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

• Published in: International journal of hematology Vol. 94; no. 6; pp. 556 - 560
• Main Authors: Imashuku, Shinsaku, Kudo, Naoko, Kaneda, Shigehiro, Kuroda, Hiroshi, Shiwa, Tsuguka, Hiraiwa, Tetsuya, Inagaki, Atsushi, Morimoto, Akira
• Format: Journal Article
• Language: English
• Published: Japan Springer Japan 01.12.2011; Springer; Springer Nature B.V
• Subjects: Adult; Biological and medical sciences; Case Report; Endocrinopathies; Female; Hematologic and hematopoietic diseases; Hematology; Histiocytosis, Langerhans-Cell - diagnosis; Histiocytosis, Langerhans-Cell - pathology; Histiocytosis, Langerhans-Cell - therapy; Humans; Hypothalamus - pathology; Hypothalamus. Hypophysis. Epiphysis (diseases); Magnetic Resonance Imaging; Male; Medical sciences; Medicine; Medicine & Public Health; Middle Aged; Neurology; Non tumoral diseases. Target tissue resistance. Benign neoplasms; Oncology; Other diseases. Hematologic involvement in other diseases; Pituitary Gland - pathology; Treatment Outcome; Tumors of the nervous system. Phacomatoses; Young Adult; Medical oncology; Hypopituitarism; Langerhans cell histiocytosis; Hypothalamic-pituitary mass; Brain tumors; Endocrinopathy; Endocrine gland; Hemopathy; Cancerology; Age of onset; Adult; Pituitary gland; Human; Nervous system diseases; Hematology; Malignant tumor; Intracranial tumor; Cerebral disorder; Mass; Medicine; Treatment; Hypophyseal insufficiency; Pituitary diseases; Central nervous system disease; Lesion; Cancer
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-011-0955-z

We report four cases of adult-onset Langerhans cell histiocytosis (LCH) with central nervous system (CNS) lesions in the hypothalamic-pituitary region. The first clinical symptoms were diabetes insipidus (two patients), hypothyroidism (one patient), and decreased libido/erectile dysfunction (one patient). Diagnosis was delayed as the CNS lesion was not initially suspected to be secondary to LCH, with a median time from symptom onset to treatment of 3.0 (range <1–5.3) years. In three patients, the tumor mass was effectively reduced by chemotherapy; however, all patients continue to exhibit hypopituitarism. Early diagnosis and initiation of treatment are required to improve the outcome of CNS-LCH in adult patients.