Histiocytosis x of the hypothalamus

Abstract

An unusual case of cutaneous and hypothalamic histiocytosis X (HX) is reported. The hypothalamic involvement occurred as a tumor that mimicked a chiasm glioma on computed tomography angioscanning. Magnetic resonance imaging after gadolinium injection localized the tumor within the third ventricle floor. The HX origin of the tumor was confirmed by histological examination of hypothalamic biopsies obtained by transventricular endoscopy. The results of endocrine evaluation were consistent with anterior panhypopituitarism resulting from a multiple releasing-hormone secretory defect, but there was no diabetes insipidus. This unusual endocrine aspect has not been previously described in the field of hypothalamic HX. Lastly, the tumor was insensitive to low dose megavoltage radiation therapy. This unusual case stresses the superiority of magnetic resonance imaging over computed tomography scanning in the assessment of suprasellar tumors and emphasizes the usefulness of transventricular endoscopy in these cases.

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

“…Anterior pituitary hormonal deficiencies have been described in up to 20% of patients with LCH almost always associated with DI [2,3,[5][6][7][8][9]. Only a very few cases of isolated anterior pituitary hormone deficiencies, without the coexistence of DI, have been reported in the literature [10]. In cases of endocrine involvement, systemic treatment appears to be of little benefit in controlling the progression of the disease, while established endocrine abnormalities are considered to be permanent [3,11,12].…”

Langerhans Cell Histiocytosis of the Thyroid Leading to the Diagnosis of a Disseminated Form

“…Pan-hypopituitarism, as in our case, is rare. Pituitary insufficiency is explained by the lack of secretion of hormones from the hypothalamus and pituitary [4]. e incidence of LCH involving the thyroid gland, either as an isolated lesion or as a part of a multisystemic disease, is extremely rare.…”

Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review

“…There are several patients presented in the literature who had APD and DI when LCH was diagnosed [16][17][18][19]. Tabarin et al reported a patient with hypothalamic LCH who had APD without DI; however, it was not isolated since cutaneous involvement was present [20].…”