Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Hyperpituitarism Clinical Presentation

  • Author: Alicia Diaz-Thomas, MD, MPH; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: May 28, 2014
 

History

The clinical presentation of a pituitary adenoma primarily results from the oversecreted hormone. The tumor mass itself may cause headaches, visual changes due to optic nerve compression, or hypopituitarism.

Excess prolactin

The presentation of prolactinomas may vary, depending on the age and sex of the child.

Prepubertal children typically present with a combination of headache, visual disturbance, and growth failure.

Pubertal females frequently present with symptoms of pubertal arrest or hypogonadism (with or without galactorrhea) due to suppression of gonadotropin secretion or local compression of the pituitary.

Pubertal males may present with headaches, visual impairment, and pubertal arrest or growth failure.

Excess adrenocorticotropic hormone

The most sensitive indicator of excess glucocorticoid secretion in children is weight gain with concurrent growth failure, which generally precedes other manifestations.

Patients commonly present with weight gain that tends to be generalized rather than centripetal.

Hirsutism and premature adrenarche may occur in prepubertal children.

Hypertension may be present.

Pubertal arrest, acne, fatigue, and depression are also common.

Snoring, poor sleep quality, deteriorating academic performance (compared with prior school terms), or other signs of obstructive sleep apnea (OSA) should prompt a formal sleep study and consultation with a pulmonologist.

Excess growth hormone

The presentation of gigantism in a child is usually dramatic, unlike the insidious onset of acromegaly in adults.

The cardinal clinical feature of gigantism is longitudinal growth acceleration secondary to GH excess.

Presentation depends on whether the epiphyseal growth plate is open. Before epiphyseal fusion, accelerated growth velocity is prominent. As epiphyseal fusion approaches, the spectrum of symptoms resembles the presentation in adults (eg, coarsening of facial features, change in ring and shoe size).

Next

Physical

Prolactinoma may include the following:

  • Hypogonadism, leading to pubertal arrest, pubertal failure, or pubertal delay
  • Menstrual abnormalities, including primary or secondary amenorrhea
  • Galactorrhea
  • Gynecomastia

Cushing disease may include the following (see images below):

A 16-year-old boy with Cushing disease. A 16-year-old boy with Cushing disease.
On the left is an unaffected patient aged 12 years On the left is an unaffected patient aged 12 years. On the right is the same patient aged 13 years after developing Cushing disease.

See the list below:

  • Cushingoid appearance includes a dorsal cervical fat pad, moon facies, bruising, and striae. These features are only observed in patients with advanced long-standing disease.
  • Growth failure and short stature may be observed.
  • Weight gain and obesity in children with Cushing disease tends to be generalized rather than centripetal.
  • Pubertal arrest, failure, or delay may occur.
  • Amenorrhea may be noted.
  • Hypertension may be present.

In patients with gigantism, all growth parameters are affected, although not necessarily symmetrically. GH excess over time is characterized by progressive cosmetic disfigurement and systemic organ manifestations. The following may be noted:

  • Tall stature
  • Mild-to-moderate obesity (common)
  • Macrocephaly, which may precede linear growth
  • Exaggerated growth of the hands and feet with thick fingers and toes
  • Coarse facial features, including frontal bossing and prognathism
  • Hyperhidrosis
  • Menstrual irregularities
  • Peripheral neuropathies (eg, carpal tunnel syndrome)
  • Cardiovascular disease: Prolonged GH excess can result in cardiac hypertrophy, hypertension, and left ventricular hypertrophy.
  • Tumors: Although benign tumors, including uterine myomas, prostatic hypertrophy, colon polyps, and skin tags, may be frequently encountered in acromegaly, the documentation of the overall prevalence of malignancies in patients with acromegaly remains controversial.
  • Endocrinopathies: Frequently associated endocrinopathies include hypogonadism, diabetes, decreased glucose tolerance, and hyperprolactinemia. OSA has been reported in up to half of patients with acromegaly, particularly those who are obese or older than 50 years.
Previous
Next

Causes

Hypothalamic dysfunction can promote tumor growth, but overwhelming evidence points to intrinsic pituicyte genetic disruption as the main underlying cause of pituitary tumorigenesis. The monoclonal nature of most pituitary adenomas, confirmed with X-inactivation studies, implies their origin from a clonal event in a single cell. Most pituitary adenomas are functional, and clinical presentation typically depends on the particular pituitary hormone that is hypersecreted. Nonfunctioning pituitary adenomas are rare in children, accounting for only 3-6% of all adenomas in 2 large series; they comprise 30% of adenomas in adults.

Previous
 
 
Contributor Information and Disclosures
Author

Alicia Diaz-Thomas, MD, MPH Assistant Professor of Pediatrics, University of Tennessee Health Science Center

Alicia Diaz-Thomas, MD, MPH is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Pediatric Endocrine Society, Tennessee Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Melanie Shim, MD 

Melanie Shim, MD is a member of the following medical societies: American Diabetes Association, Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, American College of Endocrinology

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

Acknowledgements

Robert J Ferry Jr, MD Le Bonheur Chair of Excellence in Endocrinology, Professor and Chief, Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, University of Tennessee Health Science Center

Robert J Ferry Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society

Disclosure: Eli Lilly & Co Grant/research funds Investigator; MacroGenics, Inc Grant/research funds Investigator; Ipsen, SA (formerly Tercica, Inc) Grant/research funds Investigator; NovoNordisk SA Grant/research funds Investigator; Diamyd Grant/research funds Investigator; Bristol-Myers-Squibb Grant/research funds Other; Amylin Other; Pfizer Grant/research funds Other; Takeda Grant/research funds Other

References
  1. Kasum M, Oreskovic S, Zec I, Jezek D, Tomic V, Gall V, et al. Macroprolactinemia: new insights in hyperprolactinemia. Biochem Med (Zagreb). 2012. 22(2):171-9. [Medline].

  2. Chaudhary V, Bano S. Imaging of pediatric pituitary endocrinopathies. Indian J Endocrinol Metab. 2012 Sep. 16(5):682-91. [Medline]. [Full Text].

  3. Lonser RR, Ksendzovsky A, Wind JJ, Vortmeyer AO, Oldfield EH. Prospective evaluation of the characteristics and incidence of adenoma-associated dural invasion in Cushing disease. J Neurosurg. 2012 Feb. 116(2):272-9. [Medline].

  4. van der Lely AJ, Biller BM, Brue T, et al. Long-term safety of pegvisomant in patients with acromegaly: comprehensive review of 1288 subjects in ACROSTUDY. J Clin Endocrinol Metab. 2012 May. 97(5):1589-97. [Medline].

  5. Devoe DJ, Miller WL, Conte FA, et al. Long-term outcome in children and adolescents after transsphenoidal surgery for Cushing's disease. J Clin Endocrinol Metab. 1997. 82:3196-202. [Medline].

  6. Avramides A, Karapiperis A, Triantafyllidou E, et al. TSH-secreting pituitary macroadenoma in an 11-year-old girl. Acta Paediatr. 1992. 81:1058-1060. [Medline].

  7. Benveniste RJ, King WA, Walsh J, et al. Repeated transsphenoidal surgery to treat recurrent or residual pituitary adenoma. J Neurosurg. 2005. 102:1004-1012. [Medline].

  8. Booth GL, Redelmeier DA, Grosman H, et al. Improved diagnostic accuracy of inferior petrosal sinus sampling over imaging for localizing pituitary pathology in patients with Cushing's disease. J Clin Endocrinol Metab. 1998 Jul. 83(7):2291-5. [Medline].

  9. Ciccarelli A, Daly AF, Beckers A. The epidemiology of prolactinomas. Pituitary. 2005. 8:3-6. [Medline].

  10. Colao A, Loche S, Cappabianca P. Pituitary adenomas in children and adolescents. Clinical presentation, diagnosis, and therapeutic strategies. The Endocrinologist. 2000. 10:314-27.

  11. Colao A, Lombardi G. Growth-hormone and prolactin excess. Lancet. 1998 Oct 31. 352(9138):1455-61. [Medline].

  12. Cozzi R, Attanasio R, Barausse M, et al. Cabergoline in acromegaly: a renewed role for dopamine agonist treatment?. Eur J Endocrinol. 1998 Nov. 139(5):516-21. [Medline].

  13. [Guideline] Cozzi R, Baldelli R, Colao A, Lasio G, Zini M, Attanasio R. AME Position Statement on clinical management of acromegaly. J Endocrinol Invest. 2009. 32(6 Suppl):2-25. [Medline].

  14. de Boer L, Hoogerbrugge CM, van Doorn J, et al. Plasma insulin-like growth factors (IGFs), IGF-Binding proteins (IGFBPs), acid-labile subunit (ALS) and IGFBP-3 proteolysis in individuals with clinical characteristics of Sotos syndrome. J Pediatr Endocrinol Metab. 2004. 17:615-627. [Medline].

  15. De Menis E, Visentin A, Billeci D, et al. Pituitary adenomas in childhood and adolescence. Clinical analysis of 10 cases. J Endocrinol Invest. 2001. 24:92-97. [Medline].

  16. Dhillon KS, Cohan P, Kelly DF, et al. Treatment of hyperthyroidism associated with thyrotropin-secreting pituitary adenomas with iopanoic acid. J Clin Endocrinol Metab. 2004. 89:708-711. [Medline]. [Full Text].

  17. Duncan E, Wass JA. Investigation protocol: acromegaly and its investigation. Clin Endocrinol (Oxf). 1999 Mar. 50(3):285-93. [Medline].

  18. Eugster EA, Pescovitz OH. Gigantism. J Clin Endocrinol Metab. 1999 Dec. 84(12):4379-84. [Medline].

  19. Gillam MP, Fideleff H, Boquete HR, Molitch ME. Prolactin excess: treatment and toxicity. Pediatr Endocrinol Rev. 2004. 2:108-114. [Medline].

  20. Giustina A, Barkan A, Casanueva FF, et al. Criteria for cure of acromegaly: a consensus statement. J Clin Endocrinol Metab. 2000 Feb. 85(2):526-9. [Medline].

  21. Gsponer J, De Tribolet N, Deruaz JP, et al. Diagnosis, treatment, and outcome of pituitary tumors and other abnormal intrasellar masses. Retrospective analysis of 353 patients. Medicine (Baltimore). 1999. 78:236-269. [Medline].

  22. Herman V, Fagin J, Gonsky R, et al. Clonal origin of pituitary adenomas. J Clin Endocrinol Metab. 1990 Dec. 71(6):1427-33. [Medline].

  23. Herman-Bonert VS, Zib K, Scarlett JA, Melmed S. Growth hormone receptor antagonist therapy in acromegalic patients resistant to somatostatin analogs. J Clin Endocrinol Metab. 2000 Aug. 85(8):2958-61. [Medline]. [Full Text].

  24. Howell DL, Wasilewski K, Mazewski CM, et al. The use of high-dose daily cabergoline in an adolescent patient with macroprolactinoma. J Pediatr Hematol Oncol. 2005. 27:326-329. [Medline].

  25. Joshi SM, Hewitt RJ, Storr HL, et al. Cushing's disease in children and adolescents: 20 years of experience in a single neurosurgical center. Neurosurgery. 2005. 57:281-285. [Medline].

  26. Kanter AS, Diallo AO, Jane JA Jr, et al. Single-center experience with pediatric Cushing's disease. J Neurosurg. 2005. 103:413-420. [Medline].

  27. Kiehna EN, Keil M, Lodish M, Stratakis C, Oldfield EH. Pseudotumor cerebri after surgical remission of Cushing's disease. J Clin Endocrinol Metab. 2010. 95:1528-32. [Medline]. [Full Text].

  28. Kunwar S, Wilson CB. Pediatric pituitary adenomas. J Clin Endocrinol Metab. 1999 Dec. 84(12):4385-9. [Medline].

  29. Lafferty AR, Chrousos GP. Pituitary tumors in children and adolescents. J Clin Endocrinol Metab. 1999 Dec. 84(12):4317-23. [Medline].

  30. Lonser RR, Wind JJ, Nieman LK, Weil RJ, DeVroom HL, Oldfield EH. Outcome of surgical treatment of 200 children with Cushing's disease. J Clin Endocrinol Metab. 2013 Mar. 98(3):892-901. [Medline].

  31. Melmed S, Jackson I, Kleinberg D, Klibanski A. Current treatment guidelines for acromegaly. J Clin Endocrinol Metab. 1998 Aug. 83(8):2646-52. [Medline]. [Full Text].

  32. Mindermann T, Wilson CB. Pediatric pituitary adenomas. Neurosurgery. 1995 Feb. 36(2):259-68; discussion 269. [Medline].

  33. Newman CB. Medical therapy for acromegaly. Endocrinol Metab Clin North Am. 1999 Mar. 28(1):171-90. [Medline].

  34. Ng LL, Chasalow FI, Escobar O, Blethen SL. Growth hormone isoforms in a girl with gigantism. J Pediatr Endocrinol Metab. 1999. 126:99-106. [Medline].

  35. Oliveira Mda C, Abech DD, Barbosa-Coutinho LM, Ferreira NP. Macroprolactinoma at 6 years of age: diagnostic difficulties. [Portuguese]. Arq Neuropsiquiatr. 1992. 50:397-401. [Medline].

  36. Orme SM, McNally RJ, Cartwright RA, Belchetz PE. Mortality and cancer incidence in acromegaly: a retrospective cohort study. United Kingdom Acromegaly Study Group. J Clin Endocrinol Metab. 1998 Aug. 83(8):2730-4. [Medline].

  37. Orth DN. Cushing's syndrome. N Engl J Med. 1995 Mar 23. 332(12):791-803. [Medline].

  38. Pandey P, Ojha BK, Mahapatra AK. Pediatric pituitary adenoma: a series of 42 patients. J Clin Neurosci. 2005. 12:124-127. [Medline].

  39. Rix M, Laurberg P, Hoejberg AS, Brock-Jacobsen B. Pegvisomant therapy in pituitary gigantism: successful treatment in a 12-year-old girl. Eur J Endocrinol. 2005. 153:195-201. [Medline].

  40. Saito E, Correll CU, Gallelli K, et al. A prospective study of hyperprolactinemia in children and adolescents treated with atypical antipsychotic agents. J Child Adolesc Psychopharmacol. 2004. 14:350-358. [Medline].

  41. Sakazume S, Obata K, Takahashi E, et al. Bromocriptine treatment of prolactinoma restores growth hormone secretion and causes catch-up growth in a prepubertal child. Eur J Pediatr. 2004. 163:472-474. [Medline].

  42. Smallridge RC. Thyrotropin-secreting pituitary tumors. Endocrinol Metab Clin North Am. 1987. 16:765-792. [Medline].

  43. Sotos JF. Overgrowth. Hormonal Causes. Clin Pediatr (Phila). 1996 Nov. 35(11):579-90. [Medline].

  44. Stevens JR, Kymissis PI, Baker AJ. Elevated prolactin levels in male youths treated with risperidone and quetiapine. J Child Adolesc Psychopharmacol. 2005. 15:893-900. [Medline].

  45. [Guideline] Stewart PM, Petersenn S. Rationale for treatment and therapeutic options in Cushing's disease. Best Pract Res Clin Endocrinol Metab. 2009. 23 Suppl 1:S15-22. [Medline].

  46. Storr HL, Afshar F, Matson M, et al. Factors influencing cure by transsphenoidal selective adenomectomy in paediatric Cushing's disease. Eur J Endocrinol. 2005. 152:825-833. [Medline].

  47. Tamura T, Tanaka R, Korii K, Okazaki H. Pediatric pituitary adenoma. Endocr J. 2000. 47:S95-S99. [Medline].

  48. Thorner MO, Vance ML, Laws ER. The anterior pituitary. In: Wison JD, Foster DW, Kronenberg HM, Larsen PR, eds. Williams Textbook of Endocrinology. 9th ed. Philadelphia, Pa:. WB Saunders. 1998:249-340.

  49. Trainer PJ, Drake WM, Katznelson L, et al. Treatment of acromegaly with the growth hormone-receptor antagonist pegvisomant. N Engl J Med. 2000 Apr 20. 342(16):1171-7. [Medline].

  50. van Haelst MM, Hoogeboom JJ, Baujat G, et al. Familial gigantism caused by an NSD1 mutation. Am J Med Genet A. 2005. 139:40-44. [Medline].

  51. van Haute FR, Taboada GF, Corrêa LL, Lima GA, Fontes R, Riello AP, et al. Prevalence of sleep apnea and metabolic abnormalities in patients with acromegaly and analysis of cephalometric parameters by magnetic resonance imaging. Eur J Endocrinol. 2008. 158:459-65. [Medline].

  52. Yang MH, Chuang H, Jung SM, et al. Pituitary apoplexy due to prolactinoma in a Taiwanese boy: patient report and review of the literature. J Pediatr Endocrinol Metab. 2003. 16:1301-1305. [Medline].

  53. Zimmerman D, Lteif AN. Thyrotoxicosis in children. Endocrinol Metab Clin North Am. 1998 Mar. 27(1):109-26. [Medline].

 
Previous
Next
 
Pituitary macroadenoma.
A 16-year-old boy with Cushing disease.
On the left is an unaffected patient aged 12 years. On the right is the same patient aged 13 years after developing Cushing disease.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.