title: Highly anaplastic extraventricular ependymoma arising in an adult, mimicking metastatic adenocarcinoma with heavy stromal inflammation and emperiporesis.
abstract: We report a case of extraventricular ependymoma arising in a 50-year-old woman that took an aggressive clinical course with recurrence three times. The initial tumor was a well-circumscribed nodule in the right temporal white matter measuring 2 cm in diameter. It showed variegated histological findings mimicking metastatic adenocarcinoma: an epithelioid arrangement of highly pleomorphic cells with pseudopapillary structures and perivascular pseudorosettes, and bizarre multinucleated giant cells with occasional emperiporesis surrounded by abundant mononuclear inflammatory cells, as well as a focal small area of conventional ependymoma. Emperiporesis and abundant mononuclear cell infiltration were not previously described in an ependymoma. The recurrent tumors predominantly showed an epithelioid pattern with frequent formation of astroblastoma-like pseudopapillary structures. Neoplastic cells were markedly atypical and had characteristic intracytoplasmic eosinophilic inclusion bodies. Much of the cells in both the initial and recurrent tumors showed a positive immunostaining for glial fibrillary acidic protein (GFAP) with accentuation to the cytoplasmic processes of the pseudorosettes and pseudopapillary structures. Epithelial membrane antigen (EMA) highlighted the epithelial differentiation of the tumor cells, while cytokeratin was completely negative. Although this tumor might be classified to at least WHO grade III from the histology and aggressive behavior, the exact grading is still controversial because of the rarity of such cases.
authors.affiliation: Department of Pathology, Saiseikai Shiga Hospital Imperial Gift Foundation Inc., Ohtsu, Japan. MoritaniS@aol.com
authors.forename: Suzuko
authors.initials: S
authors.lastname: Moritani
authors.forename: Ryoji
authors.initials: R
authors.lastname: Kushima
authors.forename: Masamichi
authors.initials: M
authors.lastname: Bamba
authors.forename: Tadao K
authors.initials: TK
authors.lastname: Kobayashi
authors.forename: Hideki
authors.initials: H
authors.lastname: Oka
authors.forename: Masato
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Takanori
authors.initials: T
authors.lastname: Hattori
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Okabe
mesh_terms: D000230:Adenocarcinoma / Q000473:pathology* / Q000556:secondary*; D001932:Brain Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D004806:Ependymoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D007249:Inflammation / Q000473:pathology; D008875:Middle Aged; D018396:Mucin-1 / Q000378:metabolism; D009364:Neoplasm Recurrence, Local; D013702:Temporal Lobe
chemical_list: D005904:Glial Fibrillary Acidic Protein; D018396:Mucin-1