Primary extraskeletal intradural Ewing sarcoma with acute hemorrhage: a case report and review of the literature

Abstract

Background: Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor typically originating from skeletal bone, cases of primary intradural extraskeletal Ewing sarcoma are exceptionally rare. The similarity of its presentation to other spinal tumors further complicates its identification and management.

Case presentation: We report a case of a 58-year-old Palestinian male with intradural extraskeletal lumbar Ewing sarcoma. The patient initially presented with lower back pain and bilateral S1 radiculopathy, with more severe symptoms on the left side. Magnetic resonance imaging revealed a 7 cm oval-shaped mass with homogeneous contrast enhancement, obstructing the spinal canal from L3/L4 to L5/S1 levels. Initially, a myxopapillary ependymoma was suspected, but the patient's sensory and motor functions suddenly deteriorated during hospitalization. Repeat magnetic resonance imaging indicated heterogeneous contrast enhancement, indicating acute intratumoral hemorrhage. Consequently, the patient underwent emergent L3-L5 laminotomy, with successful gross total resection of the tumor. Histopathological and immunohistochemical analyses confirmed the diagnosis of intradural extraskeletal Ewing sarcoma. Adjuvant therapy was administered to minimize the risk of local recurrence or distant metastasis. A systematic review of relevant literature, along with retrospective analysis of medical records, operative reports, radiological studies, and histopathological findings of similar cases, was also conducted.

Conclusions: Intradural extraskeletal Ewing sarcoma is an infrequently encountered condition in adult patients, emphasizing the importance of considering it in the differential diagnosis of spinal tumors. Surgeons must possess a comprehensive understanding of this rare entity to ensure accurate staging and optimal management, particularly in the early stages when prompt intervention may improve prognosis.

Keywords: Chemotherapy; Ewing sarcoma; Extra-skeletal; Intradural; Intratumoral hemorrhage; Radiotherapy; Tumor.

Fig. 1

Sagittal lumber MRI images at the initial presentation and after the deterioration of the patient’s status. At the initial presentation, T1 and T2 noncontrasted sequence showing intermediate T1, low T2 signal-intensity intradural/ extramedullary lesion extending from the lower aspect of L3 to L5–S1 disc level, while sagittal T1 contrasted image showing diffuse homogenous enhancement of the lesion. After the deterioration of patient’s symptoms, sagittal T1- and T2-contrasted images showing change in intensity in T2 and change in enhancement with contrast to a heterogenous pattern, suggestive of intratumoral hemorrhage. L: lesion; T1WI: T1 MRI image without contrast; T2WI: T2 MRI image without contrast; T1 C: T1 MRI image with contrast; T2 C: T2 MRI image with contrast (red circle indicates the letter L)

Fig. 2

Photomicrograph of resected samples. A High-power image of hematoxylin and eosin stain showing uniform small round cells with fine stippled chromatin, inconspicuous nucleoli, scant clear to eosinophilic cytoplasm, indistinct cytoplasmic membranes, and focal necrosis. Immunohistochemical findings revealed positive staining for B CD99 and C NKX2.2

Fig. 3

A, B and C, sagittal T1, T2, and T1 contrasted postoperative MRI imaging of the lumber spine respectively, showing gross total resection of the lesion with postoperative changes