scholarly journals Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor and Related Tumors

2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 108-126 ◽  
Author(s):  
Maria Tsokos ◽  
Rita D. Alaggio ◽  
Louis P. Dehner ◽  
Paul S. Dickman
Keyword(s):  
Young Adults ◽  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Cell Tumor ◽  
Adolescents And Young Adults ◽  
Gene Rearrangements ◽  
Round Cell ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements. Other tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.

Melanotic Neuroectodermal Tumor of Infancy: A Molecular Genetic Study

1998 ◽  
Vol 1 (4) ◽  
pp. 295-299 ◽  
Author(s):  
Maliheh Khoddami ◽  
Jeremy Squire ◽  
Maria Zielenska ◽  
Paul Thorner
Keyword(s):  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Molecular Genetic ◽  
Small Cell ◽  
Cell Tumor ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Melanotic neuroectodermal tumor of infancy is a rare but well-recognized entity in pediatric pathology. However, the relationship of this tumor to other pediatric small cell tumors with neuroectodermal features (such as neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, and desmoplastic small round cell tumor) is undetermined. Molecular genetic studies of melanotic neuroectodermal tumor of infancy have not been reported. We studied three typical cases of mela-notic neuroectodermal tumor of infancy in an attempt to link this tumor to other small cell tumors with well-characterized molecular genetic changes. Tests performed included: detection of MYCN gene amplification and deletion of 1p (all 3 cases), and presence of the t(11;22)(q24;q12) and the t(11;22)(p13;q12) translocations (2 of 3 cases). None of these tests yielded positive results. Thus, there is no genetic basis at present to link melanotic neuroectodermal tumor of infancy to neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, or desmoplastic small round cell tumor.

scholarly journals WT1 Staining Reliably Differentiates Desmoplastic Small Round Cell Tumor From Ewing Sarcoma/Primitive Neuroectodermal Tumor

2000 ◽  
Vol 114 (3) ◽  
pp. 345-353 ◽  
Author(s):  
D. Ashley Hill ◽  
John D. Pfeifer ◽  
Edith F. Marley ◽  
Louis P. Dehner ◽  
Peter A. Humphrey ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Cell Tumor ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

DESMOPLASTIC SMALL ROUND CELL TUMOR: A CASE REPORT

2021 ◽  
pp. 7-8
Author(s):  
Bhuwan Kumar ◽  
Rajde Singh ◽  
Anurag Mishra
Keyword(s):  
Young Adults ◽  
Case Report ◽  
Poor Prognosis ◽  
Malignant Neoplasm ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Mesenchymal Tissue

Desmoplastic small round cell tumor is a rare, highly malignant neoplasm originating from mesenchymal tissue which was initially described in 1 1991 by Gerald and Rosai. It is composed of small round tumor cells of uncertain histogenesis associated with prominent stromal desmoplasia and 2 polyphenotypic differentiation. It typically occurs in adolescents and young adults. Usually presents with widespread abdominal, serosal, and mesenteric involvement with poor prognosis.

Desmoplastic Small Round Cell Tumor in the Abdomen and Pelvis: Report of CT Findings in 11 Affected Children and Young Adults

2005 ◽  
Vol 184 (6) ◽  
pp. 1910-1914 ◽  
Author(s):  
Richard Bellah ◽  
Lisa Suzuki-Bordalo ◽  
Eric Brecher ◽  
Jill P. Ginsberg ◽  
John Maris ◽  
...  
Keyword(s):  
Young Adults ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Ct Findings ◽  
Children And Young Adults
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