The pituitary gland in patients with Langerhans cell histiocytosis: a clinical and radiological evaluation

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease in which the most common endocrine manifestation is diabetes insipidus (DI). Data on anterior pituitary function in patients with LCH are limited. Thus, the present study investigated anterior pituitary function in LCH patients with DI via the evaluation of clinical and radiological findings at disease onset and during follow-up. The present study retrospectively evaluated nine patients with LCH (five males and four females). All diagnoses of LCH were made following histological and/or immunophenotypic analyses of tissue biopsies, bronchoalveolar lavage, or cerebrospinal fluid (CSF). Basal and, if necessary, dynamic pituitary function tests were used to assess anterior pituitary function, and magnetic resonance imaging (MRI) scans were used to image the pituitary. The LCH treatment modality was based on organ involvement. The mean age at onset of DI was 27.6 years (range 15-60 years). One patient (11%) exhibited single organ involvement, while eight patients (89%) displayed multisystem organ involvement. On admittance, one patient had hypogonadotropic hypogonadism, one patient exhibited panhypopituitarism [hypogonadotropic hypogonadism, central hypothyroidism, hypocortisolism, and growth hormone (GH) deficiency], and four patients (44%) displayed hyperprolactinemia. The MRI data revealed infundibular enlargement in seven patients (78%), a thalamic mass in one patient (11%), and the absence of the bright spot in all patients. A single patient (11%) showed a mass in the pons that had a partially empty sella. The patients were treated with radiation therapy (RT), chemotherapy (CT), or a combination of both (RT+CT) and were followed up for a median of 91.8 months (range 2-318 months). Seven patients were assessed during the follow-up period, of whom four patients (57.1%) developed anterior pituitary hormone deficiency, three (43%) were diagnosed with GH deficiency, and one (14%) exhibited gonadotropin deficiency. The gonadotropin deficiency in the patient, which was diagnosed on admittance, was resolved during the follow-up period. DI persisted in all patients, and the conditions of the seven patients who have remained on follow-up are stable. In the present study, patients with LCH exhibited altered function in the anterior pituitary as well as the posterior pituitary, which may be due to the natural course of the disease or the effects of treatment. The present findings indicate that anterior pituitary function should be assessed in LCH patients on admittance and during follow-up, especially in LCH patients with multisystem organ involvement.

Langerhans cell histiocytosis and pituitary function

“…In two patients, MRI showed some improvement in the infundibular thickening but persisted in the remaining. 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].…”

“…In the current issue, a further retrospective study of nine patients with LCH (six adults), seven of whom presented with DI and the remaining two developed DI during the course of the disease (eight had multisystem disease), were followed up for a period of 92 months [9]. On initial assessment, two patients were found to have anterior pituitary hormonal dysfunction (one hypogonadism and the other panhypopituitarism), and four had elevated PRL levels.…”

“…On initial assessment, two patients were found to have anterior pituitary hormonal dysfunction (one hypogonadism and the other panhypopituitarism), and four had elevated PRL levels. Hypothalamic-pituitary MRI imaging was abnormal in all; seven patients (78 %) had infundibular enlargement, one a thalamic mass, and the remaining absence of the bright spot of the posterior pituitary [9]. Seven patients were assessed during the follow-up period four of whom developed anterior pituitary deficiency (57 %); three developed GH and one gonadotrophin deficiency.…”

The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease

“…The prevalence of DI in ECD ranges from 25% to 50% and may precede the diagnosis by many years. [55][56][57] It is associated with anterior pituitary hormonal deficiencies in more than 50% of patients, 58,59 and patients can have anterior hormone deficiencies without DI. In one study, the most common anterior pituitary deficit was growth hormone (79%), followed by gonadotropin (22%), thyrotropin (10%), and corticotropin (3%) deficiencies.…”

Imaging of Nervous System Involvement in Hematologic Malignancies: What Radiologists Need to Know

“…The common brain MRI findings in LCH are loss of normal T1 hyperintensity of the posterior pituitary gland, infundibular thickening with enhancement, and hypothalamic involvement [71]. Other manifestations include pineal gland enlargement and cystic changes and enhancing meningeal and choroid plexus masses.…”