Langerhans Cell Histiocytosis With Hypothalamic-pituitary and Bone Involvement: A Report of 2 Cases

Abstract

Case 1: A 31-year-old woman presented with secondary amenorrhea, polyuria, and polydipsia. Three years later, magnetic resonance imaging of the brain done for headaches found thickening of the pituitary infundibulum. Laboratory evaluation indicated central vasopressin deficiency, mild hyperprolactinemia, and central hypogonadism. Six months later, progression of the infundibular lesion was documented, now contacting the optic chiasm. Biopsy showed epithelioid histiocytes, chronic inflammation, and gliosis. On postoperative scan, a lesion in the parietal calvaria was identified, which was solitary on a bone scan. The patient received cytarabine for 12 months with resolution of infundibular and bone lesion on positron emission tomography-computed tomography scan 1 year later. Case 2: A 23-year-old man presented with polyuria, polydipsia, and unilateral tinnitus. Laboratory evaluation indicated vasopressin deficiency and central hypogonadism. External ear canal biopsy indicated an infiltrative lesion with eosinophils, small lymphocytes and histiocytes. Magnetic resonance of the brain revealed hypothalamic/infundibular and parietal and mastoid bone lesions; no other lesions were identified on positron emission tomography-computed tomography. Patient received cytarabine for 1 year with resolution of lesions, which was maintained during follow-up of 4 years. Although rare, Langerhans cell histiocytosis in adults should be considered in the appropriate clinical scenario. Multidisciplinary treatment is required.