Abstract
Extraosseous Ewing's sarcoma/peripheral neuroectodermal tumors (ES/PNETs) are rare neoplasms that account for approximately 10%-15% of soft tissue sarcomas in children and 5% of soft tissue sarcomas in adults. Primary spinal, extraosseous, intradural ES/PNETs are even less common. The diagnosis of ES/PNET is extremely challenging, because the tumor can have a nonspecific radiologic appearance, and the histologic features are shared by many other “small round cell tumors.” Thus, ES/PNET should be included in the radiologic and pathologic differential diagnosis, even in older patients and in unusual tumor sites. We report two cases of spinal, extraosseous, intradural ES/PNETs in adults who presented with back pain. Magnetic resonance imaging revealed contrast enhancing, intradural lesions in the area of the conus medullaris. The tumor in Case 1 was partially intramedullary, while the tumor in Case 2 was exclusively extramedullary. In both cases, the radiologic and intraoperative surgical impression favored ependymoma. The diagnosis of ES/PNET was established in both cases by histopathologic, immunohistochemical, and molecular analysis.
Highlights
Ewing’s sarcoma/peripheral neuroectodermal tumor (ES/ PNET) is a highly malignant primitive neoplasm, occurring most commonly as a primary tumor of bone in children and young adults.[1]
We present two cases of ES/PNET occurring in the spinal, intradural location
The tumor in Case 2 was positive for the presence of the Ewing sarcoma gene‐FLI1 gene (EWS‐FLI1) fusion transcript associated with ES/PNET
Summary
Ewing’s sarcoma/peripheral neuroectodermal tumor (ES/ PNET) is a highly malignant primitive neoplasm, occurring most commonly as a primary tumor of bone in children and young adults.[1]. Imaging of the entire central nervous system showed no evidence of leptomeningeal enhancement or cerebrospinal fluid (CSF) seeding With these radiologic features and tumor location, ependymoma was considered the most likely diagnosis. Due to extensive entanglement of nerve roots in the tumor, the surgical goal was to debulk as much of the mass as possible while avoiding damage to peripheral nerve function Histologic examination of both tumors showed densely packed sheets and nests of small cells with scant cytoplasm and round to ovoid nuclei [Figure 3]. The tumor in Case 2 was positive for the presence of the Ewing sarcoma gene‐FLI1 gene (EWS‐FLI1) fusion transcript associated with ES/PNET
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