Pituitary Tumors: Pituitary Adenomas and Langerhans Cell Histiocytosis Associated Diabetes Insipidus

  • Ralph P. ErmoianEmail author


Pituitary adenomas are rare in children, with corticotropinomas being the most common pituitary adenoma in pre-pubescent children and prolactinomas being the most common pituitary adenoma in adolescents. In children, the primary management of these tumors is surgical or medical, with radiation reserved for cases of uncontrolled functioning tumors or patients with unresectable residual disease. Diabetes insipidus (DI) is a common manifestation of Langerhans cell histiocytosis (LCH). There is limited data to support early radiation therapy in patients with LCH to the hypothalamic pituitary axis to increase the probability of resolution of DI.


  1. Badalian-Very G, Vergilio JA, Degar BA et al (2010) Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 116(11):1919–1923CrossRefPubMedPubMedCentralGoogle Scholar
  2. Badalian-Very G, Vergilio JA, Fleming M et al (2013) Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol 8:1–20CrossRefPubMedGoogle Scholar
  3. Barker FG, Klibanski A, Swearingen B (2003) Transsphenoidal surgery for pituitary tumors in the United States, 1996-2000: mortality, morbidity, and the effects of hospital and surgeon volume. Clin Endocrinol Metab 88(10):4709–4719CrossRefGoogle Scholar
  4. Batista D, Courkoutsakis NA, Oldfield EH et al (2005) Detection of adrenocorticotropin-secreting pituitary adenomas by magnetic resonance imaging in children and adolescents with cushing disease. J Clin Endocrinol Metab 90(9):5134–5140CrossRefPubMedGoogle Scholar
  5. Bhatia S, Nesbit ME Jr, Egeler RM et al (1997) Epidemiologic study of Langerhans cell histiocytosis in children. J Pediatr 130(5):774–784CrossRefPubMedGoogle Scholar
  6. Castinetti F, Morange I, Dufour H et al (2009) Radiotherapy and radiosurgery in acromegaly. Pituitary 12(1):3–10CrossRefPubMedGoogle Scholar
  7. Chellapandian D, Shaikh F, van den Bos C et al (2015) Management and outcome of patients with Langerhans cell histiocytosis and single-bone CNS-risk lesions: a multi-institutional retrospective study. Pediatr Blood Cancer 62(12):2162–2166CrossRefPubMedGoogle Scholar
  8. Ding D, Starke RM, Sheehan JP (2014) Treatment paradigms for pituitary adenomas: defining the roles of radiosurgery and radiation therapy. J Neuro-Oncol 117(3):445–457CrossRefGoogle Scholar
  9. Donadieu J, Rolon MA, Thomas C et al (2004) Endocrine involvement in pediatric-onset Langerhans’ cell histiocytosis: a population-based study. J Pediatr 144(3):344–350CrossRefPubMedGoogle Scholar
  10. Dunger DB, Broadbent V, Yeoman E et al (1989) The frequency and natural history of diabetes insipidus in children with Langerhans-cell histiocytosis. N Engl J Med 321(17):1157–1162CrossRefPubMedGoogle Scholar
  11. Friedman TC, Zuckerbraun E, Lee ML et al (2007) Dynamic pituitary MRI has high sensitivity and specificity for the diagnosis of mild Cushing’s syndrome and should be part of the initial workup. Horm Metab Res 39(6):451–456CrossRefPubMedGoogle Scholar
  12. Grois N, Flucher-Wolfram B, Heitger A et al (1995) Diabetes insipidus in Langerhans cell histiocytosis: results from the DAL-HX 83 study. Med Pediatr Oncol 24(4):248–256CrossRefPubMedGoogle Scholar
  13. Grois N, Pötschger U, Prosch H et al (2006) Risk factors for diabetes insipidus in Langerhans cell histiocytosis. Pediatr Blood Cancer 46(2):228–233CrossRefPubMedGoogle Scholar
  14. Guyot-Goubin A, Donadieu J, Barkaoui M et al (2008) Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000-2004. Pediatr Blood Cancer 51(1):71–75CrossRefPubMedGoogle Scholar
  15. Kane LA, Leinung MC, Scheithauer BW et al (1994) Pituitary adenomas in childhood and adolescence. J Clin Endocrinol Metab 79(4):1135–1140PubMedGoogle Scholar
  16. Kanner A, Cora B, Greenman Y (2009) Radiotherapy of nonfunctioning and gonadotroph adenomas. Pituitary 12:15–22CrossRefPubMedGoogle Scholar
  17. Kanter AS, Diallo AO, Jane JA Jr (2005) Single-center experience with pediatric Cushing’s disease. J Neurosurg 103(5 Suppl):413–420PubMedGoogle Scholar
  18. Keil MF, Stratakis CA (2008) Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics. Expert Rev Neurother 8(4):563–574CrossRefPubMedPubMedCentralGoogle Scholar
  19. Kocheta R, Venkatramani R, Jubran R et al (2014) Clinical outcomes of radiation therapy in the management of Langerhans cell histiocytosis. Am J Clin Oncol 37:592–596CrossRefGoogle Scholar
  20. Kunwar S, Wilson C (1999) Pediatric pituitary adenomas. J Clin Endocrinol Metab 84(12):4385–4389CrossRefPubMedGoogle Scholar
  21. Lafferty AR, Chrousos GP (1999) Pituitary tumors in children and adolescents. J Clin Endocrinol Metab 84(12):4317–4323CrossRefPubMedGoogle Scholar
  22. Mahmoud-Ahmed AS, Suh JH (2002) Radiation therapy for Cushing’s disease: a review. Pituitary 5(3):175–180CrossRefPubMedGoogle Scholar
  23. Minkov M, Steiner M, Pötschger U et al (2008) Reactivations in multisystem Langerhans cell histiocytosis: data of the international LCH registry. J Pediatr 153(5):700–705CrossRefPubMedGoogle Scholar
  24. Mindermann T, Wilson CB (1995) Pediatric pituitary adenomas. Neurosurgery 36(2):259–268CrossRefPubMedGoogle Scholar
  25. Minehan K, Chen M, Zimmerman D et al (1992) Radiation therapy for diabetes insipidus caused by Langerhans cell histiocytosis. Int J Radiat Biol Phys 23:519–524CrossRefGoogle Scholar
  26. Patronas N, Bulakbasi N, Stratakis CA et al (2003) Spoiled gradient recalled acquisition in the steady state technique is superior to conventional postcontrast spin echo technique for magnetic resonance imaging detection of adrenocorticotropin-secreting pituitary tumors. J Clin Endocrinol Metab 88(4):1565–1569CrossRefPubMedGoogle Scholar
  27. Prosch H, Grois N, Prayer D, Waldhauser F et al (2004) Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer 43(5):594–599CrossRefPubMedGoogle Scholar
  28. Rosenzweig K, Arceci R, Tarbell N (1997) Diabetes insipidus secondary to Langerhans cell histiocytosis: is radiation therapy indicated? Med Pediatr Oncol 29:36–40CrossRefPubMedGoogle Scholar
  29. Smith DG, Nesbit ME Jr, D’Angio GJ et al (1973) Histiocytosis X: role of radiation therapy in management with special reference to dose levels employed. Radiology 106(2):419–422CrossRefPubMedGoogle Scholar
  30. Steele CA, MacFarlane IA, Blair J et al (2010) Pituitary adenomas in childhood, adolescence and young adulthood: presentation, management, endocrine and metabolic outcomes. Eur J Endocrinol 163(4):515–522CrossRefPubMedGoogle Scholar
  31. Stratakis CA (2012) Cushing syndrome in pediatrics. Endocrinol Metab Clin N Am 41(4):793–803CrossRefGoogle Scholar
  32. Tarapore PE, Sughrue ME, Blevins L et al (2011) Microscopic endonasal transsphenoidal pituitary adenomectomy in the pediatric population. J Neurosurg Pediatr 7(5):501–509CrossRefPubMedGoogle Scholar
  33. Tritos NA, Biller BM (2015) Update on radiation therapy in patients with Cushing’s disease. Pituitary 18(2):263–268CrossRefPubMedGoogle Scholar
  34. Wattson DA, Tanguturi SK, Spiegel DY et al (2014) Outcomes of proton therapy for patients with functional pituitary adenomas. Int J Radiat Oncol Biol Phys 90(3):532–539CrossRefPubMedGoogle Scholar
  35. Webb C, Prayson RA (2008) Pediatric pituitary adenomas. Arch Pathol Lab Med 132(1):77–80PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Radiation OncologyUniversity of Washington (UWMC)SeattleUSA

Personalised recommendations