title: [Multiple endocrine neoplasia type 1 syndrome with three classical components and chiasm glioma: specific features of target organ lesions and a clinical observation].
abstract: The article briefly reviews the specific features of target-organ lesions in multiple endocrine neoplasia type 1 (MEN1) syndrome and a clinical case of genetically confirmed MEN1 syndrome in a young female patient. Despite the relative rarity of this disease, timely diagnosis, treatment and screening for its main components are very important for the overall prognosis of patients with MEN1 and their first-degree relatives who are MEN1 gene mutation carriers. The described case is noteworthy for a number of specific features. The authors could find no account of optic chiasm glioma within the framework of MEN1 in the literature. Moreover, therapy-resistant somatoprolactinoma engages attention, which points to its aggressive nature with pituitary adenoma that is not been clearly visualized on magnetic resonance imaging. Of interest is the order of detection of neoplasms, in particular the manifestation of hypoglycemic episodes as a sign of organic hyperinsulinism. which have been initially regarded as epileptic seizures, after the use of sustained-release somatostatin analogues for the treatment of acromegaly.
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: N G
authors.initials: NG
authors.lastname: Mokrysheva
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: E O
authors.initials: EO
authors.lastname: Mamedova
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: E A
authors.initials: EA
authors.lastname: Pigarova
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: Yu A
authors.initials: YA
authors.lastname: Berezkina
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: A V
authors.initials: AV
authors.lastname: Vorontsov
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: V N
authors.initials: VN
authors.lastname: Azizyan
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: A Yu
authors.initials: AY
authors.lastname: Grigoriev
authors.affiliation: N.N. Blokhin Russian Cancer Research Center, Moscow, Russia.
authors.forename: T R
authors.initials: TR
authors.lastname: Alekseeva
authors.affiliation: Acad. N.N. Burdenko Research Institute of Neurosurgery, Moscow, Russia.
authors.forename: M A
authors.initials: MA
authors.lastname: Kutin
authors.affiliation: A.V. Vishnevsky Institute of Surgery, Ministry of Health of Russia, Moscow, Russia.
authors.forename: A V
authors.initials: AV
authors.lastname: Kochatkov
authors.affiliation: Endocrinology Research Center, Ministry of Health of Russia, Moscow, Russia.
authors.forename: L Ya
authors.initials: LY
authors.lastname: Rozhinskaya
mesh_terms: D000328:Adult; D005260:Female; D005910:Glioma / Q000209:etiology / Q000473:pathology*; D006801:Humans; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications*; D009897:Optic Chiasm / Q000473:pathology*; D019574:Optic Nerve Neoplasms / Q000209:etiology / Q000473:pathology*