syndrome: growth hormone dynamics in pregnancy. J Clin Endocrinol Metab. 86:2456–2458. 2001. View Article : Google Scholar : PubMed/NCBI
6	Bercaw-Pratt JL, Moorjani TP, Santos XM, Karaviti L and Dietrich JE: Diagnosis and management of precocious puberty in atypical presentations of McCune-Albright syndrome: a case series review. J Pediatr Adolesc Gynecol. 25:e9–e13. 2012. View Article : Google Scholar : PubMed/NCBI
7	Yavuzer R, Khilnani R, Jackson IT and Audet B: A case of atypical McCune-Albright syndrome requiring optic nerve decompression. Ann Plast Surg. 43:430–435. 1999. View Article : Google Scholar : PubMed/NCBI
8	Hannon TS, Noonan K, Steinmetz R, et al: Is McCune-Albright syndrome overlooked in subjects with fibrous dysplasia of bone? J Pediatr. 142:532–538. 2003. View Article : Google Scholar : PubMed/NCBI
9	Bhansali A, Sharma BS, Sreenivasulu P, et al: Acromegaly with fibrous dysplasia: McCune-Albright Syndrome - clinical studies in 3 cases and brief review of literature. Endocr J. 50:793–799. 2003. View Article : Google Scholar : PubMed/NCBI
10	Dou W, Lin N, Ma W, et al: Transsphenoidal surgery in a patient with acromegaly and McCune-Albright syndrome: application of neuronavigation. J Neurosurg. 108:164–169. 2008. View Article : Google Scholar : PubMed/NCBI
11	Abs R, Beckers A, Van de Vyver FL, et al: Acromegaly, multinodular goiter and silent polyostotic fibrous dysplasia. A variant of the McCune-Albright syndrome. J Endocrinol Invest. 13:671–675. 1990. View Article : Google Scholar : PubMed/NCBI
12	Tanner HC Jr, Dahlin DC and Childs DS Jr: Sarcoma complicating fibrous dysplasia: probable role of radiation therapy. Oral Surg Oral Med Oral Pathol. 14:837–846. 1961. View Article : Google Scholar : PubMed/NCBI
13	Ruggieri P, Sim FH, Bond JR and Unni KK: Malignancies in fibrous dysplasia. Cancer. 73:1411–1424. 1994. View Article : Google Scholar : PubMed/NCBI
14	Osada Y, Iwasawa M and Tanaka Y: Use of image-guiding template for contouring surgery of midfacial fibrous dysplasia. Ann Plast Surg. 59:459–463. 2007. View Article : Google Scholar : PubMed/NCBI
15	Chen YR and Fairholm D: Fronto-orbito-sphenoidal fibrous dysplasia. Ann Plast Surg. 15:190–203. 1985. View Article : Google Scholar : PubMed/NCBI
16	Munro IR and Chen YR: Radical treatment for fronto-orbital fibrous dysplasia: the chain-link fence. Plast Reconstr Surg. 67:719–730. 1981. View Article : Google Scholar : PubMed/NCBI
17	Ozek C, Gundogan H, Bilkay U, et al: Craniomaxillofacial fibrous dysplasia. J Craniofac Surg. 13:382–389. 2002. View Article : Google Scholar