Case history
Case history #1
A 47-year-old man presents with arthritic pain of the knees and hips, soft-tissue swelling, and excessive sweating. He also noticed progressive enlargement of the hands and feet. He has been taking antihypertensive medication for the past 3 years. On physical examination, he has coarse facial features with prognathism and prominent supraorbital ridges. The tongue is enlarged and the fingers are thickened. His wife says that he frequently snores. Laboratory workup reveals an elevated plasma insulin-like growth factor 1 (IGF-1) concentration of 560 micrograms/L (normal for age, 120-235 micrograms/L) and a basal plasma growth hormone level of 15 micrograms/L. MRI examination of the sella turcica region shows a 14 mm pituitary mass with right cavernous sinus invasion.
Case history #2
A 15-year-old girl presents with primary amenorrhea and accelerated growth. On physical examination, her height is above the 90th percentile, her pubertal development is evaluated at Tanner stage 2, and she has soft-tissue swelling. Laboratory workup reveals a moderately elevated serum prolactin concentration of 44 micrograms/L (normal, <20 micrograms/L) and an elevated IGF-1 level of 1525 micrograms/L (normal for age, 198-551 micrograms/L). Pituitary MRI shows a 15 mm pituitary mass without parasellar extension.
Other presentations
The clinical manifestations may be due to the pituitary mass effects, such as headaches or visual field defects, or the systemic actions of excess growth hormone (GH) and insulin-like growth factor 1 (IGF-1), such as acral and soft-tissue growth and metabolic abnormalities.[4] Patients with acromegaly may present with glucose intolerance or diabetes mellitus, hypertension, cardiac hypertrophy, or sleep apnea.[5] During childhood, before the epiphyses of long bones start to fuse, increased GH production results in increased height and gigantism. Infrequently, acromegaly may manifest with familial syndromes, such as multiple endocrine neoplasia type 1 with hyperparathyroidism, digestive tract neuroendocrine tumors, McCune-Albright syndrome with polyostotic fibrous dysplasia in the bones, and Carney complex with cardiac myxomas and endocrine tumors.[6] Rarely, the disease is associated with hypothalamic or pancreatic neuroendocrine tumors secreting excess GH or GH-releasing hormone.[7]
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