Ewing Sarcoma

Domansk, Henryk A; Walther, Charles S

doi:10.1159/000475108

Cited by 3 publications

(9 citation statements)

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“…It more frequently affects male patients [5]. Approximately, 80% of patients are diagnosed before 20 years of age, with the peak incidence occurring in the second decade of life [6, 7]. Most cases arise in bones (mainly in the long bones, pelvis, and ribs), and about 12% are extraskeletal [5].…”

Section: Ewing Sarcomamentioning

confidence: 99%

“…The second one is composed of larger and paler cells with more abundant cytoplasm and nuclei with fine chromatin and single or multiple, small, indistinct nucleoli [6, 9, 13, 14]. In larger cells, cytoplasmic vacuoles with glycogen can be seen [6]. Some cases may show necrotic background, sometimes with calcifications.…”

Section: Ewing Sarcomamentioning

confidence: 99%

“…Usually, mitotic activity is easily noticed [9]. The atypical ES accounts for <10% of cases and is characterized by prominent nuclear pleomorphism (mainly pronounced anisokaryosis and prominent nucleoli) [6, 9, 12]. Double population of cells is less evident than in classic ES.…”

Section: Ewing Sarcomamentioning

confidence: 99%

“…The first population consists of smaller and darker cells with hyperchromatic nuclei and scant cytoplasm. The second one is composed of larger and paler cells with more abundant cytoplasm and nuclei with fine chromatin and single or multiple, small, indistinct nucleoli [6, 9, 13, 14]. In larger cells, cytoplasmic vacuoles with glycogen can be seen [6].…”

Section: Ewing Sarcomamentioning

confidence: 99%

“…Cytologic features of ES are well-known and thoroughly described in the literature [6,[9][10][11][12][13]. Smears are usually hypercellular.…”

Section: Ewing Sarcomamentioning

confidence: 99%

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Cytology of Undifferentiated Round-Cell Sarcomas of Bone and Soft Tissue: Ewing Sarcoma or Not Ewing Sarcoma, That Is the Question

Gajdzis

Klijanienko

2021

Acta Cytologica

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Background: Undifferentiated round-cell sarcomas (URCSs) of soft tissue and bone are a group of clinically heterogeneous tumors. Diagnosis of these malignancies is based mainly on recurrent genetic alterations. The most common and the best known representative of this group is Ewing sarcoma (ES) which is characterized by gene fusions including EWSR1 or FUS and ETS transcription factors family. Other newly described entities are CIC-rearranged sarcoma, sarcoma with BCOR genetic alterations, and round-cell sarcoma with EWSR1-non-ETS fusions. All these novel tumors are known as Ewing-like sarcomas. Summary: It is believed that morphologic features of ES and Ewing-like sarcomas vary only slightly or even that cytomorphology is not relevant. But differences are usually obvious, and some cytologic findings, such as spindle cells, connective tissue fragments, or myxoid stroma, are typical for Ewing-like sarcomas but not for ES. Each of these entities is also characterized by different immunoprofiles. The aim of this review was to summarize cytomorphologic and immunohistochemical features of URCS and compare them with other small round-cell tumors. Key Messages: Cytology can be successfully used in URCS diagnosis as a complementary tool for core-needle biopsy or even alone in selected cases, especially in recurrent and metastatic tumors. Knowing the morphologic and immunohistochemical differences between URCS is essential to provide appropriate ancillary studies and make a definitive diagnosis.

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Section: Ewing Sarcomamentioning

confidence: 99%

Section: Ewing Sarcomamentioning

confidence: 99%

Section: Ewing Sarcomamentioning

confidence: 99%

Section: Ewing Sarcomamentioning

confidence: 99%

“…Cytologic features of ES are well-known and thoroughly described in the literature [6,[9][10][11][12][13]. Smears are usually hypercellular.…”

Section: Ewing Sarcomamentioning

confidence: 99%

See 3 more Smart Citations

Cytology of Undifferentiated Round-Cell Sarcomas of Bone and Soft Tissue: Ewing Sarcoma or Not Ewing Sarcoma, That Is the Question

Gajdzis

Klijanienko

2021

Acta Cytologica

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The Small Round Cell Sarcomas Complexities and Desmoplastic Presentation

Domanski¹

2022

Acta Cytologica

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Background: Small round cell sarcomas (SRCSs) account for most solid malignancies in the pediatric age group and are a part of group of malignant tumors characterized by heterogenous clinical presentation and overlapping microscopic features of small, round, primitive cells. In addition to the recently established certain genetically defined subset of undifferentiated round cell sarcomas of soft tissue and bone, this group of sarcomas include desmoplastic small round cell tumor, poorly differentiated synovial sarcoma, alveolar rhabdomyosarcoma, mesenchymal chondrosarcoma, and small cell osteosarcoma. Although, those entities share clinical and cytomorphologic features and cannot be unequivocally classified based on clinical presentation and morphology alone. Most of SRCSs characterizes of particular patterns of protein expression or genetic changes and ancillary tests remain necessary to confirm or rule out a specific diagnosis. Subtle but occasionally distinctive cytologic features narrows the number of differential diagnoses and helps to select appropriate ancillary tests necessary for the final diagnosis. Thus, when adequate fine needle aspiration (FNA) biopsy specimen is combined with ancillary tests, a specific histologic diagnosis can be made in almost all cases. However, due to complex cytologic features of SRCS as well as various quality and diversity of FNA smears, there are cases in that cytologic features which do not entirely match the known diagnostic criteria. Summary: The aim of this review was to summarize cytomorphologic criteria and to present rare and divergent cytological features of SRCSs. Careful assessment of clinical presentation, cytological features, immunohistochemical patterns, and molecular alternations is necessary for an accurate diagnosis. Knowing of rare and divergent microscopic findings that does not fit with the known cytological criteria will help to avoid misdiagnosis. Key Messages: The role of FNA biopsies diagnosing soft tissue and bone tumors has been increasing because of the ability of ancillary tests to assist in the diagnosis of specific tumors. SRCSs may be diagnosed accurately in cytology specimens. Access to clinical and radiographic presentation, utility of ancillary tests, understanding complexity of cytological features, and awareness of the rare cytologic findings that differ from that of the established diagnostic criteria are essential to make correct diagnosis.

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A Visualized Dynamic Prediction Model for Lymphatic Metastasis in Ewing's Sarcoma for Smart Medical Services

et al. 2022

Front. Public Health

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BackgroundThis study aims to predict the lymphatic metastasis in Ewing's sarcoma (ES) patients by nomogram. The risk of lymphatic metastasis in patients with ES was predicted by the built model, which provided guidance for the clinical diagnosis and treatment planning.MethodsA total of 929 patients diagnosed with ES were enrolled from the year of 2010 to 2016 in the Surveillance, Epidemiology, and End Results (SEER) database. The nomogram was established to determine predictive factors of lymphatic metastasis according to univariate and multivariate logistic regression analysis. The validation of the model performed using multicenter data (n = 51). Receiver operating characteristics (ROC) curves and calibration plots were used to evaluate the prediction accuracy of the nomogram. Decision curve analysis (DCA) was implemented to illustrate the practicability of the nomogram clinical application. Based on the nomogram, we established a web calculator to visualize the risk of lymphatic metastases. We further plotted Kaplan-Meier overall survival (OS) curves to compare the survival time of patients with and without lymphatic metastasis.ResultsIn this study, the nomogram was established based on six significant factors (survival time, race, T stage, M stage, surgery, and lung metastasis), which were identified for lymphatic metastasis in ES patients. The model showed significant diagnostic accuracy with the value of the area under the curve (AUC) was 0.743 (95%CI: 0.714–0.771) for SEER internal validation and 0.763 (95%CI: 0.623–0.871) for multicenter data external validation. The calibration plot and DCA indicated that the model had vital clinical application value.ConclusionIn this study, we constructed and developed a nomogram with risk factors to predict lymphatic metastasis in ES patients and validated accuracy of itself. We found T stage (Tx OR = 2.540, 95%CI = 1.433–4.503, P < 0.01), M stage (M1, OR = 2.061, 95%CI = 1.189–3.573, P < 0.05) and survival time (OR = 0.982, 95%CI = 0.972–0.992, P < 0.001) were important independent factors for lymphatic metastasis in ES patients. Furthermore, survival time in patients with lymphatic metastasis or unclear situation (P < 0.0001) was significantly lower. It can help clinicians make better decisions to provide more accurate prognosis and treatment for ES patients.

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Ewing Sarcoma

Cited by 3 publications

References 0 publications

Cytology of Undifferentiated Round-Cell Sarcomas of Bone and Soft Tissue: Ewing Sarcoma or Not Ewing Sarcoma, That Is the Question

Cytology of Undifferentiated Round-Cell Sarcomas of Bone and Soft Tissue: Ewing Sarcoma or Not Ewing Sarcoma, That Is the Question

The Small Round Cell Sarcomas Complexities and Desmoplastic Presentation

A Visualized Dynamic Prediction Model for Lymphatic Metastasis in Ewing's Sarcoma for Smart Medical Services

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