Isolated Langerhans cell histiocytosis in the hypothalamic-pituitary region: a case report

• Published in: BMC endocrine disorders Vol. 19; no. 1; p. 143
• Main Authors: Zhou, Weibin, Rao, Jia, Li, Chengjiang
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 19-12-2019; BioMed Central; BMC
• Subjects: Adolescent; Autoimmune diseases; Autoimmune Hypophysitis - complications; Autoimmune Hypophysitis - drug therapy; Autoimmune Hypophysitis - pathology; Biopsy; Brain; Case Report; Case reports; Central diabetes insipidus; Central nervous system; Chemotherapy; Children; Deamino Arginine Vasopressin - therapeutic use; Dendritic cells; Diabetes insipidus; Diabetes Insipidus, Neurogenic - drug therapy; Diabetes Insipidus, Neurogenic - etiology; Diabetes Insipidus, Neurogenic - pathology; Diagnostic imaging; Differential diagnosis; Elderly patients; Family medical history; Germinoma; Glucocorticoids; Glucose; Growth hormones; Histiocytosis; Histiocytosis, Langerhans-Cell - complications; Histiocytosis, Langerhans-Cell - drug therapy; Histiocytosis, Langerhans-Cell - pathology; Hormones; Humans; Hypothalamic Diseases - complications; Hypothalamic Diseases - drug therapy; Hypothalamic Diseases - pathology; Hypothalamus; Immunohistochemistry; Isolated hypothalamic-pituitary lesion; Langerhans cell histiocytosis; Magnetic resonance imaging; Male; Morphology; NMR; Nodules; Nuclear magnetic resonance; Patients; Pituitary; Pituitary (posterior); Pituitary Diseases - complications; Pituitary Diseases - drug therapy; Pituitary Diseases - pathology; Pituitary gland; Pituitary hormones; Pituitary stalk thickening; Polydipsia; Polyuria; Prednisone; Prednisone - therapeutic use; Rare diseases; Sarcoidosis; Skin; Sodium; Somatotropin; Surgery; Thyroid gland; Urination disorders; Urine; Vasopressin; Youth; Central diabetes insipidus; Langerhans cell histiocytosis; Pituitary stalk thickening; Isolated hypothalamic-pituitary lesion
• ISSN: 1472-6823; 1472-6823
• DOI: 10.1186/s12902-019-0474-0

Langerhans cell histiocytosis (LCH) is a rare disease that mainly affects children, but this disease is significantly rarer in patients who are older than 15 years. In this disease, any organ can be involved. The skeleton, skin and lung are commonly affected, and isolated hypothalamic-pituitary (HP) involvement is relatively rare. Here we report a 17-year-old adolescent with isolated HP-LCH of enlarged pituitary stalk presented with central diabetes insipidus (CDI).
A 17-year-old male adolescent with polydipsia and polyuria accompanied with elevated serum sodium level and low urine osmolality for 3 weeks was referred to our hospital. After admission, hormonal evaluation showed that his growth hormone (GH) was slightly elevated, and serum osmolality and glucose were normal. The fluid deprivation-vasopressin test demonstrated CDI. Imaging examination showed an obvious thickening of the pituitary stalk. Lymphocytic hypophysitis, sarcoidosis and granulation tissue lesions were suspected. After oral 1-deamino-8-Darginine vasopressin (DDAVP) and prednisone were administered for 2 months, symptoms were relieved, and he discontinued taking the drugs by himself. On reexamination, imaging revealed changes in the size and shape of the pituitary stalk, with thickened nodules. Then, a diagnostic biopsy of the pituitary stalk lesion was performed. Immunohistochemistry confirmed the definitive diagnosis of LCH. The clinical symptoms subsided with oral hormone replacements.
CDI is a rare symptom in children and adolescents. Most of the causes are idiopathic, while others are caused by central nervous system (CNS) disorders. Meanwhile, lymphocytic hypophysitis, germinoma, LCH and other CNS disorders can all present as thickening of the pituitary stalk, diffuse enlargement of the pituitary gland, and weakening of high signal intensity in the neurohypophysis on magnetic resonance imaging (MRI). The differential diagnosis among these diseases depends on immunohistochemistry evidence.