Langerhans Cell Histiocytosis Involving the Hypothalamic–Pituitary System Accompanied by Adipsic Diabetes Insipidus and Multiple Anterior Pituitary Hormone Deficiencies: A Case Report
- 1Department of Gastroenterology, Nara Medical University, Kashihara 634-8521, Nara, Japan
- 2Department of Diabetes and Endocrinology, Nara Medical University, Kashihara 634-8521, Nara, Japan
Abstract
Adult-onset Langerhans cell histiocytosis (LCH) is an uncommon disease. Central nervous system involvement, particularly of the hypothalamic–pituitary complex, can provoke severe endocrine and neurological complications. We report the case of a 39-year-old female patient with a history of pulmonary LCH who developed central diabetes insipidus (CDI), anterior pituitary dysfunction, and severe hypothalamic syndrome, accompanied by adipsia, thermoregulatory dysfunction, and cognitive impairment. Brain magnetic resonance imaging revealed a hypothalamic mass with peritumoral edema. Following ineffective corticosteroid therapy, cladribine was administered. Although the tumor size and edema significantly decreased, it caused prolonged grade 4 bone marrow suppression, necessitating treatment discontinuation. Despite the marked radiological improvement, multiple anterior pituitary hormone deficiencies, cognitive impairment, and irreversible hypothalamic dysfunction persisted. Furthermore, concomitant adipsic CDI and thermoregulatory failure provoked severe dehydration, complicating clinical management. Ultimately, the patient died of sudden cardiac arrest, secondary to extreme hypernatremia (170 mEq/L) and dehydration-induced cardiopulmonary collapse. The case presented herein highlights the possible devastating consequences of hypothalamic involvement in adult multisystem LCH. While cladribine is effective at reducing the tumor burden, it fails to reverse established neuroendocrine and neurodegenerative damage. Early therapeutic and further lifelong interventions, specifically for adipsic CDI, are crucial to preventing fatal outcomes and maintaining quality of life.
Related
- Extensive vulvar and perianal langerhans cell histiocytosis with clinical improvement during low-dose thalidomide and topical therapy: a case report
- Adult-onset Langerhans cell histiocytosis in an elderly female with cutaneous and nodal involvement: A case report
- Langerhans Cell Histiocytosis of the Vulva: A Pediatric Case.
- No detectable infectious agents in Langerhans cell histiocytosis with lung involvement
- Multisystem Langerhans cell histiocytosis with hypothyroidism and sclerosing cholangitis: a case report and review of the literature.
BibTeX
@article{uejima2026langerhans,
title = {{Langerhans Cell Histiocytosis Involving the Hypothalamic–Pituitary System Accompanied by Adipsic Diabetes Insipidus and Multiple Anterior Pituitary Hormone Deficiencies: A Case Report}},
author = {Masakazu Uejima and Yukako Kurematsu and Kuniaki Ozaki and Kosuke Kaji and Kei Moriya and Tadashi Namisaki and Akira Mitoro and Hitoshi Yoshiji},
journal = {Journal of Clinical Medicine},
year = {2026},
month = oct,
doi = {10.3390/jcm15207877},
url = {https://www.mdpi.com/2077-0383/15/20/7877},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Langerhans Cell Histiocytosis Involving the Hypothalamic–Pituitary System Accompanied by Adipsic Diabetes Insipidus and Multiple Anterior Pituitary Hormone Deficiencies: A Case Report
AU - Masakazu Uejima
AU - Yukako Kurematsu
AU - Kuniaki Ozaki
AU - Kosuke Kaji
AU - Kei Moriya
AU - Tadashi Namisaki
AU - Akira Mitoro
AU - Hitoshi Yoshiji
PY - 2026
DA - 2026/10/01
JO - Journal of Clinical Medicine
DO - 10.3390/jcm15207877
UR - https://www.mdpi.com/2077-0383/15/20/7877
AB - Adult-onset Langerhans cell histiocytosis (LCH) is an uncommon disease. Central nervous system involvement, particularly of the hypothalamic–pituitary complex, can provoke severe endocrine and neurological complications. We report the case of a 39-year-old female patient with a history of pulmonary LCH who developed central diabetes insipidus (CDI), anterior pituitary dysfunction, and severe hypothalamic syndrome, accompanied by adipsia, thermoregulatory dysfunction, and cognitive impairment. Brain magnetic resonance imaging revealed a hypothalamic mass with peritumoral edema. Following ineffective corticosteroid therapy, cladribine was administered. Although the tumor size and edema significantly decreased, it caused prolonged grade 4 bone marrow suppression, necessitating treatment discontinuation. Despite the marked radiological improvement, multiple anterior pituitary hormone deficiencies, cognitive impairment, and irreversible hypothalamic dysfunction persisted. Furthermore, concomitant adipsic CDI and thermoregulatory failure provoked severe dehydration, complicating clinical management. Ultimately, the patient died of sudden cardiac arrest, secondary to extreme hypernatremia (170 mEq/L) and dehydration-induced cardiopulmonary collapse. The case presented herein highlights the possible devastating consequences of hypothalamic involvement in adult multisystem LCH. While cladribine is effective at reducing the tumor burden, it fails to reverse established neuroendocrine and neurodegenerative damage. Early therapeutic and further lifelong interventions, specifically for adipsic CDI, are crucial to preventing fatal outcomes and maintaining quality of life.
SN - 2077-0383
ER -