Horm Metab Res 2006; 38(11): 746-751
DOI: 10.1055/s-2006-955086
Original Clinical

© Georg Thieme Verlag KG Stuttgart · New York

Endocrine Disorders in Pediatric - Onset Langerhans Cell Histiocytosis

M. C. M. Amato 1 , L. L. K. Elias 1 , J. Elias 2 , A. C. D. Santos 2 , A. D. Bellucci 2 , A. C. Moreira 1 , M. De Castro 1
  • 1Division of Endocrinology
  • 2Division of Radiology, Department of Internal Medicine, School of Medicine of Ribeirao Preto, University of Sao Paulo, Sao Paulo, Brazil
Further Information

Publication History

Received 27 March 2006

Accepted after revision 7 June 2006

Publication Date:
16 November 2006 (online)

Abstract

Langerhans Cell Histiocytosis (LCH) is a rare disorder with a great variety of clinical manifestations. The purpose of this retrospective study was to evaluate the pattern and the long-term course of clinical, laboratorial and radiological findings in pediatric-onset LCH. We reviewed 46 children with histological diagnosis of LCH. Ten children (22%) showed endocrine disorders. Central diabetes insipidus (DI) was observed in all ten patients; GH deficiency was confirmed in four and hypogonadism in two children. There were no adrenal, prolactin or thyroid axis abnormalities. Obesity was observed in three patients. Eight patients showed soft tissue infiltration and five bone involvement. The MRI showed a lack of posterior pituitary bright spot in all DI patients; infundibular infiltration (II) associated or not with sellar or supra-sellar mass was observed in 4 patients. We conclude that the investigation of LCH, a multi-systemic disease, should include central nervous system images. The presence of II and/or DI should raise the diagnosis of LCH. Complete endocrine evaluation, allowing an early hormone therapy, is required to obtain a better quality of life in children with LCH.

References

  • 1 Lichtenstein L. Histiocytosis X. Integration of eosinophilic granuloma of bone, Letterer-Siwe disease and Schuller-Christian disease as related manifestations of a single nosological entity.  Arch Pathol. 1953;  56 84-102
  • 2 Osband ME. Histiocytosis X. Langerhans' cell histiocytosis.  Hematol Oncol Clin North Am. 1987;  1 737-751
  • 3 Kilborn TN, Teh J, Goodman TR. Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings.  Clin Radiol. 2003;  58 269-278
  • 4 Nicholson HS, Egeler RM, Nesbit ME. The epidemiology of Langerhans cell histiocytosis.  Hematol Oncol Clin North Am. 1998;  12 379-384
  • 5 Modan-Moses D, Weintraub M, Meyerovitch J, Segal-Lieberman G, Bielora B. Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.  J Endocrinol Invest. 2001;  24 612-617
  • 6 Kaltsas GA, Powles TB, Evanson J, Plowman PN, Drinkwater JE, Jenkins PJ, Monson JP, Besser GM, Grossman AB. Hypothalamo-pituitary abnormalities in adult patients with langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment.  J Clin Endocrinol Metab. 2000;  85 1370-1376
  • 7 Egeler RM, D'Angio GJ. Langerhans'-cell histiocytosis.  J Pediatr. 1995;  127 1-11
  • 8 Gramatovici R, D'Angio GJ. Radiation therapy in soft-tissue lesions in histiocytosis X (Langerhans' cell histiocytosis).  Med Pediatr Oncol. 1988;  16 259-262
  • 9 Lieberman PH, Jones CR, Steinman RM, Erlandson RA, Smith J, Gee T, Huvos A, Garin-Chesa P, Filippa DA, Urmacher C, Gangi MD, Sperber M. Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years.  Am J Surg Pathol. 1996;  20 519-552
  • 10 Kepes J. Histiocytosis X. In: Vinken PJ, Bruyn G (ed). Handbook of Clinical Neurology. Amsterdam: Elsevier/North Holland Biomedical Press 1979: 93-117
  • 11 Dunger DB, Broadbent V, Yeoman E, Seckl JR, Lightman SL, Grant DB, Pritchard J. The frequency and natural history of diabetes insipidus in children with Langerhans-cell histiocytosis.  N Engl J Med. 1989;  321 1157-1162
  • 12 Grois NG, Favara BE, Mostbeck GH, Prayer D. Central nervous system disease in Langerhans cell histiocytosis.  Hematol Oncol Clin North Am. 1998;  12 287-305
  • 13 Malpas JS. Langerhans cell histiocytosis in adults.  Hematol Oncol Clin North Am. 1998;  12 259-268
  • 14 Grois N, Flucher-Wolfram B, Heitger A, Mostbeck GH, Hofmann J, Gadner H. Diabetes insipidus in Langerhans cell histiocytosis: results from the DAL-HX 83 study.  Med and Pediatr Oncol. 1995;  24 248-256
  • 15 Meyer JS, Harty MP, Mahboubi S, Heyman S, Zimmerman RA, Womer RB, Dormans JP, D'Angio GJ. Langerhans cell histiocytosis: presentation and evolution of radiologic findings with clinical correlation.  Radiographics. 1995;  15 1135-1146
  • 16 Castro M, Elias PC, Quidute AR, Halah FP, Moreira AC. Out-patient screening for Cushing's syndrome: the sensitivity of the combination of circadian rhythm and overnight dexamethasone suppression salivary cortisol tests.  J Clin Endocrinol Metab. 1999;  84 878-882
  • 17 James PT, Leach R, Kalamara E, Shayeghi M. The worldwide obesity epidemic.  Obes Res. 2001;  9 228S-233S
  • 18 Nanduri VR, Bareille P, Pritchard J, Stanhope R. Growth and endocrine disorders in multisystem Langerhans' cell histiocytosis.  Clin Endocrinol. 2000;  53 509-515
  • 19 Rosenzweig KE, Arceci RJ, Tarbell NJ. Diabetes insipidus secondary to Langerhans cell histiocytosis: is radiation therapy indicated?.  Med and Pediatr Oncol. 1997;  29 36-40
  • 20 Broadbent V, Gadner H. Current therapy for Langerhans cell histiocytosis.  Hematol Oncol Clin North. 1998;  12 327-338
  • 21 Prosch H, Grois N, Prayer D, Waldhauser F, Steiner M, Minkov M, Gadner H. Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis.  Pediatr Blood Cancer. 2004;  43 594-599
  • 22 Maghnie M, Genovese E, Arico M, Villa A, Beluffi G, Campani R, Severi F. Evolving pituitary hormone deficiency is associated with pituitary vaculopathy: dynamic MR study in children with hypopituitarism, diabetes insipidus, and Langerhans cell histiocytosis.  Radiology. 1994;  193 493-499
  • 23 Gadner H, Heitger A, Grois N, Gatterer-Menz I, Ladisch S. Treatment strategy for disseminated Langerhans cell histiocytosis.  Med and Pediatr Oncol. 1994;  23 72-80
  • 24 Maghnie M, Villa A, Arico M. Correlation between magnetic resonance imaging of posterior pituitary and neurohypophysal function in children with diabetes insipidus.  J Clin Endocrinol Metab. 1992;  74 795-800
  • 25 Scherbaum WA, Wass JAH, Besser GM, Bottazzo GF, Doniach D. Autoimmune cranial diabetes insipidus: its association with oder endocrine diseases and with histiocytosis X.  Clin Endocrinol. 1986;  25 411-420
  • 26 Donadieu J, Rolon MA, Pion I, Thomas C, Doz F, Barkaoui M, Robert A, Deville A, Mazingue F, David M, Brauner R, Cabrol S, Garel C, Polak M. French LCH Study Group . Incidence of growth hormone deficiency in pediatric-onset Langerhans cell histiocytosis: efficacy and safety of growth hormone treatment.  J Clin Endocrinol Metab. 2004;  89 604-609
  • 27 Smolic EA, Devecerski M, Nelson JS, Smith Jr KR. Histiocytosis X in the optic chiasm of an adult with hypopituitarism: case report.  J Neurosurg. 1968;  24 368-372
  • 28 Bernard JD, Aguilar MJ. Localized hypothalamic histioytosis X.  Arch Neurol. 1969;  20 368-372
  • 29 Bernstrand C, Sandstedt B, Ahstrom L, Henter JI. Long-term follow-up of Langerhans cell histiocytosis: 39 years' experience at a single centre.  Acta Paediatr. 2005;  94 1073-1084
  • 30 Ha SY, Helms P, Fletcher M, Broadbent V, Pritchard J. Lung involvement in Langerhans' cell histiocytosis: prevalence, clinical features, and outcome.  Pediatrics. 1992;  89 466-469
  • 31 Smets A, Mortele K, de Praeter G, François O, Benoit Y, Kunnen M. Pulmonary and mediastinal lesions in children with Langerhans cell histiocytosis.  Pediatr Radiol. 1997;  27 873-876
  • 32 Webb DKH. Histiocytic syndromes. In: Lilleyman I, Hann I, Blanchette V (eds). Pediatric Hematology. London: Churchill Livingstone 1999: 356-361
  • 33 Willis B, Ablin A, Weinberg V, Zoger S, Wara WM, Matthay KK. Disease course and late sequelae of Langerhans' cell histiocytosis: 25-year experience at the University of California, San Francisco.  J Clin Oncol. 1996;  14 2073-2082

Correspondence

Margaret de CastroM.D., Ph.D. 

Department of Internal Medicine·School of Medicine of Ribeirao Preto·University of Sao Paulo

Av Bandeirantes 3900

14049-900 Ribeirao Preto

Sao Paulo

Brazil

Phone: +55/16/602/26 54

Fax: +55/16/633/66 95

Email: castrom@fmrp.usp.br

    >