The spectrum of endocrine abnormalities in patients with Langerhans cell histiocytosis (LCH)

Spontaneous Gonadotrophin Deficiency Recovery in an Adult Patient with Langerhans Cell Histiocytosis (LCH)

“…Thyreotrophin and ACTH deficiency are encountered even less frequently and usually in the context of generalized pituitary involvement [3]. Hyperprolactinemia is not consistently found and has been reported to range in approximately 17-25% of patients with LCH and DI [3,16]. The clinical and radiological features more frequently associated with anterior pituitary hormonal deficiencies in patients with LCH are multisystemic disease with skull vault defects, particularly temporal or orbital bony lesions with or without intracranial tumor extension, usually associated with abnormal findings on HPS imaging [5].…”

“…There is little information regarding gonadotrophin axis dysfunction in adult patients with LCH and pituitary involvement, overall revealing a relatively increased incidence of approximately 50% [3,5,11,16,21]. It is generally believed that established endocrine deficiencies do not respond to the various therapeutic modalities [11] and that the endocrine manifestations of LCH are non reversible [3].…”

Langerhans' cell histiocytosis in an adult patient manifested as recurrent skull lesions and Diabetes Insipidus

“…All available investigative procedures should be reviewed and a consensus on the best-evidence-based management should be agreed upon. As adults with LCH are rare, optimum management should be performed in centers with relevant experience and expertise in order to evaluate the results of current treatment, establish guidelines and develop new therapeutic trials 27 .…”