Overview

NCI Definition: A spectrum of malignant tumors arising from the soft tissues, characterized morphologically by the presence of small round cells. Ewing sarcoma and peripheral primitive neuroectodermal tumor represent the ends of a spectrum, with Ewing sarcoma lacking evidence of neural differentiation and the markers that characterize the peripheral primitive neuroectodermal tumor. Ewing sarcoma and peripheral primitive neuroectodermal tumor may share cytogenetic abnormalities, proto-oncogene expression, cell culture and immunohistochemical abnormalities. Pain and the presence of a mass are the most common clinical symptoms. [1]

Disease Details

Synonyms
Extraosseous Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Parent(s)
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Children
Peripheral Primitive Neuroectodermal Tumor of Soft Tissues, Extraskeletal Ewing Sarcoma, and Neuroectodermal Tumor of Soft Tissue

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.