Embryonal tumors with abundant neuropil and true Rosettes: A distinctive CNS primitive neuroectodermal tumor

Marco Gessi, Felice Giangaspero, Libero Lauriola, Marina Gardiman, Bernd W. Scheithauer, William Halliday, Cynthia Hawkins, Marc K. Rosenblum, Peter C. Burger, Charles G. Eberhart

Research output: Contribution to journalArticle

Abstract

Embryonal neoplasms of the central nervous system (CNS) generally arise in the early years of life and behave in a clinically aggressive manner, but vary somewhat in their microscopic appearance. Several groups have reported examples of an embryonal tumor with combined histologic features of ependymoblastoma and neuroblastoma, a lesion referred to as "embryonal tumor with abundant neuropil and true rosettes" (ETANTR). Herein, we present 22 new cases, and additional clinical follow-up on our 7 initially reported cases, to better define the histologic features and clinical behavior of this distinctive neoplasm. It affects infants and arises most often in cerebral cortex, the cerebellum and brainstem being less frequent sites. Unlike other embryonal tumors of the CNS, girls are more commonly affected than boys. On neuroimaging, the tumors appear as large, demarcated, solid masses featuring patchy or no contrast enhancement. Five of our cases (18%) were at least partly cystic. Distinctive microscopic features include a prominent background of mature neuropil punctuated by true rosettes formed of pseudo-stratified embryonal cells circumferentially disposed about a central lumen (true rosettes). Of the 25 cases with available follow-up, 19 patients have died, their median survival being 9 months. Performed on 2 cases, cytogenetic analysis revealed extra copies of chromosome 2 in both. We believe that the ETANTR represents a histologically distinctive form of CNS embryonal tumor.

Original languageEnglish
Pages (from-to)211-217
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume33
Issue number2
DOIs
Publication statusPublished - Feb 2009

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Keywords

  • Divergent differentiation
  • Embryonal tumor
  • Ependymoblastic rosettes
  • Neuropil
  • Pediatric brain tumors

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery
  • Medicine(all)

Cite this

Gessi, M., Giangaspero, F., Lauriola, L., Gardiman, M., Scheithauer, B. W., Halliday, W., Hawkins, C., Rosenblum, M. K., Burger, P. C., & Eberhart, C. G. (2009). Embryonal tumors with abundant neuropil and true Rosettes: A distinctive CNS primitive neuroectodermal tumor. American Journal of Surgical Pathology, 33(2), 211-217. https://doi.org/10.1097/PAS.0b013e318186235b