Neuroendocrine manifestations of Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia that can affect any organ or system in the human body. It is usually diagnosed during childhood but can also affect adults. Recent studies have demonstrated involvement of the hypothalamo-pituitary axis (HPA) in a significant proportion of patients with deficiencies in both anterior and posterior pituitary function that in the majority of cases are permanent and require specific hormone replacement regimes. Central diabetes insipidus is considered the most frequent abnormality of HPA involvement in LCH and can be encountered either as isolated deficiency or along with other pituitary deficiencies. Complete hormonal evaluation of pituitary hormones and long-term follow-up of LCH patients are strongly recommended, especially when pituitary involvement is established.