title: Clinicopathologic and neuroradiologic studies of papillary glioneuronal tumors.
abstract: BACKGROUND
  Papillary glioneuronal tumor (PGNT) is a rare, recently described distinct low-grade brain neoplasm. This study was performed to characterize the clinicopathologic and neuroradiologic features of PGNTs.
  
  
  METHODS
  We reviewed the medical records of 16 patients with PGNT who underwent surgery, including 11 males and five females (median age 27 years). The clinical, neuroradiologic, histopathologic, and immunohistochemical findings were documented.
  
  
  RESULTS
  Headache was the principal presentation. Neuroimaging showed contrast-enhancing, cystic-solid or cystic masses with a mural nodule, mostly involved the frontal or parietal lobes. Histologically, the tumors were characterized by glial fibrillary acidic protein (GFAP)-positive small cuboidal cells lining hyalinized vascular pseudopapillae and synaptophysin and/or NeuN-positive interpapillary neuronal elements. Other findings included small angiomatous areas in ten, small islands of neuropil and rosettes in seven, and microvascular proliferation and/or nuclear atypia in six. Mitoses or necrosis were absent. All lacked isocitrate dehydrogenase 1 (IDH1) R132H protein expression. Low expression of p53 was observed in three cases. Ki67 labeling index ranged from less than 1 to 3 %. All but one was totally resected. Median follow-up was 65 months, and one patient had tumor recurrence.
  
  
  CONCLUSIONS
  PGNTs display distinct clinicopathologic and imaging characteristics and indicate a favorable prognosis. However, recurrences sometimes occur. Immunohistochemistry facilitates the appropriate diagnosis of these tumors. Complete resection of the tumor is important for a favorable outcome.
authors.affiliation: Department of Pathology, The People's Hospital of Zhengzhou University, Zhengzhou, Henan, China, 450003.
authors.forename: Rui-Jiao
authors.initials: RJ
authors.lastname: Zhao
authors.affiliation: Division of Neuropathology and Department of Pathology, Huashan Hospital of Fudan University, 12 Wu Lu Mu Qi Zhong Road, Shanghai, 200040, China.
authors.forename: Xia-Ling
authors.initials: XL
authors.lastname: Zhang
authors.affiliation: Department of Radiology, Huashan Hospital of Fudan University, Shanghai, China, 200040.
authors.forename: Shu-Guang
authors.initials: SG
authors.lastname: Chu
authors.affiliation: Department of Radiology, The People's Hospital of Zhengzhou University, Zhengzhou, Henan, China, 450003.
authors.forename: Ming
authors.initials: M
authors.lastname: Zhang
authors.affiliation: Department of Pathology, The People's Hospital of Zhengzhou University, Zhengzhou, Henan, China, 450003.
authors.forename: Ling-Fei
authors.initials: LF
authors.lastname: Kong
authors.affiliation: Division of Neuropathology and Department of Pathology, Huashan Hospital of Fudan University, 12 Wu Lu Mu Qi Zhong Road, Shanghai, 200040, China. yinwang88@hotmail.com.
authors.forename: Yin
authors.initials: Y
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018302:Neoplasms, Neuroepithelial / Q000000981:diagnostic imaging* / Q000473:pathology; D009836:Oligodendroglia / Q000473:pathology; D049268:Positron-Emission Tomography
keywords: Histopathology; Immunohistochemistry; Neuroradiology; Papillary glioneuronal tumor; Prognosis