Primary Extraskeletal Ewing's Sarcoma Research Articles

Primary Extraskeletal Ewing Sarcoma, Initially Misdiagnosed as a Neurogenic Tumor

Primary Extraskeletal Ewing Sarcoma, Initially Misdiagnosed as a Neurogenic Tumor

Primary Extraskeletal Ewing Sarcoma of the Duodenum Revealed by 18 F-FDG PET/CT.

Extraskeletal Ewing sarcoma of the duodenum is a relatively uncommon entity tumor. We report a case of extraskeletal Ewing sarcoma in a 21-year-old woman. She complained of abdomen pain and melena. 18 F-FDG PET/CT showed an intense uptake by the duodenum mass along with multiple FDG-avid enlarged lymph nodes in the mesentery, which was pathologically proven as extraskeletal Ewing sarcoma.

18F-FDG PET/CT of Primary Extraskeletal Ewing Sarcoma of the Prostate.

Prostate Ewing sarcoma is rare. We report the 18F-FDG PET/CT finding of a 16-year-old adolescent boy who presented with dysuria and was confirmed to be extraskeletal Ewing sarcoma of the prostate by histopathologic findings.

Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases.

BackgroundThe clinicopathological characteristics, outcomes and prognostic factors of primary extraskeletal Ewing sarcoma (EES) remained insufficiently explored. We aimed to examine these aspects and compared the same with skeletal Ewing sarcoma (SES).Patients and methodsWe identified Ewing sarcoma, peripheral primitive neuroectodermal tumors or Askin tumor patients who were registered in the Surveillance, Epidemiology, and End Results database from 1973 to 2014. Clinicopathological features were assessed by using Fisher’s exact tests. Cancer-specific survival (CSS) and overall survival (OS) were estimated by using the Kaplan–Meier method and the Cox proportional hazards model. Prognostic factors were identified by multivariate Cox regression analysis.ResultsThe age of patients with EES was diagnosed to be higher and they were more likely to be female (46.1% vs 36.2%; P<0.001), have tumor <10 cm (49.8% vs 35.4%; P<0.001), have regional node involvement (5.4% vs 1.0%; P<0.001) and receive surgery (69.1% vs 53.8%; P<0.001) compared to patients with skeletal tumors. Metastatic status did not differ by origin. Kaplan–Meier analysis showed that the origin had significant difference in CSS and OS among patients aged 0–19 years and with metastatic stage at presentation, but not in patients aged 20–39, ≥40 years and with no-metastatic stage. A Cox multivariable model controlling for differences between groups confirmed inferior survival for patients with EES. Age, tumor size, tumor stage and surgery were the most important factors significantly influencing both CSS and OS in the EES and SES patients. Race, year of diagnosis and tumor site were associated with CSS and OS among patients with SES, but failed in EES.ConclusionThe clinicopathological characteristics, outcomes and prognostic factors differed among patients with EES compared to patients with SES. Extraskeletal origin was an unfavorable prognostic factor.

Primary Extraskeletal Ewing's Sarcoma of Nasal Cavity

Extra skeletal Ewing's Sarcoma (EES) of nasal cavity is a rare soft tissue tumor, morphologically similar to the commoner Ewing's sarcoma (ES) arising from bone. We report a case of EES of nasal cavity in a nine year old male who presented with nasal obstruction. Contrast enhanced CT scan showed mildly enhancing soft tissue attenuating mass. Histopathologically examination showed round cell tumor which was confirmed by special stain and immunohistochemistry. PAS showed positivity for intracellular glycogen, Immunohistochemistry showed positivity for MIC-2,CK,S-100,EMA.

Primary Ewing's sarcoma of vulva: A case report and a review of the literature

Ewing sarcomas/peripheral primitive neuroectodermal tumors (ES/pPNET) are extremely rare in the vulva. A review of the literature reveals only 14 previously reported possible cases. Here we reported a case of primary extraskeletal Ewing's sarcoma (EES) of the vulva in a 37-year-old woman. Characteristic histologic features of ES/pPNET were present in this case, including a monomorphic population of small round blue cells with cytoplasmic glycogen confirmed by periodic acid-Schiff, membrane staining with CD99 and nuclear staining with FLI-1. After surgery, the patient was found to have pulmonary metastasis and then received six cycles of polychemotherapy. She is still alive with stable disease after 1 year of follow up. Our findings underline the crucial role of immunohistochemical techniques in the differential diagnosis of small round cell tumors in these unusual locations. We also give a summary about the clinical and pathological features of the primary ES/pPNET in the vulva reported previously in the literature.

Spinal epidural metastasis from Ewing's sarcoma on PET/CT: A case report

Early diagnosis of metastatic spinal disease is important because functional outcome depends on neurologic condition at the time of diagnosis. Epidural metastasis to the spine from Ewing's sarcoma is very uncommon. Despite the fact that epidural involvement has been described in a few cases of primary extraskeletal Ewing's sarcoma, it is considerably rare. Metastatic epidural involvement secondary to Ewing's sarcoma is even rarer. With the rapid increase in the utilization and acceptance of PET/CT imaging in oncological practice, it is playing an ever-increasing role in detecting malignant spread to several unexpected sites. This report is, to the best of our knowledge, the first description of the PET/CT findings of this extremely rare entity.

Spinal epidural metastasis from Ewing's sarcoma on PET/CT: A case report

Early diagnosis of metastatic spinal disease is important because functional outcome depends on neurologic condition at the time of diagnosis. Epidural metastasis to the spine from Ewing's sarcoma is very uncommon. Despite the fact that epidural involvement has been described in a few cases of primary extraskeletal Ewing's sarcoma, it is considerably rare. Metastatic epidural involvement secondary to Ewing's sarcoma is even rarer. With the rapid increase in the utilization and acceptance of PET/CT imaging in oncological practice, it is playing an ever-increasing role in detecting malignant spread to several unexpected sites. This report is, to the best of our knowledge, the first description of the PET/CT findings of this extremely rare entity.

Extraskeletal Ewing's sarcoma of the thoracic epidural space: Case report and review of the literature

The occurrence of primary extraskeletal Ewing's sarcoma (EES) of the spinal epidural space has been rarely reported in the literature. The clinical, radiologic and pathologic features of a case of EES occurring in the thoracic epidural space are presented. A 37-year-old woman presented with a one-year history of back pain. Magnetic resonance imaging demonstrated an epidural mass at the T8-9 level. Laminectomy and partial resection of the tumor were performed. The differential diagnosis of a spinal epidural mass is broad. Histopathological and molecular cytogenetic examinations confirmed an EES arising from the thoracic epidural space. Despite receiving both chemotherapy and radiotherapy, the patient died of respiratory insufficiency due to medulla oblongata metastasis 22 months after the initial consultation. Awareness of this entity will allow this rare diagnosis to be considered and facilitate appropriate management. A review of the literature on spinal epidural EES is also presented.

Primary extraskeletal Ewing's sarcoma of the lung

Ewing's sarcoma is a neuroectodermal tumour characteristically presenting during the second decade of life and arising from bone. Extraskeletal Ewing's sarcoma is exceptionally rare. We present a case of a 44-year-old man who underwent pneumonectomy for a primary extraskeletal Ewing's sarcoma of the lung. This demonstrates a very rare pulmonary Ewing's sarcoma and highlights the importance of preoperative evaluation in aggressive tumours.

Primary Extraskeletal Ewing Sarcoma Originating from Chest Wall in a Child

We report the ultrasonography (US) and computed tomography (CT) findings of a rare case of extraskeletal Ewing sarcoma involving the left chest wall with a diameter of 4cm in a 5 years old girl. The lesion was heterogenously hypoechoic on sonography with a lobulated smooth contour. On CT, the lesion had central necrotic areas and was enhancing heterogeneously. With these US and CT findings surgical excision of the tumor was performed. Histopathologically the mass was evaluated as round cell malignant neoplasm (Ewing tumor/ “Askin tumor”/ primitive neuroectodermal tumor (PNET)). Chemotherapy and radiotherapy were planned to the patient.

Primary Extraosseous Ewing Sarcoma of the Kidney With Level III Inferior Vena Cava Thrombus

Ewing sarcoma typically presents as a skeletal-based tumor, with rare instances of peripheral primitive neuroectodermal tumor (PNET) arising in the soft tissues. Few examples of organ-based PNETs have been previously described in the literature. These tumors are exceedingly rare as a primary renal neoplasm. To our knowledge, this is the first report documenting a primary extraskeletal Ewing sarcoma of the kidney with thrombus extending into the intrahepatic inferior vena cava thrombus (level III).

Multimodal Treatment of Primary Extraskeletal Ewing's Sarcoma of the Chest Wall: Report of 2 Cases

Extraskeletal Ewing's sarcoma (EES) is a type of Ewing's sarcoma that arises in soft tissue and is now regarded as a member of a family of small round cell neoplasms of bone and soft tissue, including primitive neuroectodermal tumors (PNETs). EES occurs predominantly in adolescents and young adults between the ages of 10 and 30 years. The disease follows an aggressive course with a high recurrence rate. The presence of a distant metastasis is also common. EES arises in the soft tissue of either the trunk or extremities. We recently experienced two cases of EES that occurred in the chest wall. The two patients underwent wide resection and combined radiochemotherapy. There was no evidence of disease 30 and 22 months, respectively, after surgery. Although extremely rare, EES should be considered in the differential diagnosis of chest wall tumors. We report two cases of EES with a brief review of the literature.

Extraskeletal Ewing's Sarcoma of the breast

Extraskeletal Ewing's Sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from the more common Ewing's sarcoma of the bone. Isolated cases of Extraskeletal Ewing's Sarcoma of various body parts have been reported. Here we report a case of Primary Extraskeletal Ewing's Sarcoma of the breast, that to best of our knowledge, has not been reported before.