Adolescent acromegaly: clinical parameters and treatment outcome

J Pediatr Endocrinol Metab. 2010 Oct;23(10):1047-54. doi: 10.1515/jpem.2010.166.

Abstract

Background: Adolescent acromegaly is a rare disorder and these patients present with tall stature/gigantism, tumor mass effects and menstrual irregularities.

Patients and methods: 34 consecutive (26 males) patients having onset of disease prior to 21 years of age were included in this retrospective analysis. Their clinical features and treatment outcome were studied.

Results: Mean age and lag time at presentation were 21.6 +/- 3.9 years and 5.1 +/- 3.5 years respectively. Common presenting manifestations included acral enlargement, tumor mass effects and menstrual irregularities. Mean height at presentation was 174.6 +/- 13.7 cms (range: 150-210 cm) and one third had gigantism (height > or =97th percentile, WHO growth charts). Hypertension and glucose intolerance were seen in 15% and 23.5% respectively. Mean nadir GH after glucose load was 58.2 +/- 13.7 ng/ml and IGF -1 was 534.8 +/- 132.8 ng/ml. Half of the patients had concomitant hyperprolactinemia. Almost all (97%) had macroadenoma and anterior pituitary hormone deficiencies were frequent (75%). Patients with gigantism were younger (19.6 +/- 4.9 vs. 22.6 +/- 2.9 years; p = 0.001), had higher GH values (66.68 +/- 27.22 vs. 53.98 +/- 15.99 ng/ml; p = 0.04) and hypogonadism was more common (90.9% vs. 56.5%, p = 0.03) than those with normal stature. 32 patients (94.1%) were treated primarily with surgery, 7 (21.9%) received post operative radiotherapy. Mean duration of follow up was 33.1 +/- 10.1 months. Only 30% had nadir GH values of <1 ng/ml.

Conclusion: One third of adolescent patients had acrogigantism. These patients were younger, had higher GH levels and concurrent hypogonadism was more common. Cure could be achieved only in about one third of the patients.

MeSH terms

  • Acromegaly / blood
  • Acromegaly / etiology
  • Acromegaly / therapy*
  • Adenoma / blood
  • Adenoma / complications
  • Adenoma / therapy*
  • Adolescent
  • Adult
  • Child
  • Female
  • Human Growth Hormone / blood
  • Humans
  • Male
  • Pituitary Neoplasms / blood
  • Pituitary Neoplasms / complications
  • Pituitary Neoplasms / therapy*
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Human Growth Hormone