title: Rosette-forming glioneuronal tumor originating in the hypothalamus.
abstract: Rosette-forming glioneuronal tumors (RGNT) of the fourth ventricle are slow-growing tumors that primarily involve the fourth ventricular region. We here report the first patient, an 8-year-old girl, with an RGNT originating in the hypothalamus and manifesting with precocious puberty. After partial removal, the remaining tumor showed rapid enlargement, and the pathologic diagnosis at the second surgery revealed histopathologic features similar to those found in the initial samples, including biphasic patterns of neurocytic rosettes and GFAP-stained astrocytic components. These tumor cells had mildly atypical nuclei; however, mitotic figures and necrosis were absent. Eosinophilic granular bodies and a glomeruloid vasculature were found, but Rosenthal fibers were absent. The Ki-67 proliferative index was 3.5 % (vs 1.1 % at the initial surgery). No recurrence was recorded during the 3-year period after the proton radiotherapy.
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan. yamamoto_neurosurg@md.tsukuba.ac.jp.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Yamamoto
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Teppei
authors.initials: T
authors.lastname: Matsubara
authors.affiliation: Department of Pathology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
authors.forename: Kaishi
authors.initials: K
authors.lastname: Satomi
authors.affiliation: Department of Pathology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
authors.forename: Noriaki
authors.initials: N
authors.lastname: Sakamoto
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Masahide
authors.initials: M
authors.lastname: Matsuda
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Ai
authors.initials: A
authors.lastname: Muroi
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Eiichi
authors.initials: E
authors.lastname: Ishikawa
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Hiroyoshi
authors.initials: H
authors.lastname: Akutsu
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Kei
authors.initials: K
authors.lastname: Nakai
authors.affiliation: Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Matsumura
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D018303:Ganglioglioma / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D019635:Neurosurgical Procedures; D061766:Proton Therapy; D011629:Puberty, Precocious / Q000209:etiology; D011878:Radiotherapy; D012397:Rosette Formation; D011522:Protons
chemical_list: D011522:Protons
keywords: Hypothalamus; Precocious puberty; Rosette-forming glioneuronal tumor