title: Glioneuronal tumor with neuropil-like islands: clinical, morphologic, immunohistochemical, and molecular features of three pediatric cases.
abstract: Glioneuronal tumors with neuropil-like islands are rare. The 1st reported cases were localized in the cerebral hemispheres of adults, showed homogeneous histopathologic features (infiltrating astrocytic growth and neuropil-like islands rimmed by neuronal cells), and had an unfavorable behavior. We report 3 pediatric cases (1 boy and 2 girls, ages 4, 6, and 8 years, respectively). The boy had a cerebral tumor, and the girls had a spinal tumor. The younger girl also had multiple posterior fossa lesions. The boy and older girl underwent a gross total resection. The younger girl underwent a subtotal resection of the spinal tumor; posterior fossa lesions were not surgically treated. The boy and younger girl are in complete remission at 33 and 24 months, respectively, after surgery and subsequent high-dose chemoradiotherapy. The older girl had a recurrence that was partially resected. Afterward, she started high-dose chemoradiotherapy and had an optimal radiologic response at 4 months follow up. Microscopically, the common denominator was the presence of synaptophysin-positive neuropil-like islands. One tumor showed ependymal features (pseudorosettes and punctate epithelial membrane antigen immunopositivity). Two tumors had 1p deletion. 19q deletion, MGMT gene promoter methylation, EGFR amplifications or polysomy, and EGFR, IDH1, IDH2, and TP53 genes mutation analyses yielded negative results. In conclusion, glioneuronal tumor with neuropil-like islands can affect children, arise in the spinal cord, and show ependymal features in its glial component. A high-dose chemoradiotherapy program is effective.
authors.affiliation: Pathology Unit, Anna Meyer Children's Hospital, Florence, Italy. ambuccoliero@unifi.it
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Buccoliero
authors.forename: Francesca
authors.initials: F
authors.lastname: Castiglione
authors.forename: Duccio Rossi
authors.initials: DR
authors.lastname: Degl'innocenti
authors.forename: Daniela
authors.initials: D
authors.lastname: Moncini
authors.forename: Milena
authors.initials: M
authors.lastname: Paglierani
authors.forename: Iacopo
authors.initials: I
authors.lastname: Sardi
authors.forename: Laura
authors.initials: L
authors.lastname: Giunti
authors.forename: Flavio
authors.initials: F
authors.lastname: Giordano
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Sanzo
authors.forename: Federico
authors.initials: F
authors.lastname: Mussa
authors.forename: Maurizio
authors.initials: M
authors.lastname: Aricò
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Genitori
authors.forename: Gian Luigi
authors.initials: GL
authors.lastname: Taddei
mesh_terms: D001932:Brain Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009457:Neuroglia / Q000378:metabolism / Q000473:pathology*; D009474:Neurons / Q000378:metabolism / Q000473:pathology*; D019581:Neuropil / Q000378:metabolism / Q000473:pathology*; D013120:Spinal Cord Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016708:Synaptophysin / Q000032:analysis / Q000096:biosynthesis
chemical_list: D016708:Synaptophysin