Extraskeletal Ewing's Sarcoma Research Articles

CT and Positron Emission Tomography/CT Findings of Mediastinal Extraskeletal Ewing's Sarcoma with Extensive Distant Metastasis: A Case Report

Extraskeletal Ewing’s sarcoma (EES) is a rare malignant small round cell tumor of neural crest origin, which is histologically similar to the more common osseous Ewing sarcoma. Different from the osseous counterpart, it shows a wider age presentation, preferentially occurring in children and young adults, younger than 30 years (1, 2). The most frequent sites of occurrence are the chest wall, lower extremities and paravertebral region, but it rarely occurs in the mediastinum. The recently reported CT finding of mediastinal EES is a large, non-calcified mass with heterogeneous enhancement, frequently infiltrating to the adjacent tissues. In spite of its aggressive behavior, distant metastasis is rare (1, 3). Although positron emission tomography (PET)/CT findings of EES have been rarely reported, it has been reported to show relatively weak fluorodeoxyglucose (FDG) uptake, considering the growth pattern (4). We report a case of a 68-year-old man diagnosed as mediastinal EES with extensive hematogenous and lymph node metastasis by a CT and PET/CT.

Primary spinal extradural Ewing′s sarcoma (primitive neuroectodermal tumor): Report of a case and meta-analysis of the reported cases in the literature

Background:Primary spinal primitive neuroectodermal tumors (PNET) and/or spinal extraskeletal Ewing's sarcoma family tumors (ESET) are rare lesions appearing in the spinal extradural space. One hundred forty-one primary spinal PNETs, including 29 intramedullary lesions, have been reported in the literature. Encountering a case of primary epidural EES/peripheral PNET (pPNET) in sacral level, which is the fifth one occurring at this level in the literature, we have tried to conduct a meta-analysis of the reported cases.Case Description:A 44-year-old lady with epidural EES/pPNET is reported here. She was once operated for L5/S1 herniated disc, which did not ameliorate her symptoms. The clinical, imaging, surgical, and histopathologic characteristics of our case are presented and wide search of the literature is also done. All the reports were level 3 or less evidences and most of the series had missing parts. 106 cases of primary intraspinal (extradural/extramedullary-intradural) EES/pPNET and 29 cases of primary intramedullary PNET (CNS-PNET) have been reported in the literature. The most common clinical presentation in both entities was muscle weakness proportionate to the tumor location. Distant metastasis occurred in 38 of 99 (38%) cases of primary intraspinal EES/pPNET, while the rate of metastasis was 48% in patients with PNETs occurring in the intramedullary region (P > 0.05). One-year survival rate of the patients who underwent chemo-radiation after total or subtotal resection was better than those who did not receive chemotherapy or radiotherapy, or did not have total or subtotal resection. However, this difference was not repeated in 2-year survival rate in any of the tumor groups.Conclusion:It seems that total or subtotal removal of the tumor and adjuvant chemo- and radiation therapy can improve the outcome in these patients.

Requesting clarifications concerning the case report on ′extraskeletal Ewing sarcoma of the sinonasal tract

Requesting clarifications concerning the case report on ′extraskeletal Ewing sarcoma of the sinonasal tract

Rare case of extraskeletal Ewings sarcoma of the sinonasal tract

Ewings sarcoma (ES) and primitive neuroectodermal tumor are closely related family of small round cell tumors seen in childhood and adolescence. The incidence of these tumors occurring in the head and neck region is just 2-7%. Mandible and maxilla are the most common sites, whereas involvement of the sinonasal tract is very rare. We report a case of extraskeletal ES of the sinonasal tract in a 29-year-old female who presented with nasal obstruction and epistaxis. The patient was treated with 14 cycles of chemotherapy, combined with surgery and radiotherapy with complete recovery. We present this case due to its rarity, to analyze the clinical, histopathological and immunohistochemical findings, so as to differentiate from other small round cell tumors of the sinonasal tract for appropriate treatment.

Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement

A minority of patients with Ewing sarcoma present with regional lymph node involvement. We investigated if patient characteristics and outcomes differ between patients with Ewing sarcoma with and without regional node involvement. Patients <40 years of age with Ewing sarcoma or peripheral primitive neuroectodermal tumor (PNET) reported to the SEER database from 1973 to 2008 were evaluated based on the presence (n = 91) or absence (n = 1,361) of regional node involvement. Patient characteristics were analyzed using Fisher exact tests. Overall survival was estimated by Kaplan-Meier methods and evaluated using log-rank tests and Cox models. Patients with regional node involvement were more likely to have extraskeletal primary tumors (65.9% vs. 31.2%; P < 0.001) and axial tumors (71.1% vs. 59.6%; P = 0.03) compared to patients without regional node involvement. The incidence of regional node involvement was 12.4% for patients with extraskeletal primary tumors compared to 3.2% for patients with skeletal tumors. Five-year overall survival from diagnosis was inferior for patients with regional node involvement compared to those without regional node involvement (45.9% vs. 60.3%; P < 0.001). On multivariate analysis, regional node involvement was predictive of inferior overall survival independent of age, metastatic status, tumor site, and soft tissue origin (hazard ratio 1.59; 95% CI 1.16-2.19). Patients with extraskeletal Ewing sarcoma should undergo evaluation for regional node involvement. If validated, our findings indicate that regional node involvement may be an independent adverse prognostic factor in Ewing sarcoma, and potentially useful in risk-stratifying patients with otherwise localized disease.

Primary Ewing’s Sarcoma of Penis – A Rare Case Report

Ewing’s sarcoma (ES) and primitive neuroectodermal tumor (PNET) were considered as two ends of a spectrum of same tumor entity based on their similar clinical, immuno-histochemical and cytogenetic features. Extraskeletal Ewing’s sarcoma (EES) is a type of Ewing’s sarcoma that arises in soft tissue and is regarded as a member of the Ewing sarcoma/primitive neuroectodermal tumor (PNET) family. Primary visceral ES/PNETs are very rarely seen aggressive tumors. We present this case as this is forth case of ES/PNETs with penile localization [1–3]. One case of EES metastasizing to penis was also reported [4].

A to Z of Extraskeletal Ewing Sarcoma Family of Tumors in Adults: Imaging Features of Primary Disease, Metastatic Patterns, and Treatment Responses

Though classically thought of as an osseous-based sarcoma of children and adolescents, it is important to recognize that approximately 20% of all cases of Ewing sarcoma family of tumors (ESFT) occur in patients older than 18 years, often with extraskeletal disease. Understanding the specific imaging features, manners of presentation, and patterns of behavior of ESFT is therefore important in all settings. This article presents the imaging findings of ESFT in the adult, with specific attention to extraskeletal manifestations.

Rapidly growing intrathoracic extraskeletal Ewing's sarcoma

There are few reported cases of intrathoracic Ewing's sarcoma, a very rare malignant neoplasm. We report a surgical case of extraskeletal Ewing's sarcoma that had been followed-up as a stable sized tumour for many years, which then grew rapidly within a year. A 27-year old female patient with a rapidly growing abnormal shadow on chest roentgenogram was admitted to our department. She had undergone periodic examinations including chest computed tomography (CT) scans for 6 years since a small nodule in her chest had been pointed out by chest roentgenogram. The initial CT demonstrated a solitary nodule with a diameter of 20 mm on the parietal pleura that covered the V rib of the posterior chest wall. For 5 years the tumour's size did not change noticeably but it suddenly grew to about 90 mm diameter in a year. The tumour volume doubling time was calculated to be 17 days.

Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature

Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are closely related malignant neoplasms, usually affecting the skeletal system. Extraosseous ES/PNETs are uncommon, with occasional reports of tumors affecting the genitourinary tract. Only few cases of primary vulvar Ewing's sarcoma/PNET have previously been reported. We present a patient with primary vulvar Ewing's sarcoma with pulmonary metastasis who presented at the Department of Gynecology and Obstetrics, University of Schleswig-Holstein, Campus Luebeck. The present report is the third case of a primary vulvar Ewing's sarcoma and the first constellation with pulmonary metastasis at diagnosis in the current literature. We present the diagnostic and therapeutic management including surgery, chemotherapy and radiation. The treatment of the Ewing's sarcomas and PNETs requires a multidisciplinary systemic approach. Despite its rarity, the differential diagnosis of vulvar sarcoma must be considered in young women.

Primary buccal extraskeletal Ewing’s sarcoma as a rare tumor of head and neck region

Extraskeletal Ewing's sarcoma is a mesenchymal malign soft tissue tumor which generally affects the lower extremities and paravertebral region and is very rarely seen in the head and neck area. Early diagnosis and chemotherapy and radiotherapy program combines with surgical excision would be the appropriate treatment modality. In this article we present a case of extraskeletal Ewing's sarcoma which arise from the buccal area as an unusual localization together with information from the literature. In the physical examination of a 23-year old male patient who admitted to our clinic with a complaint of painless mass on his cheek which had been increasingly growing for approximately three months, a painless semi-mobile mass of approximately 5 x 3 cm in size with regular borders and elastic consistency was found within buccal soft tissue in the left maxillary area. The mass was excised under general anesthesia. No local recurrences or findings of distant metastases were observed during the 11-month postoperative follow-up.

Massive venous thrombosis of inferior vena cava as primary manifestation of renal Ewing’s sarcoma

We report an extraordinarily rare case of a 17-year-old male with an extraskeletal Ewing's sarcoma (ESS) of the kidney and a massive thrombosis involving the inferior vena cava (IVC), from the iliac axis to the right atrium. This onset resembled renal cell carcinoma (RCC), although histological examination revealed it was an extraskeletal Ewing's sarcoma/peripheral neuro-ectodermal tumor (EES/PNET). EES/PNET should benefit from neoadjuvant chemotherapy to reduce the risk of metastasis and of recurrent disease due to delay in suitable treatment. Therefore, in the presence of a renal mass with tumor extension of IVC, it is reasonable to bear in mind that other tumors, apart from RCC, could occur. In such cases, a US or CT-scan guided biopsy could be useful.

Ewing's sarcoma of the masseter muscle

To present a case of extraskeletal Ewing's sarcoma originating in the masseter muscle, and to review the diagnosis and management of these rare tumours. Case report and literature review. We report a patient with Ewing's sarcoma of the masseter muscle with mandibular invasion. She was treated with induction chemotherapy, salvage surgery and free flap reconstruction. This case highlights the management of these rare tumours. Aggressive multi-modal treatment, including multi-agent chemotherapy, surgical resection and irradiation, appears to offer the best prognosis. Contemporary reconstructive techniques can restore function and form in cases with challenging defects.

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.

Clinical features and outcomes in patients with extraskeletal ewing sarcoma

Ewing sarcoma can arise in either bone or soft tissue. The purpose of this study was to investigate whether patient characteristics, treatment strategies, and outcomes differ between skeletal Ewing sarcoma and extraskeletal Ewing sarcoma (EES). Patients <40 years of age with Ewing sarcoma or peripheral primitive neuroectodermal tumor reported to the United States Surveillance, Epidemiology, and End Results Program database from 1973 to 2007 were evaluated based on skeletal (n = 1519) versus extraskeletal (n = 683) site of origin. Patient characteristics were compared using Fisher exact tests. Overall survival was estimated via the Kaplan-Meier method and compared using log-rank tests and Cox proportional hazard models. Patients with EES had a higher mean age (19.5 vs 16.3 years; P < .001) and were less likely to be male (53.4% vs 63.3%; P < .001) or white (84.8% vs 92.5%; P < .001) compared with patients with skeletal tumors. Extraskeletal tumors were more likely to arise in axial locations (72.9% vs 54.2%; P = .001) but were less likely to arise specifically in the pelvis (19.8% vs 26.6%; P < .001). Metastatic status or tumor size did not differ by group. Five-year overall survival was superior for localized EES compared with localized skeletal tumors (69.7% vs 62.6%; P = .02). The hazard ratio for death in patients with localized skeletal tumors compared with localized EES was 2.36 (95% confidence interval, 1.61-3.44) beyond 24 months from initial diagnosis. Patient characteristics and outcomes differ among patients with EES compared with patients with skeletal Ewing sarcoma. These findings may have important implications for patient care.

Congenital extraskeletal Ewing′s sarcoma of chest wall - A rare case report

Congenital extraskeletal Ewing's sarcoma or peripheral primitive neuroectodermal tumor is an extremely uncommon and invariably fatal tumor. We report a case of extraskeletal congenital Ewing's sarcoma in a female fetus delivered at 34 weeks of gestation who died immediately after birth. In English literature, majority of cases of Ewing's sarcoma in neonates reported were skeletal. To the best of our knowledge, very few cases of extra-skeletal Ewing's sarcoma in neonates are reported in the literature.

Primary Tracheal Ewing's Sarcoma

Ewing's sarcoma is a malignant neoplasm usually occurring in the long and flat bones of children and adolescents. Extraskeletal Ewing's sarcoma is extremely rare, and we believe this has not been described previously in the trachea. We report a case of primary tracheal Extraskeletal Ewing's sarcoma that presented with acute airway obstruction. Stabilization of the airway was accomplished through a rigid bronchscopy, followed by debridement and stenting. The patient then underwent neoadjuvant chemotherapy followed by tracheal resection. Our case highlights the management of a tracheal mass and the multidisciplinary approach required for definitive treatment.

High Efficacy of Preoperative Low-Dose Radiotherapy with Sanazole (AK-2123) for Extraskeletal Ewing's Sarcoma: A Case Report

Extraskeletal Ewing's sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from Ewing's sarcoma of bone. We report a case of extraskeletal Ewing's sarcoma with several systemic problems. A 69-year-old man presented with a 5-month history of a rapidly enlarging mass in the right thigh. Because preoperative radiotherapy with sanazole (AK-2123) contributed the tumor mass reduction down to 40% in size, the tumor was successfully resected with clear surgical margins and repaired with a musculocutaneous flap. The high efficacy of pre-operative low-dose radiotherapy with sanazole was histologically confirmed that the resected tumor specimen involved no viable tumor cells and showed 100% necrosis. Based on clinical outcomes in this case, the combined modality of pre-operative low-dose radiotherapy with hypoxic cell radiosensitizer and adequate surgical resection might provide for the useful clinical application of extraskeletal Ewing's sarcoma treatment.

Extraskeletal Ewing's Sarcoma Presenting as a Mediastinal Mass

Ewing's sarcoma family of tumors is part of a rare group of malignant neoplasms with small blue, round-cell morphology on hematoxylin and eosin stain, expressing CD99, C-Kit, and Bcl2, and sharing the presence of the translocation t(11:22). Extraskeletal Ewing's sarcoma is a rare disease that typically involves the soft tissues of the trunk or extremities. We describe a case of extraskeletal Ewing's sarcoma presenting as a mediastinal mass in a 16-year-old boy.

RETRACTED ARTICLE: CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor

We report the CT and MR findings in a 30-year-old man with extraskeletal Ewing sarcoma (EES) involving the left neural foramen at L5-S1 level. The patient was evaluated with preoperative lumbosacral CT and MR imaging and postoperative lumbosacral MR imaging. The lesion was hyperdense on CT, isointense on T1- and T2-weighted MR images, and enhanced homogeneously after intravenous gadolinium injection. With these CT and MR findings, surgery was performed with a presumptive diagnosis of nerve sheath tumor, but the histopathological examination revealed EES. Chemotherapy and radiotherapy were planned postoperatively. This case illustrates that even in benign looking lesions in a neuroforamen one can never exclude malignancy, and this is even more true in a population of young adults.

CD99-positive large cell neuroendocrine carcinoma with rearranged EWSR1 gene in an infant: a case of prognostically favorable tumor

The first case of large cell neuroendocrine carcinoma arising in an infant is presented. The tumor arose at the anal verge of a 1-year-old girl. The diagnosis of this CD99-positive tumor was supported by expression of epithelial (keratins, EMA, and Ep-CAM) and neuroendocrine (chromogranin A, synaptophysin, and neuron-specific enolase) markers and absence of immunoreactivity for Fli-1. No fusion of EWSR1 with FLI-1 or ERG was detected by polymerase chain reaction. However, the split of the EWSR1 gene was demonstrated by fluorescence in situ hybridization. This case adds to the few epithelial tumors in which an EWSR1 rearrangement was demonstrated. Because the tumor was initially misclassified as an extraskeletal Ewing's sarcoma, the patient was treated according to the Ewing's sarcoma treatment protocol. She remains free of tumor 8 years after initial diagnosis.