Abstract
Introduction
Langerhans cell histiocytosis (histiocytosis X) is a rare disorder that can manifest with solitary bony lesions or disseminated disease in various tissues. A common manifestation of the disease is diabetes insipidus even in the absence of neural involvement. Disseminated disease often involves the central nervous system, but only six isolated lesions in the infundibulum have been reported.
Case report
An 18-year-old male had failed to develop secondary sexual characteristics. He also had diabetes insipidus for more than 1 year. Magnetic resonance imaging (MRI) of the brain showed a 1-cm homogeneously enhancing mass in the region of the infundibulum. No other lesions were found in other organ systems. The patient underwent an orbitozygomatic osteotomy and pterional craniotomy. The lesion, which was adherent to the infundibulum, was partially resected. Microscopic analysis showed multiple lymphocytes and eosinophils with Langerhans cells that stained positive for S-100. Langerhans cell histiocytosis was diagnosed, and the patient was discharged on postoperative day 6 with no neurological deficits. His diabetes insipidus persisted, and he underwent intensity-modulated radiation therapy to the sellar region.
Conclusions
Isolated Langerhans cell histiocytosis of the infundibulum is rare. The usual presentation is hypothalamic-pituitary axis dysfunction and diabetes insipidus. Patients with diabetes insipidus of unknown origin should undergo MRI of the sellar region to rule out infundibular abnormalities.
Similar content being viewed by others
References
Broadbent V, Pritchard J (1996) The histiocytoses. In: Weatherall DJ, Ledingham JGG, Warrell DA (eds) Oxford textbook of medicine. Oxford University Press, Oxford, pp 3606–3610
Beers GJ, Manson J, Carter AP, Bell R (1986) Intracranial histiocytosis X: a case report. J Comput Tomogr 10:237–241
O’Sullivan RM, Sheehan M, Poskitt KJ, Graeb DA, Chu AC, Joplin GF (1991) Langerhans cell histiocytosis of hypothalamus and optic chiasm: CT and MR studies. J Comput Assist Tomogr 15:52–55
Scholz M, Firsching R, Feiden W, Breining H, Brechtelsbauer D, Harders A (1995) Gagel’s granuloma (localized Langerhans cell histiocytosis) in the pituitary stalk. Clin Neurol Neurosurg 97:164–166
Asano T, Goto Y, Kida S, Ohno K, Hirakawa K (1999) Isolated histiocytosis X of the pituitary stalk. J Neuroradiol 26:277–280
Isoo A, Ueki K, Ishida T, Yoshikawa T, Fujimaki T, Suzuki I, Sasaki T, Kirino T (2000) Langerhans cell histiocytosis limited to the pituitary-hypothalamic axis—two case reports. Neurol Med Chir (Tokyo) 40:532–535
Takahashi K, Isobe T, Ohtsuki Y, Akagi T, Sonobe H, Okuyama T (1984) Immunohistochemical study on the distribution of alpha and beta subunits of S-100 protein in human neoplasm and normal tissues. Virchows Arch B Cell Pathol Incl Mol Pathol 45:385–396
Minehan KJ, Chen MG, Zimmerman D, Su JQ, Colby TV, Shaw EG (1992) Radiation therapy for diabetes insipidus caused by Langerhans cell histiocytosis. Int J Radiat Oncol Biol Phys 23:519–524
Gagel O (1941) Granulationsgeschwulst im Gebiet des Hypothalamus. Z Neurol 172:710–722
Osband ME, Pochedly C (1987) Histiocytosis-X: an overview. Hematol Oncol Clin North Am 1:1–7
Horvath E, Scheithauer BW, Kovacs K, Lloyd RV (1997) Regional neuropathology: hypothalamus and pituitary. In: Graham DI, Lantos PL (eds) Greenfield’s neuropathology. Oxford University Press, New York, pp 1007–1087
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Horn, E.M., Coons, S.W., Spetzler, R.F. et al. Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus. Childs Nerv Syst 22, 542–544 (2006). https://doi.org/10.1007/s00381-005-0022-2
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-005-0022-2