title: Treatment of Nelson's syndrome with temozolomide.
abstract: A 64-year-old woman was previously treated for Cushing's disease with trans-sphenoidal surgery, external beam radiotherapy and bilateral adrenalectomy. Progression of an aggressive corticotroph adenoma was evident 3 years post-adrenalectomy; involvement of the clivus was treated with surgery and gamma knife radiosurgery. Tumour spread through the skull base, occiput and left ear with persistent facial pain and left ear discharge; progression continued despite second gamma knife treatment. ACTH levels peaked at 2472 and 2265 pmol/l pre- and post-hydrocortisone respectively. Treatment with temozolomide resulted in a significant improvement in symptoms, a reduction of plasma ACTH to 389 pmol/l and regression of tumour on magnetic resonance imaging scan after four cycles of treatment. We propose that temozolomide is an effective and well-tolerated therapeutic tool for the treatment of Nelson's syndrome and a useful addition to the range of therapies available to treat this condition.
authors.affiliation: Department of Endocrinology, St Bartholomew's Hospital, London, UK.
authors.forename: V J
authors.initials: VJ
authors.lastname: Moyes
authors.forename: G
authors.initials: G
authors.lastname: Alusi
authors.forename: H I
authors.initials: HI
authors.lastname: Sabin
authors.forename: J
authors.initials: J
authors.lastname: Evanson
authors.forename: D M
authors.initials: DM
authors.lastname: Berney
authors.forename: K
authors.initials: K
authors.lastname: Kovacs
authors.forename: J P
authors.initials: JP
authors.lastname: Monson
authors.forename: P N
authors.initials: PN
authors.lastname: Plowman
authors.forename: W M
authors.initials: WM
authors.lastname: Drake
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000097:blood; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use*; D003606:Dacarbazine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008875:Middle Aged; D009347:Nelson Syndrome / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D000077204:Temozolomide
chemical_list: D018906:Antineoplastic Agents, Alkylating; D003606:Dacarbazine; D000324:Adrenocorticotropic Hormone; D000077204:Temozolomide