Primitive neuroectodermal tumor of the meninges: An histological, immunohistochemical, ultrastructural, and cytogenetic study
Cite this article as: Papotti, M., Abbona, G., Pagani, A. et al. Endocr Pathol (1998) 9: 275. doi:10.1007/BF02739968 Abstract
We report a case of primitive neuroectodermal tumor (PNET) arising from the meninges in a 30-yr-old female patient whose clinical and radiological features were consistent with meningioma.
The neoplasm was composed of primitive small, round cells, growing in sheets and nests in continuity with meningeal layers. Ultrastructurally, the neoplastic cells were characterized by large and regular nuclei, primitive cytoplasm with pools of glycogen, and lack of dense core granules. The neuroectodermal nature of the tumor was confirmed by positive immunostaining for vimentin, neurofilaments, neuron specific enolase (NSE), and 013 (an antibody raised against MIC2 antigen). Further support to the diagnosis was obtained by reverse transcriptase-polymerase chain reaction (RT-PCR) detection of Chromogranin A and Secretogranin II genes. t(11;22) translocation was also observed by RT-PCR, a finding that was not recorded in previously described intracranial PNET. The tumor followed a malignant course, recurring and spreading to chest wall and sacro-iliac region over a 10-yr period. The meningeal location enlarges the topographic spectrum of intracranial PNET, and this tumor has to be considered in the differential diagnosis with meningioma.
Key Words Primitive neuroectodermal tumors meninges RT-PCR Chromogranin A Secretogranin II t(11:22) translocation References
Duckett S. Pediatric neuropathology. Baltimore, MD: Williams and Wilkins, 1995.
Hart MN, Earle KM. Primitive neuroectodermal tumors of the brain in children. Cancer 32:890–897, 1973.
Enzinger FM, Weiss SW. Soft tissues tumors. 3rd ed. St. Louis: Mosby, 1995.
Charney DA, Charney JM, Ghali VS, Teplitz C. Primitive neuroectodermal tumor of the miocardium: a case report, review of the literature, immunohistochemical and ultrastructural study. Hum Pathol 27:1365–1369, 1996.
Rosai J. Ackerman’s surgical pathology. 8th ed. St. Louis: Mosby, 1996.
Fujii M, Orita T, Aoki H, Tanaka K. Primitive neuroectodermal tumor of the leptomeninges. Neuroradiology 33:260–263, 1991.
Mendal RC, Pollay M, Bobele GB, Leech RW, Brumback RA. Primary primitive neuroectodermal tumor of the leptomeninges. J Child Neurol 11:404–407, 1996.
Chirgwing JM, Przybyla AE, MacDonald RJ, Rutter WJ. Isolation of biologically active RNA from sources enriched in ribonuclease. Biochemistry 18:5294–5297, 1979.
Pagani A, Macri’ L, Rosolen A, Toffolatti L, Stella A, Bussolati G. Neuroendocrine differentiation in Ewing’s sarcomas and primitive neuroectodermal tumors revealed by reverse transcriptase-polymerase chain reaction of chromogranins’ mRNA. Diagn Mol Pathol 1988, in press.
Nakane T, Hashizume Y, Tachibana E, Mizutani N, Handa T, Mutsuga N, Yoshida J. Primary Ewing’ sarcoma of the skull base with intracerebral extension: case report. Neurologia Medico-Chirurgica 34:628–630, 1994.
Zenke K, Hatakeyama T, Hashimoto H, Sakaki S, Manabe K. Primary Ewing’s sarcoma of the occipital bone: case report. Neurologia Medico-Chirurgica 34:246–250, 1994.
Krishan VV, Saraswathy A, Misra BK, Rout D. Primary Ewing’s sarcoma of the base of skull: a case report. Indian J Pathol Microbiol 36:477–479, 1993.
Brinkhuis M, Wjinaendts LCD, van der Linden JC, van Unnik AJM, Voute PA, Baak JPA, Meijer CJLM. Peripheral primitive neuroectodermal tumor and extraosseous Ewing’s sarcoma; a histological, immunohistochemical and DNA flow cytometric study. Virch Arch 425:611–616, 1995.
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: evidence for a common histogenesis of Ewing’s and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer 67:1886–1893, 1991.
Hamilton G, Fellinger EJ, Schratter I, Fritsch A. Characterization of a human endocrine tissue and tumour-associated Ewing’s sarcoma antigen. Cancer Res 48:6127–6143, 1988.
Cavazzana A, Ninfo V, Roberts J, Triche T. Peripheral neuroepithelioma: a light microscopic, immunocytochemical, and ultrastructural study. Mod Pathol 5:71–78, 1992.
Delattre O, Zucman J, Melot T. The Ewing family of tumors: a subgroup of small round cell tumors defined by specific chimeric transcripts. N Engl J Med 331:294–999, 1994.
Ladanyi M, Lewis R, Garin-Chesa P, Rettig WJ, Huvos AG, Healey JH, Jhanwar SC. EWS rearrangement in Ewing’s sarcoma and peripheral neuroectodermal tumor: molecular detection and correlation with cytogenetic analysis and MIC2 expression. Diagn Mol Pathol 2:141–146, 1993.
Jay V, Pienkowska M, Becker L, Zielenska M. Primitive neuroectodermal tumors of the cerebrum and cerebellum: absence of t(11;22) translocation by RT-PCR analysis. Mod Pathol 8:488–491, 1995.