At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation
Abstract Background Ewing sarcoma (ES) is the second most frequent sarcoma of bone, often affecting young patients and pursuing an aggressive clinical course. Among therapeutic choices, radio- and chemotherapy are employed in neoadjuvant setting, and they yield variable histological changes in neopl...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2025-04-01
|
| Series: | Diagnostic Pathology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13000-025-01649-8 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850139442425102336 |
|---|---|
| author | Giorgia Arcovito Giacomo Aringhieri Virna Zampa Luca Coccoli Lorenzo Andreani Alessandro Franchi |
| author_facet | Giorgia Arcovito Giacomo Aringhieri Virna Zampa Luca Coccoli Lorenzo Andreani Alessandro Franchi |
| author_sort | Giorgia Arcovito |
| collection | DOAJ |
| description | Abstract Background Ewing sarcoma (ES) is the second most frequent sarcoma of bone, often affecting young patients and pursuing an aggressive clinical course. Among therapeutic choices, radio- and chemotherapy are employed in neoadjuvant setting, and they yield variable histological changes in neoplastic tissue, which mainly include necrosis and fibrosis. Cytodifferentiation is seldom observed in pediatric tumors such as nephroblastoma and rhabdomyosarcoma following treatment. Nevertheless, it represents an extremely rare phenomenon in ES. Case presentation In this study we present the clinico-pathologic and molecular features of two cases of ES undergoing neuroblastoma-like differentiation after treatment. Both tumors were primarily located in bone and presented the histologic and immunohistochemical features of classic ES in needle biopsies. They were treated with standard chemotherapy protocols followed by surgical resection. The resection specimens of the primary tumor of patient 1 and pleural metastases of patient 2 presented foci of eosinophilic fibrillary stroma resembling neuropil and containing cellular elements with wide granular eosinophilic cytoplasm, eccentric nuclei containing vesicular chromatin and prominent nucleoli, reminiscent of ganglion cells. These cells were positive for chromogranin, synaptophysin and CD56, while CD99 was negative. Molecular confirmation of EWSR1 rearrangement was provided in both cases by next generation sequencing and FISH analysis. Conclusions Evidence of neural differentiation in ES unravels interesting hints about its controversial histogenesis. Furthermore, awareness of this event must be increased to avoid misdiagnosis with neuroblastoma, which shows significant morphological overlap. |
| format | Article |
| id | doaj-art-4d99e954f50b4610bb477cf842f0bda8 |
| institution | OA Journals |
| issn | 1746-1596 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj-art-4d99e954f50b4610bb477cf842f0bda82025-08-20T02:30:18ZengBMCDiagnostic Pathology1746-15962025-04-012011710.1186/s13000-025-01649-8At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiationGiorgia Arcovito0Giacomo Aringhieri1Virna Zampa2Luca Coccoli3Lorenzo Andreani4Alessandro Franchi5Section of Anatomic Pathology, Department of Translational Research and of New Technologies in Medicine and Surgery, University of PisaSection of Radiology, Department of Translational Research and of New Technologies in Medicine and Surgery, University of PisaDiagnostic and Interventional Radiology, University Hospital of PisaDepartment of Orthopedics and Trauma Surgery, University Hospital of PisaPediatric Hematology Oncology Unit, University Hospital of PisaSection of Anatomic Pathology, Department of Translational Research and of New Technologies in Medicine and Surgery, University of PisaAbstract Background Ewing sarcoma (ES) is the second most frequent sarcoma of bone, often affecting young patients and pursuing an aggressive clinical course. Among therapeutic choices, radio- and chemotherapy are employed in neoadjuvant setting, and they yield variable histological changes in neoplastic tissue, which mainly include necrosis and fibrosis. Cytodifferentiation is seldom observed in pediatric tumors such as nephroblastoma and rhabdomyosarcoma following treatment. Nevertheless, it represents an extremely rare phenomenon in ES. Case presentation In this study we present the clinico-pathologic and molecular features of two cases of ES undergoing neuroblastoma-like differentiation after treatment. Both tumors were primarily located in bone and presented the histologic and immunohistochemical features of classic ES in needle biopsies. They were treated with standard chemotherapy protocols followed by surgical resection. The resection specimens of the primary tumor of patient 1 and pleural metastases of patient 2 presented foci of eosinophilic fibrillary stroma resembling neuropil and containing cellular elements with wide granular eosinophilic cytoplasm, eccentric nuclei containing vesicular chromatin and prominent nucleoli, reminiscent of ganglion cells. These cells were positive for chromogranin, synaptophysin and CD56, while CD99 was negative. Molecular confirmation of EWSR1 rearrangement was provided in both cases by next generation sequencing and FISH analysis. Conclusions Evidence of neural differentiation in ES unravels interesting hints about its controversial histogenesis. Furthermore, awareness of this event must be increased to avoid misdiagnosis with neuroblastoma, which shows significant morphological overlap.https://doi.org/10.1186/s13000-025-01649-8Ewing sarcomaPost-treatment effectsCytodifferentiationNeuroblastoma |
| spellingShingle | Giorgia Arcovito Giacomo Aringhieri Virna Zampa Luca Coccoli Lorenzo Andreani Alessandro Franchi At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation Diagnostic Pathology Ewing sarcoma Post-treatment effects Cytodifferentiation Neuroblastoma |
| title | At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation |
| title_full | At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation |
| title_fullStr | At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation |
| title_full_unstemmed | At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation |
| title_short | At the crossroad between Ewing sarcoma and neuroblastoma: a report of two cases of Ewing sarcoma with post treatment neuroblastoma-like differentiation |
| title_sort | at the crossroad between ewing sarcoma and neuroblastoma a report of two cases of ewing sarcoma with post treatment neuroblastoma like differentiation |
| topic | Ewing sarcoma Post-treatment effects Cytodifferentiation Neuroblastoma |
| url | https://doi.org/10.1186/s13000-025-01649-8 |
| work_keys_str_mv | AT giorgiaarcovito atthecrossroadbetweenewingsarcomaandneuroblastomaareportoftwocasesofewingsarcomawithposttreatmentneuroblastomalikedifferentiation AT giacomoaringhieri atthecrossroadbetweenewingsarcomaandneuroblastomaareportoftwocasesofewingsarcomawithposttreatmentneuroblastomalikedifferentiation AT virnazampa atthecrossroadbetweenewingsarcomaandneuroblastomaareportoftwocasesofewingsarcomawithposttreatmentneuroblastomalikedifferentiation AT lucacoccoli atthecrossroadbetweenewingsarcomaandneuroblastomaareportoftwocasesofewingsarcomawithposttreatmentneuroblastomalikedifferentiation AT lorenzoandreani atthecrossroadbetweenewingsarcomaandneuroblastomaareportoftwocasesofewingsarcomawithposttreatmentneuroblastomalikedifferentiation AT alessandrofranchi atthecrossroadbetweenewingsarcomaandneuroblastomaareportoftwocasesofewingsarcomawithposttreatmentneuroblastomalikedifferentiation |