Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass

Ewing sarcoma is a rare, highly aggressive small round cell tumor that primarily affects the bones of adolescents and young adults, with extra skeletal involvement being particularly uncommon. We present the case of an 18-year-old male with a progressively enlarging, painful swelling in the right po...

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Main Authors: Shubhlaxmi Srivastava, MBBS, Gaurav Raj, MD, Kaustubh Gupta, MD, Ajeet Kumar Gandhi, MD, Nilesh Kumar Sinha, MBBS
Format: Article
Language:English
Published: Elsevier 2025-10-01
Series:Radiology Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S1930043325006430
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author Shubhlaxmi Srivastava, MBBS
Gaurav Raj, MD
Kaustubh Gupta, MD
Ajeet Kumar Gandhi, MD
Nilesh Kumar Sinha, MBBS
author_facet Shubhlaxmi Srivastava, MBBS
Gaurav Raj, MD
Kaustubh Gupta, MD
Ajeet Kumar Gandhi, MD
Nilesh Kumar Sinha, MBBS
author_sort Shubhlaxmi Srivastava, MBBS
collection DOAJ
description Ewing sarcoma is a rare, highly aggressive small round cell tumor that primarily affects the bones of adolescents and young adults, with extra skeletal involvement being particularly uncommon. We present the case of an 18-year-old male with a progressively enlarging, painful swelling in the right posterior neck, ultimately diagnosed as Extra skeletal Ewing sarcoma involving the occipital bone and C1 vertebra. Imaging revealed extensive intracranial, intraspinal, and paravertebral extension with multiple skeletal metastases. Histopathological and immunohistochemical analysis confirmed the diagnosis, showing NKX 2.2 positivity. The patient was initiated on systemic chemotherapy using the VDC/IE regimen, leading to significant clinical and radiological improvement. This case underscores the importance of considering Ewing sarcoma in atypical paraspinal and cranial presentations and highlights the critical role of early multimodal imaging and chemotherapy in managing aggressive disease.
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series Radiology Case Reports
spelling doaj-art-527cb58015514a798ff9bc16f61ebcc02025-08-20T03:58:00ZengElsevierRadiology Case Reports1930-04332025-10-0120105296529910.1016/j.radcr.2025.06.101Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck massShubhlaxmi Srivastava, MBBS0Gaurav Raj, MD1Kaustubh Gupta, MD2Ajeet Kumar Gandhi, MD3Nilesh Kumar Sinha, MBBS4Corresponding author.; Department of Radio-Diagnosis, Dr Ram Manohar Lohia Institute Of Medical Sciences, Lucknow, Uttar Pradesh, IndiaDepartment of Radio-Diagnosis, Dr Ram Manohar Lohia Institute Of Medical Sciences, Lucknow, Uttar Pradesh, IndiaDepartment of Radio-Diagnosis, Dr Ram Manohar Lohia Institute Of Medical Sciences, Lucknow, Uttar Pradesh, IndiaDepartment of Radio-Diagnosis, Dr Ram Manohar Lohia Institute Of Medical Sciences, Lucknow, Uttar Pradesh, IndiaDepartment of Radio-Diagnosis, Dr Ram Manohar Lohia Institute Of Medical Sciences, Lucknow, Uttar Pradesh, IndiaEwing sarcoma is a rare, highly aggressive small round cell tumor that primarily affects the bones of adolescents and young adults, with extra skeletal involvement being particularly uncommon. We present the case of an 18-year-old male with a progressively enlarging, painful swelling in the right posterior neck, ultimately diagnosed as Extra skeletal Ewing sarcoma involving the occipital bone and C1 vertebra. Imaging revealed extensive intracranial, intraspinal, and paravertebral extension with multiple skeletal metastases. Histopathological and immunohistochemical analysis confirmed the diagnosis, showing NKX 2.2 positivity. The patient was initiated on systemic chemotherapy using the VDC/IE regimen, leading to significant clinical and radiological improvement. This case underscores the importance of considering Ewing sarcoma in atypical paraspinal and cranial presentations and highlights the critical role of early multimodal imaging and chemotherapy in managing aggressive disease.http://www.sciencedirect.com/science/article/pii/S1930043325006430ChemotherapyEwing sarcomaNKX 2.2Small round cell tumorSkeletal metastasisVDC/IE regimen
spellingShingle Shubhlaxmi Srivastava, MBBS
Gaurav Raj, MD
Kaustubh Gupta, MD
Ajeet Kumar Gandhi, MD
Nilesh Kumar Sinha, MBBS
Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass
Radiology Case Reports
Chemotherapy
Ewing sarcoma
NKX 2.2
Small round cell tumor
Skeletal metastasis
VDC/IE regimen
title Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass
title_full Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass
title_fullStr Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass
title_full_unstemmed Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass
title_short Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass
title_sort rare case of infiltrative multifocal ewing sarcoma presenting as neck mass
topic Chemotherapy
Ewing sarcoma
NKX 2.2
Small round cell tumor
Skeletal metastasis
VDC/IE regimen
url http://www.sciencedirect.com/science/article/pii/S1930043325006430
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