Pathologic features of extraosseous Ewing's sarcoma: A report from the intergroup rhabdomyosarcoma study

Hiroyuki Shimada, William A. Newton, Edward H. Soule, Stephen J. Qualman, Chisa Aoyama, Harold M. Maurer

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84 Scopus citations


Eighty-four cases of extraosseous Ewing's sarcoma (EOE) werefound during the pathology review of the Intergroup Rhabdomyosarcoma Study I and II. Patients commonly presented during or after adolescence with the most common primary sites including the trunk, extremities, and retroperitoneum. Males were slightly more affected. Histologic sections of 74 tumors in the pathology repository were re-reviewed with attention to rosette formation (positive in 18 cases) and glycogen deposition (++ in 21, + in 36, ± in 11, and - in 2 of 70 cases examined). Fourteen tumors (7 with rosettes and 7 without) were selected for immunohistochemical and ultrastructural studies, and 13 showed single or multiple neural markers (neuron-specific enolase in 8, S-100 protein in 6, and neurosecretory-type granules in 9). These possible cases of neural EOE could be divided into three subgroups: tumor with bidirectional neuroblastic and schwannian differentiation (5 cases), tumor with monodirectional neuroblastic differentiation (7 cases), and tumor with monodirectional schwannian differentiation (1 case). EOE with a neural nature may be categorized into a spectrum of peripheral primitive neuroectodermal tumors. Clinical, histopathologic, and biologic differences between this disease and conventional sympathetic neuroblastoma are discussed.

Original languageEnglish (US)
Pages (from-to)442-453
Number of pages12
JournalHuman Pathology
Issue number4
StatePublished - Apr 1988
Externally publishedYes


  • Ewing's sarcoma of bone
  • S-100 protein
  • extraosseous Ewing's sarcoma
  • neuroblastoma
  • neuron-specific enolase

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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