Ewing‐like sarcoma: An emerging family of round cell sarcomas

Renzi, Samuele; Anderson, Nathaniel D.; Light, Nicholas; Gupta, Abha

doi:10.1002/jcp.27558

Cited by 69 publications

(61 citation statements)

References 65 publications

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“…ES is a kind of a class of diseases called small round cell malignant tumors. In such diseases, malignant tumor cells presented similar morphology, so it was hard to differentiate histology grade [20] [21]. In our study, we found that the most patients had missed grade data.…”

Section: Discussionmentioning

confidence: 66%

Risk factors for metastasis and poor prognosis of Ewing sarcoma: a population based study

Shi

Yang

et al. 2020

J Orthop Surg Res

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Background: This study is to determine the risk factors for metastasis of Ewing sarcoma (ES) patients in SEER database. Then explore clinicopathological factors associated with poor prognosis. Furthermore, develop the nomogram to predict the probability of overall survival and cancer-specific survival Methods: Thus, we collected clinicopathological data of ES patients in SEER database, and then used chi-square test and logistic regression to determine risk factors associated to metastasis. We also did survival analysis including Kaplan-Meier curve and Cox proportional hazard model to explore the risk factors associated to overall survival and cancer-specific survival, and then developed the nomogram to visualize and quantify the probability of survival. Results: After statistics, we find that patients with older ages (11-20 years old: OR = 1.517, 95% confidence interval [CI] 1.033-2.228, p = 0.034; 21-30 years old: OR = 1.659. 95%CI 1.054-2.610, p = 0.029), larger tumor size (> 8 cm: OR = 1.914, 95%CI 1.251-2.928, p = 0.003), and pelvic lesions (OR = 2.492, 95%CI 1.829-3.395, p < 0.001) had a higher risk of metastasis. ROC curves showed higher AUC (0.65) of combined model which incorporate these three factors to predict the presence of metastasis at diagnosis. In survival analysis, patients with older ages (11-20 years:

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Section: Discussionmentioning

confidence: 66%

Risk factors for metastasis and poor prognosis of Ewing sarcoma: a population based study

Shi

Yang

et al. 2020

J Orthop Surg Res

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“…CIC rearranged sarcomas are rare but represent the most frequent molecular subtype among Ewing-like undifferentiated SBRCTs [1,22], accounting for approximately 70% of SBRCTs lacking EWSR and FUS rearrangements [2,16,[23][24][25]. CIC rearranged tumors are characterized by fusions involving CIC, a human homolog of Drosophila Capicua located on chromosome 19q13.2 and encoding a transcriptional repressor, functioning downstream of tyrosine kinase receptor signaling.…”

Section: Discussionmentioning

confidence: 99%

“…Both DUX4 and DUX4L genes contain a 3.3-kb tandem repeat sequence located in the sub-telomeric regions of the long arms of chromosomes 4 and 10, respectively [28]. They encode for double-homeobox transcription factor typically expressed in germ cells as well as in the human testis, but epigenetically silenced by methylation in differentiated cells [1,[29][30][31]. No significant clinical-pathologic differences have been reported between tumors harboring the t(4;19) versus the t(10;19) [31].…”

Section: Discussionmentioning

confidence: 99%

“…Primitive small blue round cell tumors (SBRCTs) in children and young adults pose a diagnostic challenge. Ewing sarcoma (ES) is the prototype for an undifferentiated sarcoma with such a phenotype, although it is shared by several other sarcoma entities together with the membrane expression of CD99 [1,2]. An advance in the diagnosis of Ewing's sarcoma was the discovery…”

Section: Introductionmentioning

confidence: 99%

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DNA Methylation Profiling for Diagnosing Undifferentiated Sarcoma with Capicua Transcriptional Receptor (CIC) Alterations

Miele

Vito

Ciolfi

et al. 2020

IJMS

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Undifferentiated soft tissue sarcomas are a group of diagnostically challenging tumors in the pediatric population. Molecular techniques are instrumental for the categorization and differential diagnosis of these tumors. A subgroup of recently identified soft tissue sarcomas with undifferentiated round cell morphology was characterized by Capicua transcriptional receptor (CIC) rearrangements. Recently, an array-based DNA methylation analysis of undifferentiated tumors with small blue round cell histology was shown to provide a highly robust and reproducible approach for precisely classifying this diagnostically challenging group of tumors. We describe the case of an undifferentiated sarcoma of the abdominal wall in a 12-year-old girl. The patient presented with a voluminous mass of the abdominal wall, and multiple micro-nodules in the right lung. The tumor was unclassifiable with current immunohistochemical and molecular approaches. However, DNA methylation profiling allowed us to classify this neoplasia as small blue round cell tumor with CIC alterations. The patient was treated with neoadjuvant chemotherapy followed by complete surgical resection and adjuvant chemotherapy. After 22 months, the patient is disease-free and in good clinical condition. To put our experience in context, we conducted a literature review, analyzing current knowledge and state-of-the-art diagnosis, prognosis, and clinical management of CIC rearranged sarcomas. Our findings further support the use of DNA methylation profiling as an important tool to improve diagnosis of non-Ewing small round cell tumors.

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“…The tumor suppressor gene EGR-1 can directly mediate the apoptotic function through the transcriptional upregulation of Bax-mRNA and protein and the increase of oligomerization and activation [32]. FEV is rich in alanine c-terminal, indicating that it may act as a transcriptional inhibitor [33]. FOS is considered as a regulator of cell proliferation, differentiation, and transformation and participates in MAPK signaling pathway [34,35].…”

Section: Discussionmentioning

confidence: 99%

A Risk Score Model Associated with Tumor Microenvironment Predicts Disease-Free Survival After Radical Prostatectomy

Zhao¹,

Zhang²,

Shi³

et al. 2020

Preprint

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Background Tumor microenvironment (TME) and immune checkpoint inhibitors has been shown to promote active immune responses through different mechanisms. We aimed to identify the important prognostic genes and prognostic characteristics related to TME in prostate cancer (PCa).Methods The gene transcriptome profiles and clinical information of PCa patients were obtained from the TCGA database, and the immune, stromal and estimate scores were calculated by the ESTIMATE algorithm. We evaluated the prognostic value of risk score (RS) model based on univariate Cox and LASSO Cox regression models analysis, and established a nomogram to predict disease-free survival (DFS) in PCa patients. The GSE70768 data set was used for external validation. Finally, 22 subsets of tumor-infiltrating immune cells (Tiics) were analyzed using the Cibersort algorithm.Results In this study, the patients with higher immune, stromal, and estimate scores were associated with poorer DFS, higher Gleason score, and higher AJCC T stage. Based on the immune and stromal scores, the Venny diagram screened out 515 cross DEGs. The univariate COX and Lasso Cox regression models were used to select 18 DEGs from 515 DEGs, and constructed a RS model. The DFS of the high-RS group was significantly lower than that of the low-RS group (P<0.001). The AUC of 1-year, 3-year and 5-year DFS rates in RS model were 0.778, 0.754 and 0.750, respectively. In addition, the RS model constructed from 18 genes was found to be more sensitive than Gleason score (1, 3, 5 year AUC= 0.704, 0.677 and 0.682). The nomograms of DFS were established based on RS and Gleason scores. The AUC of the nomograms in the first, third, and fifth years were 0.802, 0.808, and 0.796, respectively. These results have been further validated in GEO. In addition, the proportion of Tregs was higher in high-RS patients (P<0.05), and the expression of five immune checkpoints (CTLA-4, PD-1, LAG-3, TIM-3 and TIGIT) was higher in high-RS patients (P<0.05).Conclusion We identified 18 TME-related genes from the TCGA database, which were significantly related to DFS in PCa patients.

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Ewing‐like sarcoma: An emerging family of round cell sarcomas

Cited by 69 publications

References 65 publications

Risk factors for metastasis and poor prognosis of Ewing sarcoma: a population based study

Risk factors for metastasis and poor prognosis of Ewing sarcoma: a population based study

DNA Methylation Profiling for Diagnosing Undifferentiated Sarcoma with Capicua Transcriptional Receptor (CIC) Alterations

A Risk Score Model Associated with Tumor Microenvironment Predicts Disease-Free Survival After Radical Prostatectomy

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