title: Central diabetes insipidus as a very late relapse limited to the pituitary stalk in Langerhans cell histiocytosis.
abstract: Central diabetes insipidus (CDI) and relapse are frequently seen in multifocal Langerhans cell histiocytosis (LCH). We present two females with multifocal LCH who developed CDI 9 and 5 years after the initial diagnosis, respectively, as a relapse limited to the pituitary stalk. Combination chemotherapy with cytarabine reduced the mass in the pituitary stalk. Although CDI did not improve, there has been no anterior pituitary hormone deficiency (APHD), neurodegenerative disease in the central nervous system (ND-CNS) or additional relapse for 2 years after therapy. It was difficult to predict the development of CDI in these cases. CDI might develop very late in patients with multifocal LCH, and therefore strict follow-up is necessary, especially with regard to symptoms of CDI such as polydipsia and polyuria. For new-onset CDI with LCH, chemotherapy with cytarabine might be useful for preventing APHD and ND-CNS.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Nakagawa
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Shinkoda
authors.forename: Daisuke
authors.initials: D
authors.lastname: Hazeki
authors.forename: Mari
authors.initials: M
authors.lastname: Imamura
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Okamoto
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Kawakami
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Kawano
mesh_terms: D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D003561:Cytarabine / Q000627:therapeutic use; D020790:Diabetes Insipidus, Neurogenic / Q000000981:diagnostic imaging / Q000209:etiology* / Q000517:prevention & control; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007564:Japan; D008279:Magnetic Resonance Imaging; D009929:Organ Size / Q000187:drug effects; D010902:Pituitary Gland / Q000000981:diagnostic imaging / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology*; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D000964:Antimetabolites, Antineoplastic; D003561:Cytarabine