CASE REPORT article
Front. Pediatr.
Sec. General Pediatrics and Pediatric Emergency Care
Pediatric Thoracic Spinal Ewing Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET): A Case Report and Literature Review
Provisionally accepted- 1Siping Central People's Hospital, Siping, China
- 2The University of Hong Kong, Pokfulam, Hong Kong, SAR China
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The Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is highly malignant neoplasms composed of undifferentiated small round cells with the ability to differentiate into various tissue types. We reported the case of an 11-year-old boy who presented with unsteady gait, progressive back pain, bilateral lower limb weakness (more pronounced in the left leg), and urinary retention. Magnetic resonance imaging (MRI) demonstrated a uniformly intense extradural lesion at the T3-5 vertebral level on the left posterolateral side of the spinal canal. The lesion measured 1.35 cm × 4.8 cm on pre-contrast MRI and 4.8 cm × 1.8 cm × 1.2 cm on post-contrast imaging, causing anterior and rightward displacement of the spinal cord with associated intramedullary signal changes and moderate post-contrast enhancement. Preoperative imaging suggested possible diagnoses of lymphoma or lipovascular tumor. However, postoperative histopathological examination confirmed the diagnosis of a small round cell malignant tumor consistent with an ES/PNET. The rapid progression to intramedullary metastasis and poor outcome emphasize the need for early diagnosis and more effective treatment strategies. Spinal ES/PNET is extremely rare, and this case highlights the clinical presentation, diagnostic challenges, and histopathological features of this aggressive tumor, which remain poorly reported in the literature.
Keywords: Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET), thoracic spine, metastasis, prognosis, case report
Received: 31 Mar 2025; Accepted: 27 Nov 2025.
Copyright: © 2025 Ma, Li, Meng, Wong and Wang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Fengfeng Wang
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