+ Site Statistics
References:
52,654,530
Abstracts:
29,560,856
PMIDs:
28,072,755
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

A case of fibrous dysplasia (McCune Albright syndrome) associated with acromegaly



A case of fibrous dysplasia (McCune Albright syndrome) associated with acromegaly



Nihon Naibunpi Gakkai Zasshi 67(3): 239-248



We report a case of a 47-year-old woman with McCune-Albright syndrome associated with unusual growth-hormone and prolactin hypersecretion. Acromegaly was suspected on clinical examination, and she was referred to us. She had no history of precocious puberty or pathological fracture. She was 154cm tall, weighing 62kg with so-called acromegalic facies. There were two lumps, one of which was on her right lateral forehead. The other on the right lower extremity seemed to be due to a bone deformity. Some brown pigmented macules with irregular borders were present on her lips and oral mucosa. Endocrine examination revealed elevated basal levels of plasma GH (54 ng/ml) and PRL (36.2 ng/ml) which paradoxically increased after injection of TRH. Plasma levels of these hormones did not change after LHRH test, and plasma GH level increased after GHRH test. A skull X-ray film showed a double floor of the sella turcica and hyperostotic formation of the right sphenoid bone. MRI and CT showed a tumor shadow in the right sella turcica. Bone roentogenography of the right fibula and tibia showed a large centrally expanding lesion with a ground-glass or cystic appearance and a thin cortex. The left fibula showed a similar lesion. We recognized similar findings in other bones which also showed abnormal accumulation in a radionuclide bone scan with 99mTc. On the basis of physical, endocrinological and roentogenographical examination, we diagnosed her as displaying McCune-Albright syndrome. We operated on her by transsphenoidal surgery and confirmed a functioning chromophobe adenoma which was removed during surgery. She has been free of GH and PRL hypersecretion since then. The pathology of the sphenoidal sinus affected with fibrous bone was in accordane with the findings of fibrous dysplasia. McCune-Albright syndrome (MCAS) is known as a syndrome characterized by polyostotic fibrous dysplasia and cutaneous pigmentation and multiple endocrinopathy. Its etiology is unknown. This syndrome with acromegaly is rare, and although 26 cases have been described in literature, pathological examinations have been undertaken in only three cases. This case was very rare and valuable because, by operating on her by the transsphenoidal route in spite of the sphenoidal sinus being affected with fibrous bone, we were able to confirm a chromophobe adenoma secreting both GH and PRL. We recognized that acromegaly with MCAS was endocrinologically, morphologically and immunohistologically the same as acromegaly without MCAS. Therefore, we concluded that acromegaly with MCAS is much the same as acromegaly without MCAS.

(PDF emailed within 0-6 h: $19.90)

Accession: 006932448

Download citation: RISBibTeXText

PMID: 2070893

DOI: 10.1507/endocrine1927.67.3_239


Related references

Galphas-activating mutations A cause of acromegaly, thyroid adenomas, fibrous dysplasia and the McCune-Albright syndrome. Spiegel, A M Contemporary Endocrinology; G proteins, receptors, and disease 57-69, 1998

Acromegaly with fibrous dysplasia: McCune-Albright Syndrome -- clinical studies in 3 cases and brief review of literature--. Endocrine Journal 50(6): 793-799, 2004

A patient with acromegaly presenting with polyostotic fibrous dysplasia on bone scan: McCune-Albright syndrome. Clinical Nuclear Medicine 30(12): 813-815, 2005

Visualization of fibrous dysplasia with somatostatin receptor scintigraphy in a girl with acromegaly and the McCune-Albright syndrome. Hormone Research (Basel) 48(SUPPL 2): 173, 1997

Acromegaly, multinodular goiter and silent polyostotic fibrous dysplasia. A variant of the McCune-Albright syndrome. Journal of Endocrinological Investigation 13(8): 671-675, 1990

Pamidronic acid and cabergoline as effective long-term therapy in a 12-year-old girl with extended facial polyostotic fibrous dysplasia, prolactinoma and acromegaly in McCune-Albright syndrome: a case report. Journal of Medical Case Reports 6(): 32-32, 2012

McCune-Albright syndrome with acromegaly and fibrous dysplasia associated with the GNAS gene mutation identified by sensitive PNA-clamping method. Internal Medicine 46(18): 1577-1583, 2007

Polyostotic fibrous dysplasia. (Albright-McCune's syndrome) report of a case. Acta Societatis Medicorum Upsaliensis 64: 226-231, 1959

A case of craniofacial fibrous dysplasia associated with McCune-Albright syndrome lost to follow-up. Bmj Case Reports 2015: -, 2016

McCune Albright syndrome - diffuse orbital involvement due to fibrous dysplasia: a case report. Arquivos Brasileiros de Oftalmologia 70(6): 1021-1023, 2008

Residents' corner. Answer to case of the month #12. McCune-Albright syndrome (polyostotic fibrous dysplasia). Canadian Association of Radiologists Journal 43(2): 149-151, 1992

Fibrous dysplasia and McCune-Albright syndrome: case-report and differential diagnose with Paget bone disease. Acta Reumatologica Portuguesa 35(5): 497-503, 2011

Oral rehabilitation with endosseous implants in a patient with fibrous dysplasia (McCune-Albright syndrome): a case report. Journal of Oral and Maxillofacial Surgery 66(12): 2605-2608, 2008

Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor: a rare case of McCune-Albright syndrome. International Journal of Clinical Oncology 16(3): 270-274, 2011

Gigantism treated by pure endoscopic endonasal approach in a case of McCune-Albright syndrome with sphenoid fibrous dysplasia: a case report. Journal of Neurological Surgery. Part A, Central European Neurosurgery 74 Suppl 1: E140-E144, 2014