Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis.
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.
Division of Pediatrics, Takasago-seibu Hospital, 1-10-41 Nakasuji, Takasago 676-0812, Japan. shinim95@mbox.kyoto-inet.or.jp
Shinsaku
S
Imashuku
Naoko
N
Kudo
Shigehiro
S
Kaneda
Hiroshi
H
Kuroda
Tsuguka
T
Shiwa
Tetsuya
T
Hiraiwa
Atsushi
A
Inagaki
Akira
A
Morimoto
D000328:Adult; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007031:Hypothalamus / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010902:Pituitary Gland / Q000473:pathology*; D016896:Treatment Outcome; D055815:Young Adult