- Erica A Eugster, MD
Erica A Eugster, MD
- Professor of Pediatrics
- Indiana University School of Medicine
Pituitary gigantism refers to growth hormone (GH) excess that occurs before fusion of the epiphyseal growth plates. Therefore, by definition the condition is only seen in growing children. In this setting, elevated levels of serum GH and insulin-like growth factor I (IGF-I) cause rapid, excessive linear growth and, if unchecked, extremely tall adult stature. In contrast, GH excess that begins in adulthood, after complete epiphyseal fusion, has no effect on stature and is called acromegaly. Although many historical claims of gigantism have been exaggerated, case reports of true pituitary giants with heights of up to 8 feet 11 inches (272 cm) are documented in the medical literature . (See "Causes and clinical manifestations of acromegaly".)
Pituitary gigantism is a rare disorder. Most pediatric endocrinologists may see at most one or two patients with the condition during their careers. In one large series of 2367 children and adolescents with pituitary adenomas, only 15 (0.6 percent) had pituitary gigantism . Although much of our understanding of this disease has been derived from isolated case reports and extrapolation from the adult literature , a retrospective review of 208 cases of pituitary gigantism from around the globe provides important insights . These include male predominance and a lack of identifiable genetic etiology in more than 50 percent of cases. Gigantism may occur at any age, and has been observed as early as the first two to three months of life.
Pituitary gigantism typically is a sporadic and isolated condition. However, it may occur within the context of a coexisting disorder or arise according to a pattern of familial inheritance.
Syndromes in which gigantism is a well-recognized feature include McCune Albright Syndrome (MAS); multiple endocrine neoplasia type 1 (MEN1); multiple endocrine neoplasia type 4 (MEN4); Carney complex; and the paraganglioma, pheochromocytoma and pituitary adenoma association known as 3PA. Growth hormone excess has also been observed in the setting of neurofibromatosis and optic nerve tumors. The frequency of gigantism is established in only a subset of these conditions and varies significantly:
●Approximately 20 percent of patients with MAS are thought to have growth hormone (GH) hypersecretion [5-7], although few large case series are available . In one review of 112 patients with MAS and acromegaly, the mean age at diagnosis of acromegaly was just over 24 years . Subclinical GH excess also has been reported in MAS, where it results in unexpected normal (rather than short) stature in the setting of untreated precocious puberty . (See "Definition, etiology, and evaluation of precocious puberty", section on 'McCune-Albright syndrome'.)
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