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Review
. 2012;15(1 Suppl):108-26.
doi: 10.2350/11-08-1078-PB.1.

Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors

Maria Tsokos  1 , Rita D Alaggio, Louis P Dehner, Paul S Dickman
Affiliations
Review

Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors

Maria Tsokos et al. Pediatr Dev Pathol. 2012.

Abstract

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.

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Figures

Figure 1.
Figure 1.
Ewing sarcoma/peripheral primitive neuroectodermal tumor of soft tissue of arm shows a firm white and yellow mass with fine trabeculae.
Figure 2.
Figure 2.
Ewing sarcoma/peripheral primitive neuroectodermal tumor of the chest wall shows a tumor on the inner surface of the rib with abundant hemorrhage and necrosis.
Figure 3.
Figure 3.
Ewing sarcoma/peripheral primitive neuroectodermal tumor of the mesentery shows a white and red focally hemorrhagic necrotic mass.
Figure 4.
Figure 4.
Ewing sarcoma/peripheral primitive neuroectodermal tumor of the kidney shows extensive hemorrhage and necrosis within lobules of a firm white tumor.
Figure 5.
Figure 5.
Ewing sarcoma/peripheral primitive neuroectodermal tumor touch imprint for cytology. Nuclear features are well preserved, hematoxylin and eosin (H&E), ×600.
Figure 6.
Figure 6.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with sheets of uniform cells, H&E, ×200.
Figure 7.
Figure 7.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with uniform cells in sheets and delicate nuclear chromatin, hematoxylin and eosin (H&E), ×600.
Figure 8.
Figure 8.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with light and dark cells, H&E, ×600.
Figure 9.
Figure 9.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with spindle cells, H&E, ×600.
Figure 10.
Figure 10.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with cytoplasmic glycogen, periodic acid–Schiff, ×400.
Figure 11.
Figure 11.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with filigree pattern, H&E, ×200.
Figure 12.
Figure 12.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with neuropil, H&E, ×400.
Figure 13.
Figure 13.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with poorly formed rosettes, hematoxylin and eosin (H&E), ×200.
Figure 14.
Figure 14.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with neuropil and poorly formed rosettes.
Figure 15.
Figure 15.
Ewing sarcoma/peripheral primitive neuroectodermal tumor after chemotherapy with a pattern simulating alveolar rhabdomyosarcoma, H&E, ×200.
Figure 16.
Figure 16.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with abundant cytoplasmic glycogen, electron microscopy.
Figure 17.
Figure 17.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with cluster of membrane-bound granules in cell process, electron microscopy.
Figure 18.
Figure 18.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with uniform CD99 immunostaining in a membranous pattern, CD99, ×400.
Figure 19.
Figure 19.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with diffuse nuclear FLI-1 immunostaining, ×400.
Figure 20.
Figure 20.
Ewing sarcoma/peripheral primitive neuroectodermal tumor with synaptophysin staining in a dot-like cytoplasmic pattern.
Figure 21.
Figure 21.
Ewing sarcoma/peripheral primitive neuroectodermal tumor karyotype with translocation between chromosomes 11 and 22.
Figure 22.
Figure 22.
Ewing sarcoma/peripheral primitive neuroectodermal tumor karyotype with translocation between chromosomes 7 and 22.
Figure 23.
Figure 23.
Ewing sarcoma/peripheral primitive neuroectodermal tumor computed tomographic study of multifocal abdominal DSRCT with multiple liver nodules.
Figure 24.
Figure 24.
Intra-abdominal mass with fibrous bands separating nests of tumor cells. Photograph courtesy of William L. Gerald, M.D.
Figure 25.
Figure 25.
Desmoplastic small round cell tumor with hemorrhagic and necrotic abdominal mass.
Figure 26.
Figure 26.
Desmoplastic small round cell tumor with sheets of round cells with little cytoplasm, hematoxylin and eosin (H&E), ×400.
Figure 27.
Figure 27.
Desmoplastic small round cell tumor with geographic pattern of tumor cells and intervening fibrotic tissue, H&E, ×200.
Figure 28.
Figure 28.
Desmoplastic small round cell tumor with strands of tumor cells of varying width, separated by fibrotic stroma, H&E, ×400.
Figure 29.
Figure 29.
Cytospin preparation of desmoplastic small round cell tumor cells with fine nuclear chromatin and inconspicuous nucleoli, H&E, ×600.
Figure 30.
Figure 30.
Desmoplastic small round cell tumor with diffuse cytokeratin immunostaining, cytokeratin AE1/AE3, ×400.
Figure 31.
Figure 31.
Desmoplastic small round cell tumor with epithelial membrane antigen immunostaining, ×400.
Figure 32.
Figure 32.
Desmoplastic small round cell tumor with desmin immunostaining, ×400.
Figure 33.
Figure 33.
Desmoplastic small round cell tumor with focal positive neuron-specific enolase immunostaining, ×600.
Figure 34.
Figure 34.
Desmoplastic small round cell tumor with diffuse WT1 immunostaining, ×400x.
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