5-Year-old boy with a clival mass: Com January 2009 case 1

Benedetta Ludovica Pettorini, Federica Novegno, Alessandro Cianfoni, Luca Massimi, Pasquale De Bonis, Giuseppe Esposito, Massimo Caldarelli, Gianpiero Tamburrini, Concezio Di Rocco, Felice Giangaspero, Libero Lauriola

Research output: Contribution to journalArticlepeer-review

Abstract

Epithelioid sarcoma is a rare tumor originating from mesenchymal cells, usually involving the extremities of young adults and is found only sporadically in the head and neck region. Only one case involving the central nervous system has been described so far. We report a 5 year-old boy with a large solid, osteolytic lesion of the clivus with a wide soft tissue component expanding into the intracranial compartment and obliterating the prepontine cistern. Histopathological examination revealed epithelioid neoplastic cells with abundant eosinophilic cytoplasm, rounded nuclei and prominent nucleoli. Areas of geographic necrosis and numerous mitoses were present. Neoplastic cells immunostained for vimentin, cytokeratin, and epithelial membrane antigen (EMA). No immunostaining was observed for glial fibrillary associated protein (GFAP), S-100, PLAP, a-fetoprotein, CD 117, CD 34, CD 31, BAF-47 (INI1). The Ki67 proliferation index exceeded 40%. These histological findings favor a diagnosis of epithelioid sarcoma. This report adds epithelioid sarcoma to the differential diagnosis of both clival tumors and pediatric skull base tumors.

Original languageEnglish
Pages (from-to)523-526
Number of pages4
JournalBrain Pathology
Volume19
Issue number3
DOIs
Publication statusPublished - Jul 2009

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology

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