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

Abstract

Langerhans cell histocytosis (LCH) is a rare disease which exhibits a particular predilection for pituitary involvement leading to diabetes insipidus (DI) and other anterior pituitary hormonal deficiencies that are usually permanent and unresponsive to treatment. We report a 35 year old woman with a 10 year history of multisystemic LCH who developed DI, mild hyperprolactinemia, gonadotrophin and partial growth hormone deficiency following a normal delivery that was accompanied with infundibular thickening on pituitary magnetic resonance imaging (MRI). Following several courses of glucocorticoid administration, that were not associated with any substantial improvement, the patient was started on estrogen replacement therapy and cabergoline. After a three year period free of further relapses she developed irregular uterine bleeding. Following estrogen and cabergoline discontinuation she resumed normal menstruation while a repeated MRI of the pituitary showed an almost normal infundibulum. Endocrine investigation revealed normal gonadotrophin axis and prolactin levels, while the patient continues to menstruate, every 30-40 days, ten months after the resumption of her menstrual cycle. This case demonstrates for the first time that LCH induced pituitary deficiencies can run a variable clinical course and even spontaneously recover.

Langerhans cell histiocytosis and pituitary function

“…Anterior pituitary hormonal deficiencies and DI persisted during the follow-up period irrespective of the treatment administered, except in one patient in whom hypogonadism resolved [9]. The results of the present study confirm previous findings and highlight the importance of assessing and following up pituitary function in patients with multisystem LCH and DI or abnormal hypothalamic-pituitary region imaging [7,8,10]. Anterior pituitary hormonal deficiencies should be diagnosed early and treated appropriately as they can affect quality of life and can also be life threatening.…”

Management of Langerhans Cell Histiocytosis (LCH)-Induced Central Diabetes Insipidus and Its Associated Endocrinological/Neurological Sequelae

“…To date, while several reports have indicated that CDI can be reversed (as discussed above), this has not been observed for APHD (2,22,23). There is one exception: Makras et al (33) reported that a 35-year-old female resumed normal menstrual cycling after steroid administration. Since the hypothalamic-pituitary space-occupying mass lesions of LCH respond very well to 2-CDA (2, 26, 34), additional studies are needed to determine whether early administration of 2-CDA could reverse APHD as well as CDI.…”

Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis

“…Although rare and controversial, some reports suggest that DI can be reversed [12,17,18]. Particularly, a previous report indicated that hypothalamic-pituitary radiation therapy can effectively reverse DI if it is provided early, namely\14 days after DI diagnosis [18].With regard to anterior pituitary dysfunction, while most reports suggest that it cannot be reversed [6,9,10], Makras et al [19] have described the case of a 35-year-old female who resumed normal menstruation following steroid administration. Case 1 became free from DDAVP several years after treatments with immediate hypothalamic-pituitary radiation therapy for the primary mass and rapid introduction of multiagents' chemotherapy for the recurred CNS mass, but not resumed menstruation.…”