Extraskeletal Ewing's Sarcoma Research Articles

Wunderlich syndrome as the first manifestation of an extraskeletal Ewing sarcoma.

We recently encountered an extremely rare case of spontaneous perirenal hemorrhage in a 34-year-old man. He initially had undergone radical nephrectomy owing to suspicion of renal cell carcinoma. The final diagnosis was extraskeletal Ewing sarcoma.

Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children’s Oncology Group.

10051 Background: The prognostic significance of having extraskeletal Ewing sarcoma (EES) using modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical chara...

The Rare Presentation of Extraskeletal Ewing's Sarcoma on the Forehead.

The Rare Presentation of Extraskeletal Ewing's Sarcoma on the Forehead.

A Unique Case of Primary Ewing's Sarcoma of the Cervical Spine in a 53-Year-Old Male: A Case Report and Review of the Literature.

Extraskeletal Ewing's sarcoma (EES) is a rare presentation, representing only 15% of all primary Ewing's sarcoma cases. Even more uncommon is EES presenting as a primary focus in the spinal canal. These rapidly growing tumors often present with focal neurological symptoms of myelopathy or radiculopathy. There are no classic characteristic imaging findings and thus the physician must keep a high index of clinical suspicion. Diagnosis can only be definitively made by histopathological studies. In this report, we discuss a primary cervical spine EES in a 53-year-old man who presented with a two-month history of left upper extremity pain and acute onset of weakness. Imaging revealed a cervical spinal canal mass. After undergoing cervical decompression, histopathological examination confirmed a diagnosis of Ewing's sarcoma. A literature search revealed fewer than 25 reported cases of primary cervical spine EES published in the past 15 years and only one report demonstrating this pathology in a patient older than 30 years of age (age = 38). Given the low incidence of this pathology presenting in this age group and the lack of treatment guidelines, each patient's plan should be considered on a case-by-case basis until further studies are performed to determine optimal evidence based treatment.

Extraskeletal Ewing Sarcoma in a Young Patient During Pregnancy

Background: Ewing’s sarcoma is usually identified as a primary malignancy of the bone affecting children and young adults. It is associated with soft tissue extension in 90% of cases. Primarily extraskeletal Ewing’s sarcoma is a rare subtype, and very few data are available concerning optimal treatment modalities. Case Presentation: We report a case of an 18 year-old female from Cabo Verde in her 34th week of pregnancy presenting with a rapid growing cervical tumor. It had initially started as an indolent swelling at the left cervico-posterolateral region. CT had shown involvement of both superficial and deep cervical fascia. At time of presentation, the histologic diagnosis of a peripheral Primitive Neuroectodermal Tumor/Extraskeletal Ewing’s sarcoma had already been provided. To reduce risk of fetal metastasis, the child was delivered by caesarean section. A chest CT of the patient revealed pulmonary metastasis and compressive atelectasis of the left lung caused by the tumor. Three weeks post partum, she presented with loss of muscular function of the extremities as well as parestesia and bladder dysfunction. A repeat CT confirmed medullar compression at the level of C5-C6, C6-C7 and C7-D1 by the tumor without signs of bone metastasis. 5 sessions of palliative radiotherapy were performed that led to parcial recovery of muscular function of the upper right extremity. Palliative chemotherapy was refused, so that the patient was released from hospital. No metastasis had been detected in the child so far. Conclusion: Extraskeletal Ewing’s sarcoma can manifest as a rapidly growing localized mass causing local compression symptoms. Palliative radiotherapy can achieve local tumor reduction and symptom relief.

Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution.

ObjectiveTo comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults.Materials and MethodsWe performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ≥ 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs.ResultsAmong the 70 patients (mean age, 35.8 ± 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%).ConclusionPrimary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.

Extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of the mediastinum: Significant response to chemoradiotherapy.

Primary mediastinal extraskeletal Ewing’s sarcoma (EES) is quite rare. To the best of our knowledge, only five cases have been reported. Given the paucity of data, there is consequently no optimal treatment strategy available. The current study presents the case of a 51-year-old female with unresectable EES of the posterior mediastinum. Chemoradiotherapy achieved near complete remission without severe side-effects. The literature associated with EES is also reviewed. The present case highlights the possibility of the diagnosis of EES for a mediastinal mass. Chemoradiotherapy may be a good option for unresectable cases. In the future, large-scale collaborative clinical trials should be initiated to provide an improved understanding of the characteristics of EES and the best treatment strategy.

An Unusual Case of Extradural Primary Ewing Sarcoma of Lumbar Spine in an Adult

Ewing sarcoma (ES) is a malignant neoplasm of bones with mesenchymal origin that rarely occurs in extraosseous sites, most frequently presents in the pediatric population and is extremely rare in the adult population, with only 30 extradural spine cases reported in the literature. We present a case of an extraskeletal ES that belongs to the primitive neuroectodermal tumor/ES family occurring in the lumbar epidural space. A 46-year-old woman was admitted to neurosurgery department with the complaint of 1 month of worsening leg pain. Lumbosacral magnetic resonance imaging revealed the presence of a large, enhancing, extradural mass lesion at the level of L4 without any bony involvement. The patient underwent laminectomy and total tumor resection. ES diagnosis arising in the lumbar epidural space was confirmed by histopathologic examination. The patient received both chemotherapy and radiotherapy after the operation. Hereby, we report an extremely rare case of primary ES in adult patient with regard to tumor resection, prevention of recurrence, and metastasis.

Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients

Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients

Prevalence of malignant soft tissue tumors in extremities: an epidemiological study in syria.

Although the majority of soft tissue masses are benign, it is important to consider malignancy in differential diagnoses. Because most soft tissue sarcomas present as a painless mass, clinicians must watch for signs suggestive of malignancy, including large size, rapid growth, and site deep into the deep fascia. The purpose of this study was to determine the relative prevalence according to sex and age, site of tumor, skeletal distribution, and treatment (surgery, chemotherapy and radiotherapy) before and after surgery, and ascertain the relative frequency of these tumors in specific anatomic sites and age groups based on pathological studies. A total of 308 patients, with a musculoskeletal tumor were evaluated retrospectively. All of the patients enrolled into this study were referred to the Beirouni Hospital of Damascus University with a proven diagnosis of malignant soft tissue tumors from the beginning of January 2008 until the end of 2010. The prevalence of the malignant soft tissue tumors in these patients was analyzed. For purposes of analysis, all lesions were placed in 1 of 9 categories: hand and wrist, forearm, humorous (arm), proximal limb girdle (axilla and shoulder), foot and ankle, thigh, hip and buttocks region, trunk, and other lesions. Age and sex also were recorded. MALIGNANT TUMORS CONSISTED OF SEVEN DIAGNOSTIC CATEGORIES: malignant fibrous histiocytoma (23%), liposarcoma (22%), rhabdomyosarcoma (9%), leiomyosarcoma (8%), malignant schwannoma (5%), dermatofibrosarcoma protuberans (5%), synovial sarcoma (10%), fibrosarcoma (13%), extraskeletal chondrosarcoma (1%), and extraskeletal Ewing sarcoma (4%). Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the site of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in establishing a suitably ordered differential diagnosis when a soft-tissue tumor has a nonspecific radiologic appearance.

Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients.

To describe the multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma (EES). In this institutional review board-approved, health insurance portability and accountability act-compliant retrospective study, we included 26 patients (17 females and 9 males; mean age, 36 years; range, 18-85 years) with pathologically confirmed EES seen at our institute between 1999 and 2011, who had imaging of primary tumour. Imaging of primary tumour in all 26 patients and follow-up imaging in 23 was reviewed by two radiologists in consensus. Clinical data were extracted from electronic medical records. The most common primary sites were the torso (n = 13), extremities (n = 10) and head and neck (HN) region (n = 3). The mean tumour size was 9 cm (range, 3-22 cm); tumours of the torso were larger than those of other areas (p > 0.05). Compared with the skeletal muscle, tumours were isodense on CT (21/21), hypointense (n = 5) to isointense (n = 14) on T1 weighted image, hyperintense on T2 weighted image (19/19) and were fluorine-18 fludeoxyglucose ((18)F-FDG)-avid [10/10; mean maximum standardized uptake value of 7 (range, 3-11)]. Necrosis (15/26), haemorrhage (5/26) and adjacent organ invasion (14/26) were present without calcification. Median follow-up was 16 months. 5 patients had local recurrence (torso, 3; extremity, 1; and HN, 1). Metastases developed in 11 patients (torso, 7; extremities, 3; and HN, 1; p > 0.05); 8 at presentation, most commonly to lung (9/11), peritoneum (4/11), muscles (4/11) and lymph nodes (4/11). Nine patients (torso, 7; extremity, 1; and HN, 1) died (10 months median survival) (p > 0.05). Adult EESs are large tumours, which frequently invade adjacent organs and metastasize to the lung. EESs of the torso are larger, have more frequent metastases and poorer outcomes. Adult EESs of the torso have poor outcomes compared with other EESs.

Phase I and pharmacokinetic study of trabectedin, a DNA minor groove binder, administered as a 24-h continuous infusion in Japanese patients with soft tissue sarcoma

Summary Background Trabectedin is a novel anticancer agent used to treat soft tissue sarcoma (STS). This phase I study of trabectedin was performed to determine the recommended dose for phase II studies in Japanese patients with STS. Methods Patients who had STS refractory to, or who could not tolerate, anthracycline-based chemotherapy were enrolled. The starting dose of trabectedin was 0.9 mg/m2, given as a 24-h continuous infusion every 21 days. The dose was escalated to 1.2 mg/m2 and then to 1.5 mg/m2, using a “3 + 3” cohort expansion design. Plasma samples were collected for pharmacokinetic analysis. Results Fifteen patients received 1 of 3 dose levels of trabectedin. Dose-limiting toxicity occurred in two of three patients at 1.5 mg/m2: 1 had a grade 3 increase in creatine phosphokinase and grade 3 anorexia, and the other had grade 4 platelet count decreased. Frequent grade 3 or 4 adverse events (AEs) included elevations of alanine aminotransferase and aspartate aminotransferase and decrease in neutrophil count. The frequency and severity of AEs were clearly greater at 1.5 mg/m2 than at the lower doses. Pharmacokinetic analysis showed that the area under the concentration-time curve at a dose of 1.2 mg/m2 was adequate to produce antitumor activity. A partial response was obtained in three patients with translocation-related sarcomas (1 each with myxoid liposarcoma, synovial sarcoma, and extraskeletal Ewing sarcoma). Conclusions The recommended dose of trabectedin for phase II studies is 1.2 mg/m2 in Japanese patients with STS. Trabectedin may be especially effective against translocation-related sarcomas.

Primary cutaneous extraskeletal Ewing's sarcoma/PNET: possibility of better prognosis than deep ES/PNET

Extraskeletal Ewing sarcoma (ES) and Primitive Neuroectodermal Tumour (PNET) were first described by Angerval and Enzinger in 1975 [1]. Primary ES/PNET arising in skin is distinctly rare [2-6]. Unlike ES/PNET in deep soft tissue, which has a poor prognosis, cutaneous and subcutaneous ES seem to be associated with an indolent course and a favourable prognosis [3-5, 7]. We report a case of cutaneous ES (CES) in a young adult and additionally review the cases of CES which have been previously reported [...]

Primary Extraskeletal Ewings Sarcoma of Ala of Nose.

Primary Extraskeletal Ewings Sarcoma of Ala of Nose.

P1–091 - A Case of Extra-Skeletal Ewing Sarcoma Elevated with Plasma Pro-GRP and Serum CA125

We experienced the extra-skeletal Ewing sarcoma in which plasma progastrin-releasing peptide (Pro-GRP) and serum carbohydrate antigen 125 (CA125) were elevated at diagnosis and correlated with his clinical course.

Retraction Note to: 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.

Extraskeletal Ewing’s Sarcoma Arising in the Larynx

Extraskeletal Ewing's sarcoma (EES), or primitive neuroectodermal tumor, is an uncommon neoplasm with low incidence in the head and neck. Occurrences in the larynx are even more exceptional with only two previous reported cases of EES arising from the larynx. We report the case of a 45-year-old woman with a laryngeal Ewing's sarcoma treated with chemotherapy with radiotherapy to follow. Here we describe the histology, molecular diagnosis and treatment of this unusual tumor.

Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin

The aim of the study was to determine the impact of negative but close resection margins on local recurrence in children with extraskeletal Ewing sarcoma (EES). We reviewed records of 32 patients with EES treated between March 2005 and March 2013. All patients except one underwent surgical excision either upfront or after induction chemotherapy. Patients with viable tumor and negative surgical margins, which were categorized as less than or greater than 1 cm, were selected. Local control and survival analysis were performed for patients in both the groups. The 5-year event-free and overall survival rates of entire cohort is 68 and 77 %, respectively. Surgical margins were negative in 23/26 (90.3 %) patients. There were no local recurrences in any of the patients with margins of less than 1 cm. Only one patient with a margin greater than 1 cm had a local recurrence along with distant metastases. A tumor-free margin of more than 1 cm did not affect overall or event-free survival (p = NS). Optimal local control is feasible in children with EES regardless of the quantitative extent of negative margins. Achieving a three-dimensional tumor-free margin should be the goal of surgical resection.

Ewing’s sarcoma of the larynx

Extraskeletal Ewing's sarcoma arising in the head and neck region is an extremely rare malignant neoplasm. We describe the unusual case of a tumor originating in the larynx of a 68-year-old male with hoarseness and occasional aphonia. We report successful treatment with sequential chemo- and radiotherapy. Complete regression and larynx preservation with voice function recovery was achieved. To our knowledge, this is the first report of this type of tumor in the larynx with cartilage invasion that documents the effectiveness of radiotherapy as an alternative to surgical management. At present, after 30months of follow-up, the patient is free of tumor relapse and in very good condition.

Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS. EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.