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Highly anaplastic extraventricular ependymoma arising in an adult, mimicking metastatic adenocarcinoma with heavy stromal inflammation and emperiporesis
Author(s) -
Moritani Suzuko,
Kushima Ryoji,
Bamba Masamichi,
Kobayashi Tadao K.,
Oka Hideki,
Fujimoto Masato,
Hattori Takanori,
Okabe Hidetoshi
Publication year - 2003
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1046/j.1440-1827.2003.01517.x
Subject(s) - pathology , ependymoma , vimentin , cytokeratin , giant cell , biology , s100 protein , cd34 , glial fibrillary acidic protein , stromal cell , immunostaining , intermediate filament , immunohistochemistry , medicine , cell , stem cell , genetics , cytoskeleton
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.