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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Steinbok P, Flodmark O, Norman MG, Chan KW, Fryer CJ: Primary Ewing's sarcoma of the base of the skull. Neurosurgery 19: 104–107, 1986
Hadfield MG, Luo VY, Williams RL, Ward JD, Russo CP: Ewing's sarcoma of the skull in an infant. Pediatr Neurosurg 25: 100–104, 1996
Batsakis JG, El-Naggar AK: Ewing's sarcoma and primitive neuroectodermal tumors: cytogenetic cynosures seeking a common histogenesis. Adv Anat Pathol 4: 207–220, 1997
Delattre O, Zucman J, Melot T, Garau XS, Zucker JM, Lenoir GM, Ambros PF, Sheer D: The Ewing family of tumors - a subgroup of small round-cell tumors defined by specific chimeric transcripts. New Engl J Med 331: 294–299, 1994
Gold P, Freedman SO: Demonstration of tumor-specific antigens in human colonic carcinomata by immunological tolerance and absorption techniques. J Exp Med 121: 439–462, 1965
Louis DN, Hamilton AJ, Sobel RA, Ojemann RG: Pseudopsammomatous meningioma with elevated serum carcinoembryonic antigen: a true secretory meningioma. Case report. J Neurosurg 74: 129–132, 1991
Suzuki Y, Tanaka R: Carcinoembryonic antigen in patients with intracranial tumors. J Neurosurg 53: 355–360, 1980
Partington MD, Scheithauer BW, Piepgras DG: Carcinoembryonic antigen production associated with an osteolytic meningioma. Case report. J Neurosurg 82: 489–492, 1995
de Alava E, Ladanyi M, Rosai J, Gerald WL: Detection of chimeric transcripts in desmoplastic small round cell tumor and related development tumors by reverse transcriptase polymerase chain reaction: a specific diagnostic assay. Am J Pathol 147: 1584–1591, 1995
de Alava E, Kawai A, Healey JH, Fligman I, Meyers PA, Huvos AG, Gerald WL, Jhanwar SC, Argani P, Antonescu CR, Pardo-Mindan FJ, Ginsberg J, Womer R, Lawlor ER, Wunder J, Andrulis I, Sorensen PHB, Barr FG, Ladanyi M: EWS-FLI1fusion transcript structure is an independent determinant of prognosis in Ewing's sarcoma. J Clin Oncol 16: 1248–1255, 1998
Doran SE, Gebarski SS, Hoff JT: Tumors of the skull, In: Neurological Surgery. 5th edn. Youmans JR (ed), Philadelphia: WB Saunders, 1996, Vol 4, pp 2998–3023
Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN: Primary Ewing's sarcoma of the cranium. Neurosurgery 46: 62–69, 2000
Dehner LP: Primitive neuroectodermal tumor and Ewing's sarcoma. Am J Surg Pathol 17: 1–13, 1993
Fellinger EJ, Garin-Chesa P, Triche TJ, Huvos AG, Rettig WJ: Immunohistochemical analysis of Ewing's sarcoma cell surface antigen p30/32MIC2. Am J Pathol 139: 317–325, 1991
Carlotti Jr CG, Drake J, Hladky J-P, Teshima I, Becker LE, Rutka JT: Primary Ewing's sarcoma of the skull in children. Pediatr Neurosurg 31: 307–315, 1999
Scotlandi K, Serra M, Manara MC, Benini S, Sarti M, Maurici D, Lollini PL, Picci P, Bertoni F, Baldini N: Immunostaining of the p30/32MIC2 antigen and molecular detection of EWS rearrangements for the diagnosis of Ewing's sarcoma and peripheral neuroectodermal tumor. Hum Pathol 27: 408–416, 1996
Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, Salzer-Kuntschik M: MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Cancer 67: 1886–1893, 1991
Katayama Y, Kimura S, Watanabe T, Yoshino A, Koshinaga M: Peripheral-type primitive neuroectodermal tumor arising in the tentorium. J Neurosurg 90: 141–144, 1999
Sorensen PH, Liu XF, Delattre O, Rowland JM, Biggs CA, Thomas G, Triche TJ: Reverse transcriptase PCR amplification of EWS/FLI-1 fusion transcripts as a diagnostic test for peripheral primitive neuroectodermal tumors of childhood. Diag Mol Pathol 2: 147–157, 1993
Akeyson EW, McCutcheon IE, Pershouse MA, Steck PA, Fuller GN: Primitive neuroectodermal tumor of the median nerve. Case report with cytogenetic analysis. J Neurosurg 85: 163–169, 1996
Hammarström S: The carcinoembryonic antigen (CEA) family: structures, suggested functions and expression in normal and malignant tissues. Semin Cancer Biol 9: 67–81, 1999
Bajetta E, Ferrari L, Martinetti A, Celio L, Procopio G, Artale S, Zilembo N, DiBartolomeo M, Seregni E, Bombardieri E: Chromogranin A, neuron specific enolase, carcinoembryonic antigen, and hydroxyindole acetic acid evaluation in patients with neuroendocrine tumors. Cancer 86: 858–865, 1999
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Tamiya, T., Ono, Y., Daido, S. et al. Primary Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor at the Vertex of the Skull with Elevated Serum Carcinoembryonic Antigen: Case Report. J Neurooncol 52, 173–180 (2001). https://doi.org/10.1023/A:1010676521149
Issue Date:
DOI: https://doi.org/10.1023/A:1010676521149