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CC BY 4.0 · Surg J (N Y) 2025; 11: a27487991
DOI: 10.1055/a-2748-7991
Case Report

Langerhans Cell Histiocytosis of Skull Base: A Rare Cause of Central Diabetes Insipidus in Young Adults

Authors

  • Guive Sharifi

    1   Department of Neurosurgery, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
    2   Division of Neurosurgery, Skull Base Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

  • Esmaeil Mohammadi 

    3   Department of Neurosurgery, Tehran University of Medical Sciences, Tehran, Iran

  • Nader A. Dilmaghani

    4   Department of Otolaryngology, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

  • Mohammad Ansari

    2   Division of Neurosurgery, Skull Base Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

  • Elham Paraandavaji 

    2   Division of Neurosurgery, Skull Base Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

  • Zahra Davoudi

    5   Department of Endocrinology, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

  • Yashar Goorakani 

    1   Department of Neurosurgery, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

  • Ali Jafari

    1   Department of Neurosurgery, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
    2   Division of Neurosurgery, Skull Base Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

  • Oveis Salehi

    6   Department of Internal Medicine, Tehran University of Medical Sciences, Tehran, Iran
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Abstract

Background

Langerhans cell histiocytosis (LCH) is a rare disorder that can affect multiple organs, including the brain. One of the most common manifestations of LCH in the brain is involvement of the pituitary stalk, which can lead to central diabetes insipidus (CDI).

Case Description

We retrospectively reviewed the medical records of a series of patients with LCH who presented with CDI. The patients' clinicopathology features, laboratory findings, imaging studies, and treatment outcomes were investigated. The three patients were all young adults (22–38 years old) and presented with polyuria and polydipsia. MRI scans showed thickening of the pituitary stalk in all three, while common etiologies were ruled out. Biopsies of the pituitary stalk confirmed the diagnosis of LCH. The patients were further treated with chemotherapy and steroids, and their symptoms improved.

Conclusion

This case series highlights the importance of considering LCH in the differential diagnosis of CDI, especially in young adults. Early diagnosis and treatment are essential to improve outcomes for patients with LCH.

Keywords

pituitary - LCH - diabetes insipidus - skull base - transnasal transsphenoidal surgery

Availability of Data and Material

Due to the use of identifiable material, data cannot be shared.


Patients' Consent

All patients provided informed consent for participation prior to inclusion.




Publication History

Received: 21 January 2025

Accepted: 17 November 2025

Article published online:
04 December 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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Bibliographical Record
Guive Sharifi, Esmaeil Mohammadi, Nader A. Dilmaghani, Mohammad Ansari, Elham Paraandavaji, Zahra Davoudi, Yashar Goorakani, Ali Jafari, Oveis Salehi. Langerhans Cell Histiocytosis of Skull Base: A Rare Cause of Central Diabetes Insipidus in Young Adults. Surg J (N Y) 2025; 11: a27487991.
DOI: 10.1055/a-2748-7991
 

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