Extraskeletal Ewing's Sarcoma Research Articles

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

BackgroundSpinal 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 presentationWe 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.ConclusionsIntradural 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.

Extraskeletal Ewing sarcoma arising from stomach and ovary: A case series of two rare sites

Ewing sarcoma is the second most common tumor in children and young adults. Primitive neuroectodermal tumors/Ewing sarcoma family consist of small round cell of neuroectodermal origin. Ewing sarcoma commonly arises from long bones and pelvic region but extraskeletal Ewing sarcoma (EES) is a very rare tumor with highly malignant potential associated with poor outcome. We present a case series of two cases, one is from the ovary in a 24-year-old young, married, multiparous woman who presented with metastatic disease and was treated with palliative chemotherapy. The second is an Ewing sarcoma arising from the stomach in a 33-year-old male who underwent surgery followed by adjuvant chemotherapy. EES is usually an uncommon tumor and many times, the diagnosis becomes delayed and presents with metastatic disease. Immunohistochemical and molecular analysis is a must to diagnose the EES. There are no standard treatment guidelines. Surgery, various chemotherapy regimens, and radiotherapy are given for treatment. Early diagnosis and interventions are required for better outcomes.

Rare origin - Ewing's sarcoma of the pleura: a case report and literature review

Ewing sarcoma (ES) was first reported by Ewing in 1921. It is the second largest malignant bone tumor in children and adolescents, typically occurring in the bones of trunk or limbs . Extraskeletal Ewing sarcoma (EES) was first reported by Tefft et al. in 1969 and is extremely rare, accounting for less than 1% of all sarcomas. It can occur in any part of soft tissue, mostly in the trunk and lower limbs, and rarely in the pleura. We report a 22-year-old case of extraosseous Ewing sarcoma of pleural origin discovered and pathologically confirmed by physical examination. We report its CT manifestations and pathological results, and review the literature to summarize and analyze the clinical and imaging characteristics of extraosseous Ewing sarcoma, in order to improve our understanding of the disease.

Ewing sarcoma: a tumor with an uncanny predilection for uncommon sites

Ewing sarcoma is one of the most common bone tumors diagnosed in pediatric age group second only to osteosarcoma. The tumor is known to involve most commonly pelvic bones and long bones while extra-skeletal Ewing sarcoma accounts for 15%. Ewing sarcoma of other sites like scapula, clavicle, hands, and feet are quite rare, accounting for only 3-5%. To determine the epidemiological profile of Ewing sarcoma of unusual sites treated in our department including age at diagnosis, site of origin and mode of management along with survival data. Details of Ewing sarcoma patients reported our department in last 9 years were collected and data was analysed. We report a total of 20 cases of Ewing sarcoma in last 9 years with 4 (20%) extra skeletal Ewing sarcoma arising from soft tissues of extremities and 30% from rare sites (3 scapula, 1 clavicle, 1 phalanx, 1 calcaneum). All patients were operated after 4-7 cycles of chemotherapy with 66% limb salvage rate. With an average necrosis of 40%, with a median follow up of 36 months, the overall survival of the group was found to be 77.7%. Ewing sarcoma is one of the rare tumors of bone and soft tissue with the predilection for unusual sites, with reasonable survival outcomes in localized disease. Though scapula, clavicle, hands and feet are rare sites for Ewing sarcoma, they accounted for 30% in our department, possibly because of the referral patterns.

Gastrointestinal Ewing Sarcoma: A Clinicopathological and Molecular Genetic Analysis of 25 Cases.

Occurrence of extraskeletal Ewing sarcoma (ES) in the gastrointestinal (GI) tract is extremely rare. Here, we report 25 cases of ES arising primarily in the GI tract with a focus on the clinicopathological and molecular features, differential diagnosis, and biological behavior. Thirteen patients (52%) were male, and 12 (48%) were female with age ranging from 9 to 59 years (mean: 36.2 years; median: 38 years). Twenty-one tumors (84%) occurred in the small intestine, 3 (12%) in the stomach, and 1 (4%) in the anal canal. At operation, 8/18 (44.4%) patients presented with abdominopelvic disseminated disease. Tumor size measured from 2 to 25cm (mean: 8.2cm; median: 6cm) in maximum size. Microscopically, the tumors were composed of infiltrative small round, ovoid, or short spindle cells arranged mostly in lobular and solid sheet-like patterns with a rich capillary vasculature. Focal formation of Homer Wright-type rosettes and pseudoalveolar architecture was noted each in 2 (8%) cases and 3 (12%) cases. Besides CD99 (25/25; 100%), Fli-1 (15/15, 100%), and NKX2.2 (14/16; 87.5%), the tumor cells also showed variable staining of CD117 (14/17; 82.4%). Of 25 cases, 23 (92%) demonstrated EWSR1 rearrangement by fluorescence in situ hybridization analysis. The 2 cases with negative fluorescence in situ hybridization results were found to harbor EWSR1::ERG and EWSR1::FLI1 fusion by further RNA sequencing, respectively, with a median follow-up of 12 months (range: 1 to 42 months), 5/19 (26.3%) patients developed visceral metastasis and 12/19 (63.2%) patients died of the disease (range:1 to 33 months; median: 9 months). This study showed that GI ES had a predilection for the small intestine, although other sites of the GI tract could also be involved. GI ES had a poor prognosis with a high rate of mortality, particularly in patients with abdominopelvic disseminated disease. In light of appropriate therapeutic strategies and prognostic considerations, it is essential not to misdiagnose GI ES as gastrointestinal stromal tumor owing to the expression of aberrant CD117.

S4003 Multiple Primary Bone Tumors in the GI Tract? Extra-Skeletal Ewing Sarcoma in the Stomach, Duodenum, and Pancreas

S4003 Multiple Primary Bone Tumors in the GI Tract? Extra-Skeletal Ewing Sarcoma in the Stomach, Duodenum, and Pancreas

Primary pancreatic Ewing sarcoma with metastases on FDG PET/CT

BackgroundEwing sarcoma (ES) is a highly aggressive malignant tumor most commonly affecting long bones. Extraskeletal Ewing sarcoma (EES) is a malignant tumor with aggressive behavior carrying bad prognosis with pancreas being an extremely rare primary site. We present a case of histopathologically proven EES of the pancreas in a young female who presented with abdominal pain. 18F-fluorodeoxyglucose positron emission tomography (18F FDG PET/CT) is a useful modality for detecting distant metastases in EES. It helps in diagnosis, localizing the primary, its extension, optimal treatment planning and evaluation of response to standard treatments available.Case presentationAn 18-year-old female presented with complaints of progressive abdominal pain and distention since 6 weeks. Physical examination was suggestive of a solid large mass in the upper left abdomen and decreased breath sounds with dullness in the left lower lung fields. On Contrast enhanced computed tomography (CECT) imaging, a large heterogeneously enhancing mass was seen arising from pancreas along with retroperitoneal lymphadenopathy. A moderate sized left sided pleural effusion with atelectasis of lower lobe of left lung was also noted. Histopathological analysis was suggestive of pancreatic ES following which the patient underwent five cycles of chemotherapy. Following this, she underwent 18F FDG PET/CT which showed hypermetabolic large mass arising from body and tail of pancreas with areas of internal necrosis along with left adrenal metastasis, retroperitoneal lymphadenopathy, a massive left pleural effusion and compressive atelectasis of left lower lobe. The patient expired within a week following PET/CT.ConclusionsEES most often presents in the late stage of the disease with vague symptoms. Timely diagnosis and initiation of treatment is of utmost importance considering the aggressiveness of the tumor. Establishing a diagnosis of Ewing sarcoma is especially difficult when the mass is arising from the pancreas. Imaging, histopathology and immunohistochemistry (IHC) play a key role in accurate diagnosis of such masses. 18F FDG PET/CT can be useful for detecting local and distant spread, operability, treatment planning and evaluation of response to chemotherapy.

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.

CORR Insights®: Is Bone Marrow Aspiration and Biopsy of Clinical Importance in the Initial Staging of Extraskeletal Ewing Sarcoma?

1Professor and Chair, Department of Orthopedic Surgery, School of Medicine, National & Kapodistrian University of Athens, Attikon University General Hospital, Athens, Greece P. J. Papagelopoulos, National & Kapodistrian University of Athens, School of Medicine, Attikon University General Hospital, First Department of Orthopaedic Surgery, Neapoleos 15, Amarousion, Athens, 15123, Greece, Email: [email protected] This CORR Insights® is a commentary on the article “Is Bone Marrow Aspiration and Biopsy of Clinical Importance in the Initial Staging of Extraskeletal Ewing Sarcoma?” by Wahlig and colleagues available at: DOI: 10.1097/CORR.0000000000002661. The author certifies that there are no funding or commercial associations (consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the submitted article related to the author or any immediate family members. All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research® editors and board members are on file with the publication and can be viewed on request. The opinions expressed are those of the writer, and do not reflect the opinion or policy of CORR® or The Association of Bone and Joint Surgeons®.

КОСТНАЯ И ВНЕСКЕЛЕТНАЯ САРКОМА ЮИНГА: СРАВНИТЕЛЬНАЯ ХАРАКТЕРИСТИКА ОСОБЕННОСТЕЙ ТЕЧЕНИЯ И ИСХОДОВ ЗАБОЛЕВАНИЯ (ОПЫТ НИИ ДЕТСКОЙ ОНКОЛОГИИ И ГЕМАТОЛОГИИ им. Л.А. ДУРНОВА)

Ewing's sarcoma (ES) is a highly malignant tumor of children and adolescents that affects bones and soft tissues with the same frequency. The origins of ES are the subject to many discussions. Differential diagnosis is complicated and requires a full range of immunohistochemical and molecular genetic studies. The prognostic value for extraskeletal and bone ES under current chemotherapy protocols is unknown and requires further analysis. The purposes of this research were a comparative analysis of clinical characteristics, therapeutic approaches and outcomes of the disease in patients with extraskeletal and bone localizations of ES. Materials and methods used: a single-center retrospective cohort study was conducted, which included 330 patients (237 (71.8%) boys/93 (28.2%) girls) aged 0 to 18 y/o (median 11 [7; 14] y/o) with confirmed diagnosis of ES who received treatment in 2008-2022, of which 280 (84.85%) with primary bone localization (group of bone ES - BES), and 50 (15.15%) with soft tissue localization (group of extraskeletal ES - ESES). Comparative analysis of survival rates for primary tumor localization in the area of bones and soft tissues was performed. The median follow-up time for all patients was 35.5 [18.2; 68.5] months, 37.0 [18.0; 71.0] months with BES, and 29.5 [16.8; 65.5] months with ESES. All patients received treatment according to the protocols adopted at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia): MMES-99, ES-2017. Overall survival (OS) was calculated with the Kaplan-Meier estimator. Results: the selected groups differed statistically significantly by gender (74% of boys in the BES group, and 60% of boys the ESES group, p=0.035) and age (10.5 [8; 15] years in the BES group, and 8.5 [4; 12] y/o in the ESES group, p=0.001). BES was diagnosed statistically significantly more often in older age groups than ESES (p=0.004). Compared with BES, in ESES the tumor was statistically significantly more often located in the region of the axial skeleton and visceral organs (24.0% vs. 56%, p<0.001). Disseminated form of the disease in the BES group was recorded in 110 (39.3%) patients, and in 15 (30.0%) in the ESES group. Authors did not find statistically significant differences in overall 5-year OS for localized forms of BES and ESES (79% and 78.5%, respectively), the median OS in these groups was not reached. The OS of patients with disseminated stages of BES and ESES was statistically significantly lower than in the group of localized forms. At the same time, the 5-year OS was 41.2% and 40.6%, the median OS was 46.9 and 28.4 months (p=0.001, respectively). Differences in 5-year progression-free survival (PFS) for localized forms were 71.6% for BES and 75.6% for ESES (p=0.001), for disseminated forms - 32.4% vs. 44.9% (p=0.036, respectively). In the disseminated stage of BES, progression/relapse was detected in 50% of cases after 21.1 months, and after 20.3 months for ESES. Conclusions: Authors have identified the following unfavorable prognostic factors. As for BES these were: older age, disease stage, primary tumor volume over 200 cm3 and length over 8 cm, radiation therapy in the treatment program or combined treatment. As for ESES, the unfavorable PFS factors were the stage of the disease, the volume of the primary tumor and the process dissemination. In this regard additional analysis of key genomic characteristics is required in order to further determine the risk group stratification and prognosis.

КОСТНАЯ И ВНЕСКЕЛЕТНАЯ САРКОМА ЮИНГА: СРАВНИТЕЛЬНАЯ ХАРАКТЕРИСТИКА ОСОБЕННОСТЕЙ ТЕЧЕНИЯ И ИСХОДОВ ЗАБОЛЕВАНИЯ (ОПЫТ НИИ ДЕТСКОЙ ОНКОЛОГИИ И ГЕМАТОЛОГИИ им. Л.А. ДУРНОВА)

Ewing's sarcoma (ES) is a highly malignant tumor of children and adolescents that affects bones and soft tissues with the same frequency. The origins of ES are the subject to many discussions. Differential diagnosis is complicated and requires a full range of immunohistochemical and molecular genetic studies. The prognostic value for extraskeletal and bone ES under current chemotherapy protocols is unknown and requires further analysis. The purposes of this research were a comparative analysis of clinical characteristics, therapeutic approaches and outcomes of the disease in patients with extraskeletal and bone localizations of ES. Materials and methods used: a single-center retrospective cohort study was conducted, which included 330 patients (237 (71.8%) boys/93 (28.2%) girls) aged 0 to 18 y/o (median 11 [7; 14] y/o) with confirmed diagnosis of ES who received treatment in 2008-2022, of which 280 (84.85%) with primary bone localization (group of bone ES - BES), and 50 (15.15%) with soft tissue localization (group of extraskeletal ES - ESES). Comparative analysis of survival rates for primary tumor localization in the area of bones and soft tissues was performed. The median follow-up time for all patients was 35.5 [18.2; 68.5] months, 37.0 [18.0; 71.0] months with BES, and 29.5 [16.8; 65.5] months with ESES. All patients received treatment according to the protocols adopted at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia): MMES-99, ES-2017. Overall survival (OS) was calculated with the Kaplan-Meier estimator. Results: the selected groups differed statistically significantly by gender (74% of boys in the BES group, and 60% of boys the ESES group, p=0.035) and age (10.5 [8; 15] years in the BES group, and 8.5 [4; 12] y/o in the ESES group, p=0.001). BES was diagnosed statistically significantly more often in older age groups than ESES (p=0.004). Compared with BES, in ESES the tumor was statistically significantly more often located in the region of the axial skeleton and visceral organs (24.0% vs. 56%, p<0.001). Disseminated form of the disease in the BES group was recorded in 110 (39.3%) patients, and in 15 (30.0%) in the ESES group. Authors did not find statistically significant differences in overall 5-year OS for localized forms of BES and ESES (79% and 78.5%, respectively), the median OS in these groups was not reached. The OS of patients with disseminated stages of BES and ESES was statistically significantly lower than in the group of localized forms. At the same time, the 5-year OS was 41.2% and 40.6%, the median OS was 46.9 and 28.4 months (p=0.001, respectively). Differences in 5-year progression-free survival (PFS) for localized forms were 71.6% for BES and 75.6% for ESES (p=0.001), for disseminated forms - 32.4% vs. 44.9% (p=0.036, respectively). In the disseminated stage of BES, progression/relapse was detected in 50% of cases after 21.1 months, and after 20.3 months for ESES. Conclusions: Authors have identified the following unfavorable prognostic factors. As for BES these were: older age, disease stage, primary tumor volume over 200 cm3 and length over 8 cm, radiation therapy in the treatment program or combined treatment. As for ESES, the unfavorable PFS factors were the stage of the disease, the volume of the primary tumor and the process dissemination. In this regard additional analysis of key genomic characteristics is required in order to further determine the risk group stratification and prognosis.

Rectal Ewing’s Sarcoma: A Case Report of An Unusual Extra-Skeletal Tumor Presentation

Introduction: Extraskeletal Ewing’s sarcoma (EES) is a rare malignant soft tissue tumor. Those aggressive mesenchymal tumors are characterized by genetic alterations of the Ewing sarcoma gene (EWS) on chromosome 22. Signs and symptoms are usually non-specific. The clinical diagnosis of EES is challenging. Case Presentation: A 31-year-old female patient presented to our hospital with rectal bleeding. Physical examination revealed a painful and erythematous bleeding rectal mass. A pelvic MRI revealed a right-sided well-circumscribed perianal mass measuring 6.8 × 6.0 cm. Surgical excision of the rectal mass was subsequently performed. The resected specimen, including the rectal mass with a pedicle from the posterior rectal wall was sent for histopathological examination. The postoperative period was uneventful. Histopathology examination was suggestive of EES of the rectum. Clinical Discussion: EES is an unusual entity of Ewing's Sarcoma Family of Tumors, which are characterized by pathognomonic translocations. The clinical features of EES include localized pain and/or swelling. Diagnosis of EES relies on histopathology and immunohistochemistry analysis. Imaging modalities such as CT, MRI, and PET/CT are crucial to evaluate EES metastasis and assess local tumor resectability. Conclusion: Due to the rare entity of EES of the rectum, we report the case of a 31-year-old female with rectal bleeding, found to be rectal Ewing’s sarcoma.

Posterior mediastinal Ewing sarcoma with multiple metastases on FDG PET/CT: a rare entity

BackgroundEwing sarcoma and peripheral primary neuroendocrine tumors are aggressive neoplasms which consist of small, round, blue cells of neuroectodermal origin. They usually arise from the skeleton and consist of genetic mutations EWSR1 in chromosome 22 and FL1 gene on chromosome 11. Extraskeletal Ewing sarcomas (EES) are rare entities with most common sites of EES being extremities, head and neck region and retroperitoneum. Posterior mediastinal Ewing sarcoma is rare. For its evaluation, 18F-fluorodeoxyglucose positron emission tomography (18F FDG PET/CT) plays significant role in staging, management planning and prognostication.Case presentationWe describe a rare case of EES of posterior mediastinum in a 20-year-old boy who presented with signs of upper motor neuron lesion below D10 level. Contrast-enhanced magnetic resonance imaging (CEMRI) showed a heterogeneously enhancing posterior mediastinal mass in pre- and paravertebral region with intraspinal extension in D2-D4 levels. Fluorodeoxyglucose PET/CT showed a metabolically active mass occupying the superior, middle and posterior mediastinum on the left, displacing the trachea and esophagus toward the right side and causing complete collapse of the left lung. Posteriorly the mass was seen destroying the D2-D5 vertebrae with intraspinal extension at D2-D4 level. Metabolically active metastatic disease was seen in pleura, skull, D12 vertebra, right iliac bone and bilateral proximal femorae. Biopsy obtained from lung and adjacent pleura showed features of a round cell tumor positive for NKX 2.2, weak positive for FLI 1 and negative for PAN CK, LCA, Vimentin and TLE-1, suggestive of Ewing sarcoma. Based on these investigations, a diagnosis of EES of posterior mediastinum was made.ConclusionExtraskeletal Ewing sarcoma of posterior mediastinum is a rare and aggressive entity. Management of metastatic EES comprises radiotherapy and systemic chemotherapy which reduces tumor burden and micrometastasis. However, response to treatment in metastatic EES is poorer than in localized disease with overall 5-year survival rates of less than 30%. Fluorodeoxyglucose PET/CT can be a useful tool to accurately detect the extent of local disease in the presence of atelectasic lung for radiotherapy planning as well as evaluating response to therapy.

Is Bone Marrow Aspiration and Biopsy of Clinical Importance in the Initial Staging of Extraskeletal Ewing Sarcoma?

Extraskeletal Ewing sarcoma are rare tumors within the Ewing sarcoma family. Initial staging studies for extraskeletal Ewing sarcoma historically have included imaging and bone marrow aspiration and biopsy (BMAB). However, recent studies on Ewing sarcoma of bone have questioned the utility of BMAB in the initial staging of patients, but no studies of which we are aware have evaluated the role of BMAB in extraskeletal Ewing sarcoma. We suspected that BMAB was of low diagnostic yield in patients with extraskeletal Ewing sarcoma and exposed patients to potential morbidity without an impact on their clinical course. Is BMAB a useful test in the staging of extraskeletal Ewing sarcoma? Between January 1996 and December 2021, our institution evaluated 109 patients with a listed diagnosis of extraskeletal Ewing sarcoma. Those patients were retrospectively reviewed for this study. Of those, we considered patients with biopsy-confirmed diagnosis of extraskeletal Ewing sarcoma. Biopsy was performed based on institutional protocols, with all diagnoses assigned by a board-certified pathologist. Based on that criteria, 96% (105 of 109) were eligible. An additional 18% (20 of 109) were excluded because records of their initial diagnostic and staging workup were not available. This left 78% (85 of 109) for analysis. Of those, 52% (44 of 85) were male. The average age was 32 ± 16 years. Primary tumor locations included extremities in 26% (22 of 85), paraspinal in 20% (17 of 85), chest in 19% (16 of 85), retroperitoneum in 13% (11 of 85), intraabdominal in 12% (10 of 85), intrapelvic in 7% (6 of 85), and head or neck in 4% (3 of 85). Initial diagnostic and staging information, including the use of PET-CT, bone scan, CT chest, and BMAB, was collected. Metastatic disease at the time of presentation or during follow-up was noted. The utility of BMAB was determined by the rate of positive tests in those undergoing BMAB during the initial staging process. Descriptive statistical analysis was sufficient to address the study question, and therefore no comparative statistics were performed. BMAB was obtained during the initial staging process in 64% (54 of 85) of patients. This BMAB was negative in all 54 patients, including those with known metastatic disease. Diagnosing metastatic disease in extraskeletal Ewing sarcoma is important as the presence of metastases at diagnosis adversely affects prognosis. The routine use of BMAB in the staging process of extraskeletal Ewing sarcoma is of low diagnostic yield. BMAB is unlikely to diagnose metastatic involvement even in patients with known metastases to bone. We do not have enough data to suggest whether other modalities, such as PET-CT, might be more useful. Similar studies should be pursued to determine the utility of the remainder of staging modalities in patients with extraskeletal Ewing sarcoma to elucidate the most efficient and effective staging protocol. Level III, diagnostic study.

Ewing's sarcoma in adolescents and adults - 10-year experience from a tertiary cancer center in India.

Ewing's sarcoma (EWS) is an aggressive small round cell tumor, affecting bone and soft tissues and is mostly seen in childhood and second decade of life. EWS accounts for 10-12% of bone tumors in more than 15 years age group and is even rarer after 40 years of age. This retrospective analysis was conducted among patients aged more than 15 years with histologically proven EWS. Among 240 cases of EWS treated at our center during 2001-2010, 130 (54%) were more than 15 years of age. The median age was 20 years with a male: female ratio of 2.4:1. Ninety percent had skeletal EWS, 10% had extra skeletal EWS, and 37% patients were metastatic at presentation. Eighty-two received curative treatment with chemotherapy (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide (VAC/IE)) along with local treatment, radiotherapy (RT) in 61, surgery alone in seven, and RT plus surgery in 14. Two- and 5-year overall survival (OS) was 43.3% and 25.5%, respectively, for the entire series. The OS for the non-metastatic group was 63.2% at 2 years and 36.5% at 5 years, and the progression free survival was 53.7% at 2 years and 37.8% at 5 years. High lactate dehydrogenase was found to be a significant poor prognostic factor (P = 0.001). Median OS for localized central EWS was 49.2 months and that for peripheral EWS was 24 months. Patients more than 20 years of age with non-metastatic disease had better OS compared to those with 15-20 years of age. Treatment of EWS requires a multidisciplinary approach with radical surgery and/or radiation to control local disease and multiagent chemotherapy to control systemic disease. Long-term follow-up is essential because of disease relapse and treatment-related complications.

A Teenage Girl with Large Lung Mass

Extraskeletal Ewing Sarcoma is rarely reported. A fifteen-year-old girl presented with the right-sided pleuritic chest pain, shortness of breath, and productive cough with whitish sputum associated with poor oral intake. She subsequently underwent contrast-enhanced Computerized Tomography thorax, which showed lobulated heterogenous hypodensity occupying the entrance of the right superior pulmonary vein, extending to the left atrium.

Retroperitoneal Extraskeletal Ewing’s Sarcoma in Adult

Ewing’s sarcoma is a highly malignant tumour of osseous or non-osseous origin, typically seen in the paediatric and adolescent age group. However, extraskeletal Ewing’s sarcoma is an uncommon tumour. It could arise from the soft tissue in the paravertebral area, chest wall, head and neck, and retroperitoneum. Retroperitoneal extraosseous Ewing’s sarcoma is even rarer, with only a few reported cases in the literature. We describe the radiologic findings of this rare retroperitoneal extraskeletal Ewing sarcoma manifested in an adult patient. Our patient is a 32-year-old gentleman who presented with abdominal pain and constitutional symptoms for two months. Imaging shows a retroperitoneal tumour with local infiltration. It was confirmed via histopathological analysis as retroperitoneal Ewing’s sarcoma. We discussed the diagnostic strategy as well as the literature review of this rare disease.

Sino-nasal and pharyngeal mass- Immunohistochemistry cuts the gordian knot

Sinonasal and pharyngeal mass lesion of Extraskeletal Ewing sarcomas (EEWSs) are extremely rare. Herein we present a case of EEWSs of ethmoid sinus extending into nasal and pharyngeal spaces mimicking as angiofibroma clinically. Histopathological examination in conjunction with immunohistochemistry puzzled out the diagnostic dilemma.

Sarcoma de Ewing extraesquelético: relato de um caso raro e exuberante simulando lipoma

Extraskeletal Ewing sarcoma (EES) is a rare tumor that affects men between 10-20 years old and represents 25% of Ewing sarcomas. We report a case due to the previous diagnosis of a fast-growing lipoma to highlight the importance of histopathological diagnosis. A 14-year-old boy presented 10x10 cm tumor in the right shoulder for a year with ultrasonography suggesting lipoma. The lesion was excised and EES was confirmed by immunohistochemical analysis. EES diagnosis in young people is challenging compared to subcutaneous tumors, and histopathology is essential. The rapid progression of the tumor and high metastatic rates highlight the significance of early treatment.

Prognostic Factors and Clinical Outcomes in Extraskeletal Ewing Sarcoma: A Cohort Study.

Extraskeletal Ewing sarcoma (ESE) is a lesser-known, rarer counterpart of Ewing sarcoma of bone. This single-center study sought to evaluate the prognosticators and outcomes following multimodality therapy in patients with ESE. Forty-seven patients with ESE, treated between 2013 and 2018 with a standardized protocol and multimodality therapy using established doxorubicin-based regimens, were followed-up to assess outcomes. Median age at diagnosis was 20 (range 7-56) years, and 57.4% were male. Median tumor size was 7 (range 2-21) cm. The symptom-duration ranged from 1 to 8 (median 4) months. Tumor-site was trunkal in 61.7%, extremity in 23.4%, and head and neck 14.9%. Of the 35 patients with nonmetastatic disease at presentation, 13 underwent upfront surgery. The rest received chemotherapy followed by local treatment, which was surgical in 15 and radiotherapy in 5. At median follow-up of 24 (range 5-98) months, 55.3% patients had experienced events, and 29.8% had diedof progressive disease. Three-year event-free survival was 41.1%, and overall survival was 53%. On univariate analysis, trunkal location, upfront surgery, and positive surgical margins were associated with inferior EFS. Trunkal tumors and upfront surgery were alsoassociated with poorer OS. On multivariate analysis, trunkal location and margin-positive resections retained statistical significance for adverse EFS. Unless clearly resectable upfront, ESE should be downstaged with chemotherapy before local treatment. A margin-negative resection should be the objective when performing surgery. Definitive radiotherapy is an alternative in tumors not amenable for complete excision or when anticipated postoperative morbidity precludes radical surgery.