Skip to main content

Advertisement

Log in

Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl

  • Case Report
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Case report

We report a case of central diabetes insipidus, hypothyroidism, and subsequent hypopituitarism due to lymphocytic hypophysitis masking a germinoma in a 13-year-old pubertal girl. Magnetic resonance revealed an enlarged pituitary gland and a mass lesion in the pituitary stalk and inferior hypothalamus. Open cranial surgery of the anterior pituitary showed active hypophysitis with lymphocytic infiltrates but without necrosis. Despite prednisolone therapy, 1 year later an enlarged, irregular cystic mass lesion had developed; in the pituitary stalk and inferior hypothalamus, a endoscopic biopsy revealed germinoma.

Conclusion

Lymphocytic hypophysitis in children may be the first sign of a host reaction to an occult germinoma. The diagnosis of central diabetes insipidus with a thickened pituitary stalk requires long-term follow-up to establish the underlying cause.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Packer RJ, Cohen BH, Coney K (2000) Intracranial germ cell tumors. Oncologist 5:312–320

    PubMed  CAS  Google Scholar 

  2. Horowitz MB, Hall WA (1991) Central nervous system germinomas: a review. Arch Neurol 48:652–657

    PubMed  CAS  Google Scholar 

  3. Kellie SJ, Boyce H, Dunkel IJ, Diaz B, Rosenblum M, Brualdi L, Finlay J (2004) Intensive cisplatin and cyclophosphamide-based chemotherapy without radiotherapy for intracranial germinomas: failure of primary chemotherapy approach. Pediatr Blood Cancer 43:126–133

    Article  PubMed  Google Scholar 

  4. Aoyama H, Shirato H, Ikeda J, Fujieda K, Miyasaka K, Sawamura Y (2002) Induction chemotherapy followed by low-dose involved-field radiotherapy for intracranial germ cell tumors. J Clin Oncol 20:857–865

    Article  PubMed  CAS  Google Scholar 

  5. Cemeroglu AP, Blaivas M, Muraszko KM, Robertson PL, Vazquez DM (1997) Lymphocytic hypophysitis presenting with diabetes insipidus in a 14-year-old girl: case report and review of the literature. Eur J Pediatr 156:684–688

    Article  PubMed  CAS  Google Scholar 

  6. Brandes JC, Cerletty JM (1989) Pregnancy in lymphocytic hypophysitis: case report and review. Wisc Med J 88:29–32

    CAS  Google Scholar 

  7. Imura H, Nakao K, Shimatsu A, Ogawa Y, Sando T, Fujisawa I, Yamabe H (1993) Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus. New Engl J Med 329:683–689

    Article  PubMed  CAS  Google Scholar 

  8. Waki K, Yamada S, Ozawa Y, Seki K, Endo Y (1999) A case of lymphocytic infundibuloneurohypophysitis: histopathological studies. Pituitary 1:285–290

    Article  PubMed  CAS  Google Scholar 

  9. Fehn M, Bettendorf M, Ludecke DK, Sommer C, Saeger W (1999) Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl—a case report. Pituitary 1:303–307

    Article  PubMed  CAS  Google Scholar 

  10. Mootha SL, Barkovich AJ, Grumbach MM, Edwards MS, Gitelman SE, Kaplan SL, Conte FA (1997) Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening and the occult intracranial germinoma in children and adolescents. J Clin Endocrinol Metab 82:1362–1367

    Article  PubMed  CAS  Google Scholar 

  11. Schmitt S, Wichmann W, Martin E, Zachmann M, Schoenle EJ (1993) Pituitary stalk thickening with diabetes insipidus preceding typical manifestations of Langerhans cell histiocytosis in children. Eur J Pediatr 152:399–401

    Article  PubMed  CAS  Google Scholar 

  12. Leger J, Velasquez A, Garel C, Hassan M, Czernichow P (1999) Thickened pituitary stalk on magnetic resonance imaging in children with central diabetes insipidus. J Clin Endocrinol Metab 84:1954–1960

    Article  PubMed  CAS  Google Scholar 

  13. Bettendorf M, Fehn M, Grulich-Henn J, Selle B, Darge K, Ludecke DK, Heinrich UE, Saeger W (1999) Lymphocytic hypophysitis with central diabetes insipidus and consequent panhypopituitarism preceding a multifocal, intracranial germinoma in a prepubertal girl. Eur J Pediatr 158:288–292

    Article  PubMed  CAS  Google Scholar 

  14. Weismann E, Molenkamp G, Bohles HJ (1997) Diabetes insipidus due to hypophysitis. Horm Res 47:81–84

    Article  PubMed  Google Scholar 

  15. Thodou E, Asa SL, Kontogeorgos G, Kovacs K, Horvath E, Ezzat S (1995) Clinical case seminar: lymphocytic hypophysitis: clinicopathological findings. J Clin Endocrinol Metab 80:2302–2311

    Article  PubMed  CAS  Google Scholar 

  16. Ahmed AR, Aiello DP, Page R, Hopper K, Towfighi J, Santen RJ (1993) Necrotizing infundibulo-hypophysitis: a unique syndrome of diabetes insipidus and hypopituitarism. J Clin Endocrinol Metab 76:1499–1504

    Article  PubMed  CAS  Google Scholar 

  17. Maghnie M, Cosi G, Genovese E, Manca-Bitti ML, Cohen A, Zecca S, Tinelli C, Gallucci M, Bernasconi S, Boscherini B, Severi F, Arico M (2000) Central diabetes insipidus in children and young adults. N Engl J Med 343:998–1007

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Masaharu Akiyama.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mikami-Terao, Y., Akiyama, M., Yanagisawa, T. et al. Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl. Childs Nerv Syst 22, 1338–1343 (2006). https://doi.org/10.1007/s00381-006-0078-7

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-006-0078-7

Keywords

Navigation