Work

Primary dural-based intracranial ewing sarcoma in a child: diagnostic challenges, management, and review of literature

Year: 2025

Type: article

Abstract: Background Ewing’s sarcoma primarily involving the skull is an uncommon occurrence, representing approximately 1% of all reported cases. Only a limited number of such cases have been described in the literature. The principal diagnostic challenge lies in distinguishing these tumors from other small round blue cell neoplasms of the central nervous system (CNS), such as primitive neuroectodermal tumors (PNETs). Ewing’s sarcoma typically arises in bone and is not related to intraparenchymal embryonal CNS tumors. When the CNS is affected, the disease usually extends locally from the skull or epidural space, or less commonly, through intracranial or spinal metastases. Primary intracranial lesions are exceedingly rare and have been reported predominantly as dural-based tumors. Case presentation We report the case of an 8-year-old girl diagnosed with primary intracranial Ewing’s sarcoma involving the skull. The patient presented with progressive neurological symptoms and was managed at our institution. Radiological and histopathological assessments, supported by immunohistochemical analysis and molecular confirmation of the characteristic EWSR1 gene rearrangement, established the diagnosis. The patient received multimodality therapy within a multidisciplinary treatment framework. Conclusion Primary intracranial Ewing’s sarcoma is an exceptionally rare entity that may closely resemble other dural-based pediatric tumors. Accurate diagnosis necessitates a combination of histopathological, immunohistochemical, and molecular investigations. Early recognition and the implementation of a multimodal therapeutic strategy are essential for achieving optimal clinical outcomes.

Source: The Egyptian Journal of Neurosurgery : the official publication of the Egyptian Society of Neurological Surgeons/Egyptian journal of neurosurgery

DOI: 10.1186/s41984-025-00513-7

Authors: Hemant Muktaram Jawale, Aadil S. Chagla, Akshay Pol, Srikant Balasubramaniam, Nakul Rathore, Nidhi Roy

Institution: Topiwala National Medical College & BYL Nair Charitable Hospital

Language: English

Topic: Sarcoma Diagnosis and Treatment

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: diamond

OpenAlex ID: W7117161104