Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

A patient with diabetes insipidus, anterior hypopituitarism and pituitary stalk thickening

Nature Reviews Endocrinology volume 7, pages 54–59 (2011)Cite this article

Subjects

This article has been updated

Abstract

Background. A 42-year-old woman presented to the neuroendocrine unit of a hospital with recent-onset polydipsia, polyuria and oligomenorrhea. She had no visual symptoms, head injury or history of malignancy.

Investigations. Measurement of serum sodium and osmolality (as well as urine osmolality) after water deprivation, both before and after desmopressin administration. Measurement of basal serum concentrations of pituitary hormones, insulin-like growth factor 1 and thyroid hormone, cosyntropin stimulation testing of adrenal function, and growth-hormone-releasing hormone–arginine stimulation testing. MRI of the pituitary, CT of the chest and abdomen, skeletal surveys, analyses of cerebrospinal fluid, serology and histologic examination of an excised, painful submandibular salivary gland.

Diagnosis. Central diabetes insipidus and anterior hypopituitarism secondary to Langerhans cell histiocytosis.

Management. Replacement therapies, including desmopressin, levothyroxine, cyclic estrogen with medroxyprogesterone, and growth hormone. The stalk lesion remained stable after 7 years without specific therapy.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Contrast-enhanced T1-weighted MRI images of the sella of the case patient.
Figure 2: Histologic examination of a submandibular salivary gland specimen excised from the patient.
Figure 3: Etiologies of central diabetes insipidus, on the basis of data from 332 patients.1,2,3

Change history

  • 25 November 2010

    In the version of this article initially published online, there was an omission in the Acknowledgments section. The first sentence of this section should have read: Written consent for publication was obtained from the patient. The error has been corrected in all electronic versions of the text.

References

  1. Robertson, G. L. Diabetes insipidus. Endocrinol. Metab. Clin. North Am. 24, 549–572 (1995).

    Article  CAS  PubMed  Google Scholar 

  2. Greger, N. G. et al. Central diabetes insipidus. 22 years' experience. Am. J. Dis. Child. 140, 551–554 (1986).

    Article  CAS  PubMed  Google Scholar 

  3. Czernichow, P., Pomarede, R., Basmaciogullari, A., Brauner, R. & Rappaport, R. Diabetes insipidus in children. III. Anterior pituitary dysfunction in idiopathic types. J. Pediatr. 106, 41–44 (1985).

    Article  CAS  PubMed  Google Scholar 

  4. Hamilton, B. E., Salzman, K. L. & Osborn, A. G. Anatomic and pathologic spectrum of pituitary infundibulum lesions. AJR Am. J. Roentgenol. 188, W223–W232 (2007).

    Article  PubMed  Google Scholar 

  5. Rupp, D. & Molitch, M. Pituitary stalk lesions. Curr. Opin. Endocrinol. Diabetes Obes. 15, 339–345 (2008).

    Article  PubMed  Google Scholar 

  6. Carpinteri, R., Patelli, I., Casanueva, F. F. & Giustina, A. Pituitary tumours: inflammatory and granulomatous expansive lesions of the pituitary. Best Pract. Res. Clin. Endocrinol. Metab. 23, 639–650 (2009).

    Article  CAS  PubMed  Google Scholar 

  7. Leonidas, J. C., Guelfguat, M. & Valderrama, E. Langerhans' cell histiocytosis. Lancet 361, 1293–1295 (2003).

    Article  PubMed  Google Scholar 

  8. Tatevossian, R. et al. Adults with LCH—orphans with an orphan disease. Clin. Med. 6, 404–408 (2006).

    Article  CAS  Google Scholar 

  9. Aricò, M. Langerhans cell histiocytosis in adults: more questions than answers? Eur. J. Cancer 40, 1467–1473 (2004).

    Article  PubMed  Google Scholar 

  10. Chu, T. & Jaffe, R. The normal Langerhans cell and the LCH cell. Br. J. Cancer Suppl. 23, S4–S10 (1994).

    CAS  PubMed  PubMed Central  Google Scholar 

  11. Broadbent, V., Egeler, R. M. & Nesbit, M. E. Jr. Langerhans cell histiocytosis--clinical and epidemiological aspects. Br. J. Cancer Suppl. 23, S11–S16 (1994).

    CAS  PubMed  PubMed Central  Google Scholar 

  12. Lichtenstein, L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer–Siwe disease, and Schüller–Christian disease as related manifestations of a single nosologic entity. AMA Arch. Pathol. 56, 84–102 (1953).

    CAS  PubMed  Google Scholar 

  13. Makras, P., Alexandraki, K. I., Chrousos, G. P., Grossman, A. B. & Kaltsas, G. A. Endocrine manifestations in Langerhans cell histiocytosis. Trends Endocrinol. Metab. 18, 252–257 (2007).

    Article  CAS  PubMed  Google Scholar 

  14. Nanduri, V. R., Bareille, P., Pritchard, J. & Stanhope, R. Growth and endocrine disorders in multisystem Langerhans' cell histiocytosis. Clin. Endocrinol. (Oxf.) 53, 509–515 (2000).

    Article  CAS  Google Scholar 

  15. D'Ambrosio, N., Soohoo, S., Warshall, C., Johnson, A. & Karimi, S. Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients. AJR Am. J. Roentgenol. 191, 589–597 (2008).

    Article  PubMed  Google Scholar 

  16. Makras, P. et al. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH). Neuroradiology 48, 37–44 (2006).

    Article  CAS  PubMed  Google Scholar 

  17. Aricò, M. et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur. J. Cancer 39, 2341–2348 (2003).

    Article  PubMed  Google Scholar 

  18. Kaltsas, G. A. et al. Hypothalamo-pituitary abnormalities in adult patients with langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment. J. Clin. Endocrinol. Metab. 85, 1370–1376 (2000).

    Article  CAS  PubMed  Google Scholar 

  19. Wnorowski, M. et al. Pattern and course of neurodegeneration in Langerhans cell histiocytosis. J. Pediatr. 153, 127–132 (2008).

    Article  PubMed  Google Scholar 

  20. Imashuku, S. et al. Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group. Haematologica 93, 615–618 (2008).

    Article  PubMed  Google Scholar 

  21. Dhall, G. et al. Analysis of outcome for patients with mass lesions of the central nervous system due to Langerhans cell histiocytosis treated with 2-chlorodeoxyadenosine. Pediatr. Blood Cancer 50, 72–79 (2008).

    Article  PubMed  Google Scholar 

  22. Donadieu, J. et al. Incidence of growth hormone deficiency in pediatric-onset Langerhans cell histiocytosis: efficacy and safety of growth hormone treatment. J. Clin. Endocrinol. Metab. 89, 604–609 (2004).

    Article  CAS  PubMed  Google Scholar 

  23. Makras, P. et al. Spontaneous gonadotrophin deficiency recovery in an adult patient with Langerhans cell histiocytosis (LCH). Pituitary 8, 169–174 (2005).

    Article  PubMed  Google Scholar 

  24. Varan, A. et al. Radiological evaluation of patients with pituitary langerhans cell histiocytosis at diagnosis and at follow-up. Pediatr. Hematol. Oncol. 25, 567–574 (2008).

    Article  PubMed  Google Scholar 

  25. Cook, D. M. et al. American Association of Clinical Endocrinologists medical guidelines for clinical practice for growth hormone use in growth hormone-deficient adults and transition patients—2009 update: executive summary of recommendations. Endocr. Pract. 15, 580–586 (2009).

    Article  PubMed  Google Scholar 

  26. Biller, B. M. et al. Sensitivity and specificity of six tests for the diagnosis of adult GH deficiency. J. Clin. Endocrinol. Metab. 87, 2067–2079 (2002).

    Article  CAS  PubMed  Google Scholar 

  27. Yuen, K. C., Biller, B. M., Molitch, M. E. & Cook, D. M. Clinical review: Is lack of recombinant growth hormone (GH)-releasing hormone in the United States a setback or time to consider glucagon testing for adult GH deficiency? J. Clin. Endocrinol. Metab. 94, 2702–2707 (2009).

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

Written consent for publication was obtained from the patient. L. Barclay, freelance writer and reviewer, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

Author information

Authors and Affiliations

  1. Neuroendocrine Unit, Massachusetts General Hospital, Zero Emerson Place, Suite 112, Boston, 02114, MA, USA

    Nicholas A. Tritos & Anne Klibanski

  2. Department of Neurology, Massachusetts General Hospital, 55 Fruit Street, Boston, 02114, MA, USA

    Thomas N. Byrne

  3. Department of Pathology, Massachusetts General Hospital, 55 Fruit Street, Boston, 02114, MA, USA

    Chin-Lee Wu

Authors
  1. Nicholas A. Tritos
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Thomas N. Byrne
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Chin-Lee Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Anne Klibanski
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors researched the data for the article, provided a substantial contribution to discussions of the content, contributed equally to writing the article and reviewed and/or edited the manuscript before submission.

Corresponding author

Correspondence to Nicholas A. Tritos.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Tritos, N., Byrne, T., Wu, CL. et al. A patient with diabetes insipidus, anterior hypopituitarism and pituitary stalk thickening. Nat Rev Endocrinol 7, 54–59 (2011). https://doi.org/10.1038/nrendo.2010.198

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/nrendo.2010.198

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing