Endocrine Abstracts

JOINT1706

Introduction: Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue, causing the most frequent endocrinological manifestation which is central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). Growth hormone is the most commonly affected anterior pituitary hormone.

Case Report: We report the case of an 11-year-old boy who had been treated since the age of 1.5 years for multisystemic Langerhans cell histiocytosis with chemotherapy and who was admitted for short stature. Magnetic resonance imaging showed a moderate reduction in the thickness of the extra-axial mass of the brain scythe, with no detectable abnormalities in the pituitary or pituitary stalk. Endocrinological examinations revealed a profound growth hormone deficiency and partial central hypocorticism. The patient was put on hydrocortisone and growth hormone, with a good progression in height to the mean at the age of 19. The patient did not present with diabetes insipidus nor any other antehypophyseal deficiency.

Discussion/Conclusion: Patients who develop endocrine LCH disorders are at a high risk of neurodegeneration of CNS tissueand require long-term follow-up. Despite significant advances in the knowledge of LCH in recent years, little progress has been made in preventing long-term sequelae such as those affecting the hypothalamic-pituitary system.