Primary Ewing sarcoma of the kidney mimicking cystic papillary renal cell carcinoma in an older patient:A case repor

BACKGROUND Ewing’s sarcoma(ES)is a neuroectodermal tumor that typically occurs in the bones and soft tissues of children and young adults.Primary renal ES is rare;only a few cases and a small case series have been documented,and only four cases involved primary renal ES in older people(>65 years old).CASE SUMMARY Herein,we describe the radiological and pathological features of primary renal ES in an older person.A 76-year-old man complained of poor oral intake and was found to have a large cystic renal mass with indistinct margins on computed tomography.Ultrasound-guided biopsy revealed that the tumor contained small round blue cells.The patient underwent a right radical nephrectomy.The tumor cells showed diffuse membranous CD99,and nuclear friend leukemia integration 1 transcription factor and NK2 Homeobox 2.Fluorescence in situ hybridization revealed EWSR1 translocation.Postoperatively,18F-fluorodeoxyglucose positron emission tomography revealed no evidence of metastasis.The patient was diagnosed with primary renal ES.Six months following the surgery,local recurrence and distant metastasis were observed.Primary renal ES is rare and often lethal in older individuals.The specific imaging findings are unknown,and treatment protocols have not been standardized.CONCLUSION This case report describes the radiological and pathological features of primary renal ES in an older person.

Bioinformatics Analysis of the Biological Properties of Ewing Sarcoma

Purpose: Bioinformatics-based approach to screen and analyze differentially expressed genes associated with the biological characteristics of Ewing sarcoma. Means: The GSE17674 dataset was selected for analysis, obtained by data retrieval based on the GEO public database. The R language limma toolkit was used to screen DEmRNAs. After the data were normalized, the Metascape online analysis software and the R language clusterProfiler package were used to analyze the GO function and KEGG pathway enrichment of DEmRNAs lines, respectively. The string database was selected for PPI analysis, and the results were imported into Cytoscape software to derive the core modules and predicted core genes. The genes selected above were analyzed for tissue localization specificity. Results: Through the analysis of GSE17674, differentially expressed genes were screened out, and GO and KEGG analyses were performed on the differentially expressed genes. The GO functional enrichment analysis was mainly enriched in the process of muscle system, muscle contraction, myocyte development, contractile fibers, myogenic fibers, myofibers, myofibrillar segments, actin binding, structural composition of muscle, and actin filament binding. KEGG pathway analysis showed that the core pathways associated with the development of ES were the core genes for myocardial contraction, congestive cardiomyopathy, and hypertrophic cardiomyopathy. Five Hub genes were obtained based on Cytoscape prediction. Tissue localization specificity analysis of Hub genes was performed, and a total of 2 Hub genes with tissue specificity were screened;MYH6 was specifically expressed in cardiac cells and MYL1 was specifically expressed in skeletal muscle cells. Conclusions: The differential genes screened will help to understand the molecular mechanisms underlying the highly invasive and metastasis-prone biological characteristics of ES, as well as provide new ideas for clinical drug-targeted treatment of ES.

Primary adrenal Ewing sarcoma:A systematic review of the literature

BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.

An Analysis of Conditional Survival Rates for Ewing Sarcoma Patients

Using data from the Surveillance, Epidemiology, and End Results (SEER) Program based at the National Cancer Institute in the US, conditional survival rates are reported for 1,988 Ewing Sarcoma patients diagnosed during the period 2000-2015. These patients represent the experience of 26.5% of the US population. Specifically, 5-year conditional relative survival rates are calculated for these patients for the first eight years subsequent to diagnosis of their cancer by Extent of Disease (EOD) (Localized, Regional, and Distant as coded by the SEER Program), gender, and age (<18, 18 - 34, and 35+). Findings include showing how the conditional survival rate patterns improve over time and that there are differences by gender, age, and EOD.

Osteosarcoma of the Humerus Developing as Second Malignancy in the Irradiation Field Outside the Primary Tumor: 11 Years after Ewing Sarcoma of the Scapula and 29 Years after Breast Cancer

Purpose: Development of sarcoma is a known late rare negative side effect of radiotherapy. We add two cases to emphasize the need for open-end follow-up and critical evaluation to avoid misinterpretation. Patients, Methods, and Results: Two patients developed osteosarcoma as a second malignancy in the humerus after adjuvant radiotherapy of a primary tumor not directly involving the later affected bone. The first patient had a Ewing sarcoma of the scapula at age 13 years. Though after neoadjuvant chemotherapy the resected specimen showed only fibrotic necrotic areas within clear resection margins, the study group indicated adjuvant radiotherapy in a field including the shoulder joint. At age 24 years she developed an osteosarcoma of the humeral head, which was resected and reconstructed with a proximal humerus endoprosthesis. She is alive without disease at age 32 years. The second patient presented with an osteosarcoma of the proximal humerus 29 years after irradiation for breast cancer including the shoulder joint. The sarcoma was misinterpreted as radiation-induced necrosis and the patient was treated with a reverse shoulder endoprosthesis. Pathologic examination of the resected humeral head then showed a typical osteosarcoma. Two years later the humeral reverse shoulder implant was resected and a proximal humerus tumor prosthesis implanted leaving the original glenosphere. Conclusions: In both cases radiation-induced osteosarcoma developed in bone not affected by the primary cancer. Protecting uninvolved structures must be warranted in the planning of radiotherapy. The long latency between the primary and second cancer mandates long-term—best indefinite—follow-up, as with appropriate treatment of a radiation-induced osteosarcoma good healing rates comparable to those of primary osteosarcoma can still be achieved.

Extraskeletal Ewing sarcoma of the stomach:A rare case report

BACKGROUND Extraskeletal Ewing sarcoma(EES)is a rare and highly malignant small round cell tumor associated with a poor clinical outcome.Ewing sarcoma(ES)involving the stomach is an uncommon presentation and can be easily confused with other small round cell tumors.We herein present a rare case of ES involving the gastric area.CASE SUMMARY We report a case of gastric ES in a 19-year-old female patient who initially presented with a complaint of a tender epigastric mass for 5 d.Contrast-enhanced abdominal computed tomography revealed a soft-tissue-density mass with a diameter of 8.5 cm between the liver and stomach;the mass was connected to the gastric antrum.Then,the mass was surgically excised completely.Upon histopathological,immunophenotype and molecular analysis,the mass was identified to be a primary gastric ES.CONCLUSION EES is an aggressive tumor with poor prognosis.Therefore,early diagnosis and timely intervention are essential for a good prognosis.It is imperative for us to raise awareness about this rare tumor.Surgical resection is still the best treatment option.

Ewing sarcoma of the jejunum: A case report and literature review

BACKGROUND Ewing sarcomas(ESs)are highly aggressive malignancy and are predominant in the long bones of extremities of children and young adults with a slight male predilection and rarely presents at extra skeletal locations.CASE SUMMARY A 55-year-old woman came to our hospital after finding elevated tumor biomarkers during her physical examination.Her enhanced computed tomography scan showed a jejunal mass.The patient underwent laparoscopic enterectomy.The mass was later diagnosed as ES,evidenced by fluorescence in situ hybridization whereby the GLP ES breakpoint region 1 probe was used,showing that more than 10%of the cells showed a red-green-yellow signal proving the breakpoint rearrangement of the ES breakpoint region 1 gene in chromosome 22.CONCLUSION We describe a case of localized ES at the jejunum in China based on the literature.

Ewing sarcoma of the ileum with wide multiorgan metastases:A case report and review of literature

BACKGROUND Ewing sarcoma(ES)is an aggressive small round cell tumor that usually occurs in younger children and young adults but rarely in older patients.Its occurrence in elderly individuals is rare.ES of the ileum with wide multiorgan metastases is rarely reported and difficult to distinguish radiologically from other gastrointestinal tract tumors.CASE SUMMARY A 53-year-old man presented with right lower quadrant pain for 2 wk.Computed tomography results showed a heterogeneous mass within the ileum and widespread multiorgan metastases.This mass was biopsied,and pathological examination of the resected specimen revealed features consistent with an extraskeletal ES.CONCLUSION This case emphasizes the importance of recognizing this rare presentation in the small intestine to broaden the differential diagnosis of adult intraabdominal tumors.

Imaging-based diagnosis for extraskeletal Ewing sarcoma in pediatrics: A case report

BACKGROUND Extraskeletal Ewing sarcoma(EES)is a member of the Ewing sarcoma family of tumors which is pathologically known as a small,round,blue cell tumor involving bone and soft tissue.The prevalence of EES is only 15%-25%of all Ewing sarcoma and EES often occurs in patients aged from 20-mo-old to 30-yearsold resulting in an unfavorable prognosis.CASE SUMMARY The present case report described a 7-year-old patient with a palpable EES mass of 33 mm×27 mm×28 mm in the deep neck with symptoms of persistent dyspnea over the past 5 mo.After laboratory examinations,abnormal physiological and biochemical indicators were not found.Ultrasound images presented the mass to be complex,solid and fluid-filled with circumscribed margins and posterior acoustic enhancement.The mass also presented with partial internal vascularity.The contrast-enhanced magnetic resonance imaging scan illustrated the outstanding enhancement with fast perfusion mode in the early arterial phase. CONCLUSIONOur study suggested that a quick-growing mass in the pediatric patient is possibly a malignanttumor whether the mass has well-defined margins or not.

Ewing sarcoma/primitive neuroectodermal tumor of the ureter:A case report and literature review

Ewing sarcoma/primary neuroectodermal tumors are rare,invasive,and small round blue cell tumors.There are few reports of its occurrence in the urinary system.Here,we present the first middle-aged female patient whose Ewing sarcoma primary site was in the ureter.The main clinical manifestation was intermittent hematuria.She was in good health after complete surgical resection and adjuvant radiotherapy.To date,there has been no recurrence or metastasis.Accurate early diagnosis and appropriate treatment can help prolong survival.18F-fluorodeoxyglucose positron emission tomography/computed tomography is expected to be an effective means of evaluating treatment effects and detecting metastasis and recurrence.In this article,besides introducing a case of Ewing sarcoma/primitive neuroectodermal tumor of the ureter,we review the literature to discuss the current status of diagnosis and treatment.

Pleural Effusion Revealing Bone Ewing Sarcoma: About Three Cases

Costal Ewing sarcoma is a rare primary malignant bone tumor with delayed diagnosis and complex treatment. The diagnosis, evoked in front of swelling, a parietal pain, supported on the tomodensitometry, rests on the anatomopathological examination. The treatment is based on the combination of chemotherapy surrounding complete surgical removal of the tumor. Radiotherapy should remain reserved for a few specific indications because of its complications. We report the case of three children who presented with a pleural effusion revealing Ewing’s sarcoma affecting the ribs.

Unlocking epigenetics for precision treatment of Ewing’s sarcoma

Ewing’s sarcoma(EWS)is a highly aggressive malignant bone tumor primarily affecting adolescents and young adults.Despite the efficacy of chemoradiotherapy in some cases,the cure rate for patients with metastatic and recurrent disease remains low.Therefore,there is an urgent need for innovative therapeutic approaches to address the challenges associated with EWS treatment.Epigenetic regulation,a crucial factor in physiological processes,plays a significant role in controlling cell proliferation,maintaining gene integrity,and regulating transcription.Recent studies highlight the importance of abnormal epigenetic regulation in the initiation and progression of EWS.A comprehensive understanding of the intricate interactions between EWS and aberrant epigenetic regulation is essential for advancing clinical drug development.This review aims to provide a comprehensive overview of both epigenetic targets implicated in EWS,integrating various therapeutic modalities to offer innovative perspectives for the clinical diagnosis and treatment of EWS.

Prostatic sarcoma of the Ewing family in a 33-year-old male e A case report and review of the literature

Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents,typically presenting between 10 and 20 years of age.Extraosseous sarcomas of the Ewing family in adults are rare.We report a manifestation of this tumor entity in the periprostatic tissue of a 33-year-old male and discuss our treatment approach.Transrectal biopsy is a feasible and simple diagnostic tool for unclear pelvic masses.Multi-modal therapy and central registries are needed to gain knowledge of rare pelvic tumors like Ewing sarcoma.

Perigastric extraskeletal Ewing's sarcoma: A case report

Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specifi c enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.

CT and multimodal imaging findings of primary orbital Ewing＇s sarcoma involving the middle cranial fossa： a case report

This report details the CT and MR imaging findings of a primary orbital Ewing＇s sarcoma case involving the middle cranial fossa in a 13-year old boy. CT showed an ill-circumscribed homogeneous soft-tissue density mass with needle-like bone reaction. On MRI, the mass showed homogeneous iso-intensity on T1WI, heterogeneous hyper- intensity on T2WI, and marked homogeneous enhancement. Meanwhile, the mass was hyper-intense on DWI, and ADC alue was 575x10-6 mm2/s. The mass showed as high rCBV and rCBF, prolonged MTT based on DCS- PWI, and wash-out pattern of TIC derived from the DCE-MRI. Our case suggests that functional MRI modalities, including DWI, DSC-PWI or DCE-MR, could provide additional information for differential diagnoses. Both CT and MRI should be performed and comprehensively analyzed for limiting differential diagnoses, determining lesion extension and facilitating operative approach.

Resection with limb salvage in an Asian male adolescent withEwing’s sarcoma: A case report

BACKGROUNDEwing’s sarcoma is a highly malignant primary bone tumor that commonly affectschildren. For young patients, multidisciplinary treatment and limb salvage arerecommended, and surgical plans considering the growth potential and boneactivity after tumor resection are essential.CASE SUMMARYAn 11-year-old Asian boy had a 1-mo history of a right-sided limping gait.Imaging revealed a proximal tumor with bone destruction and physealinvolvement over the right femoral neck. He was diagnosed with stage IV(T1N0M1aG3) Ewing’s sarcoma with bilateral lung metastases. Neoadjuvantchemotherapy decreased the tumor size and confined it to the metaphysealregion. The patient underwent four stages of surgery: wide tumor excision plusreconstruction with vascular fibular bone graft plus internal fixation;repeat openreduction and internal fixation;femoral lengthening with orthosis after physealmaturity;and orthosis removal and bone elongation (approximately 6 cm).Following surgery, he could walk without discomfort and had almost equal-sizedbilateral femoral heads, indicating physis preservation. The surgery wassuccessful, and normal femoral head growth was achieved after completeremission. The patient was able to resume normal activities with equal length ofthe bilateral lower limbs.CONCLUSIONTumor treatment and reconstruction following resection are important in skeletally immature patients with Ewing’s sarcoma to improve quality of life.

Anesthesia and perioperative management for giant adrenal Ewing’s sarcoma with inferior vena cava and right atrium tumor thrombus:A case report

BACKGROUND Ewing’s sarcoma of the adrenal gland with inferior vena cava(IVC)and right atrium thrombus is extremely rare.Here,we report a case of giant adrenal Ewing’s sarcoma with IVC and right atrium tumor thrombus and summarize the anesthesia and perioperative management.CASE SUMMARY A young female was admitted to the Department of Urology with intermittent pain under the right costal arch for four months.Enhanced abdominal computed tomography revealed a large retroperitoneal mass(22 cm in diameter),which may have originated from the right adrenal gland and was closely related to the liver.Transthoracic echocardiography showed a strong echogenic filling measuring 70 mm extended from the IVC into the right atrium and ventricle.After preoperative preparation with cardiopulmonary bypass,sufficient blood products,transesophageal echocardiography and multiple monitoring,tumor and thrombus resection by IVC exploration and right atriotomy were successfully performed by a multidisciplinary team.Intraoperative hemodynamic stability was the major concern of anesthesiologists and the status of tumor thrombus and pulmonary embolism were monitored continuously.During transfer of the patient to the intensive care unit(ICU),cardiac arrest occurred without external stimulus.Cardiopulmonary resuscitation was performed immediately and cardiac function was restored after 1 min.In the ICU,extracorporeal membrane oxygenation(ECMO)and continuous renal replacement therapy(CRRT)were provided to maintain cardiac,liver and kidney function.Histopathologic examination confirmed the diagnosis of Ewing’s sarcoma.After postoperative treatments and rehabilitation,the patient was discharged from the urology ward.CONCLUSION An adrenal Ewing’s sarcoma with IVC and right atrium thrombus is extremely rare,and its anesthesia and perioperative management have not been reported.Thus,this report provides significant insights in the perioperative management of patients with adrenal Ewing’s sarcoma and IVC tumor thrombus.Intraoperative circulation fluctuations and sudden cardiovascular events are the major challenges during surgery.In addition,postoperative treatments including ECMO and CRRT provide essential support in critically ill patients.Moreover,this case report also highlights the importance of multidisciplinary cooperation during treatment of the disease.

Primary extraskeletal Ewing’s sarcoma of the lumbar nerve root:A case report

BACKGROUND Ewing’s sarcoma(ES)is a highly aggressive bone malignancy.Extraskeletal ES(EES)originating in the spinal canal is extremely rare.Herein,we report on a rare case of EES with a primary lumbar spinal nerve root including the complete diagnosis and treatment.CASE SUMMARY A young female patient presented with a complaint of right lower limb pain for 1 mo.Magnetic resonance imaging(MRI)revealed an 11 mm×14 mm×31 mm mass in the lumbar epidural region extending at the fifth lumbar spine(L5)level toward the right L5 neural foramen.Our initial diagnosis was an epidural schwannoma.The patient underwent total laminectomy,tumor resection and pedicle screw internal fixation and the L5 root tumor was found to have been completely removed intraoperatively.Histopathological examination of the lesion showed a typical ES with a large number of small,round cells.Immunohistochemistry analysis indicated positive CD99 and S100.After surgery,the patient received chemotherapy and radiotherapy with a 1 year of follow-up and no recurrent tumors or new lesions were found upon spine MRI and positron emission tomography/computed tomography reexamination.CONCLUSION Clinically,ES outside the bone should be considered when nerve root tumors are encountered inside and outside the spinal canal and the diagnosis should be determined by pathological biopsy.After surgical resection,chemotherapy and radiotherapy should be performed.After treatment,active follow-up and regular review should be completed.

Primary extraosseous Ewing's sarcoma of the lung in a 36-year-old female patient:clinical,imaging,and pathologic findings

Primary extraosseous Ewing's sarcoma is a very rare clinical entity. In the report, we present the case of a 36year-old female patient who underwent resection of lung mass and was diagnosed as having Ewing's sarcoma by pathological examination. The clinical, imaging, and pathologic features of the case are described and discussed.

Ewings Sarcoma-Primitive Neuroectodermal Tumour-Rare Extraosseous Presentation

Introduction: Ewings sarcoma is uncommon in extra osseous locations. They belong to Ewings family of tumours and the primitive neuroectodermal tumour is often diagnosed based on immuno histochemistry and molecular genetic studies. Discussion: A 50-year-old gentleman presented with complaints of a swelling in the right thigh. With a diagnosis of soft tissue sarcoma, the patient underwent a wide local excision of the tumour. We review literature of the small round blue cell tumour. Conclusion: Extraosseous Ewings sarcoma presenting as a thigh swelling is uncommon. The sarcoma was vimentin positive and CD99 membrane positivity.