title: Primary Ewing's sarcoma/peripheral primitive neuroectodermal tumor at the vertex of the skull with elevated serum carcinoembryonic antigen: case report.
abstract: A primary Ewing's sarcoma arising in the skull is relatively rare. Although a small number of case reports noted elevated carcinoembryonic antigen (CEA) in patients with primary central nervous system (CNS) neoplasms, there is no report of Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PNET) with elevated serum levels of CEA. A 7-year-old boy who had episodes of headache and vomiting had noticed a solid mass in the vertex of the head. Imaging studies revealed a large intra- and extracranial tumor at the vertex of the skull. Hematological examination demonstrated high serum levels of CEA: 91.09 ng/ml. The patient initially underwent an embolization of the bilateral middle meningeal arteries with Gelfoam particles. One week later, the patient was operated on and a subtotal resection of the tumor was performed. On histopathological and molecular genetic examination, the tumor was diagnosed as a Ewing's sarcoma/peripheral PNET. Immunohistochemical study showed strongly positive staining for CEA in the tumor cells. The serum level of CEA was normalized at 0.83 ng/ml after the tumor was removed and the boy underwent radiotherapy and 3 courses of chemotherapy. This is the first reported case of a primary Ewing's sarcoma/peripheral PNET at the vertex of the skull with elevated serum CEA.
authors.affiliation: Department of Neurological Surgery, Okayama University Medical School, Japan. ttamiya@md.okayama-u.ac.jp
authors.forename: T
authors.initials: T
authors.lastname: Tamiya
authors.forename: Y
authors.initials: Y
authors.lastname: Ono
authors.forename: S
authors.initials: S
authors.lastname: Daido
authors.forename: K
authors.initials: K
authors.lastname: Tokunaga
authors.forename: S
authors.initials: S
authors.lastname: Hamazaki
authors.forename: A
authors.initials: A
authors.lastname: Kawai
authors.forename: T
authors.initials: T
authors.lastname: Ohmoto
mesh_terms: D002272:Carcinoembryonic Antigen / Q000032:analysis / Q000097:blood*; D002648:Child; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D012512:Sarcoma, Ewing / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D012888:Skull Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D014178:Translocation, Genetic
chemical_list: D002272:Carcinoembryonic Antigen