The association of the
Albright syndrome (
polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and endocrine disorders) with
acromegaly has been infrequently substantiated. The case of an 18-year-old girl with the classic
Albright syndrome and
acromegaly is described. The patient had a history of coarsening of acral and facial features, an
insulin-resistant form of
diabetes mellitus and elevated fasting
growth hormone values. Neuro-endocrine studies demonstrated failure of
growth hormone to suppress to less than 5 ng/ml during an oral
glucose tolerance test, and the abnormal release of
growth hormone upon injection of
thyrotropin-releasing hormone. Although
L-dopa failed to decrease
growth hormone levels,
bromocriptine produced a modest decline in
growth hormone within two hours of ingestion. The patient had also experienced secondary
amenorrhea with sub-normal
follicle-stimulating-hormone (FSH) and
luteinizing hormone (LH) levels, both of which demonstrated a prolonged sluggish response to an injection of
gonadotropin-releasing hormone (
GnRH); this response suggested
hypogonadotropic hypogonadism, possibly on the basis of a
tumor involving both pituitary and hypothalamus. Sellar polytomography demonstrated an enlarged sella with dorsal erosion and an asymmetric floor. Computerized tomography of the brain visualized a suprasellar mass extending into the hypothalamus. These findings suggest a hypersecretion of hypothalamic releasing factors,
pituitary hormones, or both as an etiology for the endocrinopathy in this patient, and lend support to the theory that the endocrinopathies associated with the
Albright syndrome result from over-production of
hypothalamic-releasing hormones or autonomous secretion of
pituitary hormones from an
adenoma.