title: Polar spongioblastoma of the spinal cord: a case report.
abstract: "Rhythmic palisading" is a striking histologic pattern infrequently encountered in a variety of central nervous system (CNS) tumors. We present the case of an infant with a large spinal cord lesion wherein all sampled tissue showed columnar arrangements of palisaded cells, typical of polar spongioblastoma. The tumor was briskly proliferative, focally necrotic, and variably expressed S100, glial fibrillary acidic protein, neuron specific enolase, and p53 by immunohistochemistry. Fluorescence in situ hybridization failed to reveal isochromosome 17q, EGFR amplification, or deletions of 1p, 19q, 22q11.2, 10q, or p16. Despite chemotherapy and decadron, he developed lesional necrosis and intracranial metastases and died less than 1 mo from presentation. This case illustrates polar spongioblastoma as a distinctive histologic pattern that can occur in embryonal CNS tumors. Discrimination of these rare aggressive lesions from other CNS tumors with focal palisaded architecture is crucial as the treatment and prognosis of the latter may differ significantly.
authors.affiliation: Department of Pathology, St Jude Children's Research Hospital, 332 N Lauderdale Street, Memphis, TN 38105, USA. christine.fuller@stjude.org
authors.forename: Christine
authors.initials: C
authors.lastname: Fuller
authors.forename: Kathleen
authors.initials: K
authors.lastname: Helton
authors.forename: James
authors.initials: J
authors.lastname: Dalton
authors.forename: Maryam
authors.initials: M
authors.lastname: Fouladi
authors.forename: Larry
authors.initials: L
authors.lastname: Kun
authors.forename: Peter
authors.initials: P
authors.lastname: Burger
mesh_terms: D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D049109:Cell Proliferation; D003907:Dexamethasone / Q000627:therapeutic use; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D018302:Neoplasms, Neuroepithelial / Q000737:chemistry / Q000188:drug therapy / Q000473:pathology*; D011859:Radiography; D013116:Spinal Cord / Q000000981:diagnostic imaging / Q000473:pathology*; D013120:Spinal Cord Neoplasms / Q000737:chemistry / Q000188:drug therapy / Q000473:pathology*
chemical_list: D018931:Antineoplastic Agents, Hormonal; D014408:Biomarkers, Tumor; D003907:Dexamethasone