McCune–Albright syndrome and acromegaly: clinical studies and responses to treatment in five cases

Abstract

We report here five new patients with McCune-Albright syndrome and acromegaly. In the five patients studied (three males and two females aged 18-42 years), acromegaly began before the age of 20 years and was recognized after the diagnosis of fibrous dysplasia, which was polyostotic in three cases and monostotic in two. Bone fibrous dysplasia always involved the base of the skull and in four patients prevented surgical removal of the pituitary adenoma, which was visualized easily by magnetic resonance imaging. Serum growth hormone (GH) levels ranged between 20 and 48 micrograms/l and were not suppressed by an oral glucose load. Thyrotropin-releasing hormone administration produced a paradoxical increase in serum GH levels in all the patients. Four of the five patients had hyperprolactinemia (43-670 micrograms/l). In the sole patient who could be operated on, a typical adenoma with positive immunostaining for GH was incompletely removed and postoperative radiation therapy failed to cure the acromegaly. In two patients, medical therapy with bromocriptine and/or octreotide was partially or totally ineffective whatever the dose (up to 1.5 mg per day) and duration (2-4 years) of octreotide treatment.

Acromegaly with Fibrous Dysplasia: McCune-Albright Syndrome-Clinical Studies in 3 Cases and Brief Review of Literature-

“…In almost every case, the base of the skull was involved as seen in all of our patients. However, the course of dysplastic bone disease is independent of the time of onset and degree of disease activity [3].…”

“…Till 2002, only 51 cases of MAS with acromegaly have been reported in English literature 34]. However, pathological examination of the pituitary either at surgery or necropsy has been reported in only 8 of them [3,6,7,10,14,24,28,30]. We report 3 patients, in the existing repertoire of this rare entity, who had fibrous dysplasia and acromegaly due to growth hormone (GH) secreting pituitary adenoma.…”

“…Polyostotic fibrous dysplasia and café-au-lait macules are invariably present while monostotic lesions are less common [3]. Endocrine hyperfunction other than precocious puberty in association with MAS include hyperthyroidism, hypercortisolism, hypersomatotropism and hypophosphatemic rickets [1][2][3]. Association of MAS with hypersomatotropism is rare, and pituitary adenoma is demonstrable in only 40-50% of these patients [3,4].…”

“…Endocrine hyperfunction other than precocious puberty in association with MAS include hyperthyroidism, hypercortisolism, hypersomatotropism and hypophosphatemic rickets [1][2][3]. Association of MAS with hypersomatotropism is rare, and pituitary adenoma is demonstrable in only 40-50% of these patients [3,4]. Till 2002, only 51 cases of MAS with acromegaly have been reported in English literature 34].…”

McCune Albright syndrome in association with excessive GH secretion: case report

“…Treatment-related side effects include impaired glucose tolerance with use of long-acting somatostatin and gallbladder stones following combination treatment (12)(13)(14). In our patient, stone was observed in the gallbladder at the end of the one-year follow-up period, but fasting and postprandial blood glucose levels were found to be normal.…”