Clinical and Molecular Differentiation Between Malignant Rhabdoid Tumor of the Kidney and Normal Tissue: A Two-Case Report

Zhanghuang, Chenghao; Chen, Shuo; Li, Li; Yang, Zhen; Xie, Yucheng; Li, Jiwei; Tang, Haoyu; He, Xiaoli; Dong, Liuyi; Yan, Bing

doi:10.3389/fonc.2021.659709

Cited by 7 publications

(7 citation statements)

References 26 publications

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“…In conclusion, married patients showed significant improvement in RR compared with unmarried patients for cancer with a relatively low degree of malignancy, such as patients with ccRCC included in this study. Marriage is not beneficial for highly malignant cancers, such as pancreatic cancer and renal malignant rhabdomyoma tumor ( 31 , 32 ).…”

Section: Discussionmentioning

confidence: 99%

A Web-Based Prediction Model for Cancer-Specific Survival of Elderly Patients With Clear Cell Renal Cell Carcinoma: A Population-Based Study

Zhanghuang

Wang

Zhang

et al. 2022

Front. Public Health

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BackgroundClear cell renal cell carcinoma (ccRCC) is expected in the elderly and poor prognosis. We aim to explore prognostic factors of ccRCC in the elderly and construct a nomogram to predict cancer-specific survival (CSS) in elderly patients with ccRCC.MethodsClinicopathological information for all elderly patients with ccRCC from 2004 to 2018 was downloaded from the Surveillance, Epidemiology, and End Results (SEER) program. All patients were randomly assigned to a training cohort (70%) or a validation cohort (30%). Univariate and multivariate Cox regression models were used to identify the independent risk factors for CSS. A new nomogram was constructed to predict CSS at 1-, 3-, and 5 years in elderly patients with ccRCC based on independent risk factors. Subsequently, we used the consistency index (C-index), calibration curves, and the area under the receiver operating curve (AUC) and decision curve analysis (DCA) to test the prediction accuracy of the model.ResultsA total of 33,509 elderly patients with ccRCC were enrolled. Univariate and multivariate Cox regression analyses results showed that age, sex, race, marriage, tumor size, histological grade, tumor, nodes, and metastases (TNM) stage, and surgery were independent risk factors for CSS in elderly patients with ccRCC. We constructed a nomogram to predict CSS in elderly patients with ccRCC. The C-index of the training cohort and validation cohort was 0.81 (95% CI: 0.802–0.818) and 0.818 (95% CI: 0.806–0.830), respectively. The AUC of the training cohort and validation cohort also suggested that the prediction model had good accuracy. The calibration curve showed that the observed value of the prediction model was highly consistent with the predicted value. DCA showed good clinical application value of the nomogram.ConclusionIn this study, we explored prognostic factors in elderly patients with ccRCC. We found that age, sex, marriage, TNM stage, surgery, and tumor size were independent risk factors for CSS. We constructed a new nomogram to predict CSS in elderly patients with ccRCC with good accuracy and reliability, providing clinical guidance for patients and physicians.

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Section: Discussionmentioning

confidence: 99%

A Web-Based Prediction Model for Cancer-Specific Survival of Elderly Patients With Clear Cell Renal Cell Carcinoma: A Population-Based Study

Zhanghuang

Wang

Zhang

et al. 2022

Front. Public Health

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“…According to the German Children's Cancer Registry, the absolute number of reported cases of rhabdoid tumors between 2010 and 2015 was 139, with male prevalence in all cases, in which only 9% of the MRTK were mentioned [ 3 ]. It occurs more frequently in children aged 2-year-old or older and is low in the neonatal form [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…The tumor has an overall poor prognosis. Recent research found that only one patient out of 35 survived after a five-year follow-up [ 4 ]. The prognosis depends on several parameters, including age, comorbidities, metastases, early detection, and treatment.…”

Section: Discussionmentioning

confidence: 99%

“…The most frequently reported symptoms are abdominal masses and hematuria which can often be detected in the metastatic stage. The definitive diagnosis is made by detecting the SWItch/sucrose nonfermentable (SWI / SNF) mutation-related matrix-associated actin-dependent regulator of chromatin subfamily B1 (SMARCB1) gene or by negative immunohistochemistry integrase interactor 1 (INI-1) [ 4 ]. Surgery, postoperative radiotherapy, and chemotherapy are the main approaches to treating patients with MRIK.…”

Section: Introductionmentioning

confidence: 99%

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Malignant Rhabdoid Tumor of the Kidney in a Child With Xeroderma Pigmentosum: Incidence or Coincidence?

Lahmar¹,

Ayad²,

Ramdani³

et al. 2022

Cureus

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Malignant rhabdoid tumor of the kidney (MRTK) is a rare aggressive malignant rhabdoid tumor that mainly affects children. At the onset of the disease, the usual clinical manifestations are gross hematuria, abdominal pain, and abdominal distension. The prognosis remains poor. Patients with rhabdoid tumors (RT) are treated according to institutional preferences that combine surgery, radiation therapy, and chemotherapy. The authors present the rare case of a child with xeroderma pigmentosum (XP) who presented with an abdominal mass accompanied by hematuria and abdominal pain. The radiological and histological results were congruent with the MRTK. The patient received preoperative chemotherapy but unfortunately died of septic shock. This case highlights the importance of being aware of MRTK and its fatal complications, as well as the increased risk of kidney tumors in patients with XP.

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“…Our previous studies have shown that the PI3K-AKT signaling pathway and microRNA-related proteins have a very high potential value for the diagnosis and treatment of MRTK. ( Zhanghuang et al, 2021 ). However, the prognosis prediction of MRTK has not been fully clarified.…”

Section: Introductionmentioning

confidence: 99%

Identification of Prognostic Biomarkers in Patients With Malignant Rhabdoid Tumor of the Kidney Based on mTORC1 Signaling Pathway-Related Genes

Zhanghuang

Yao

Tang

et al. 2022

Front. Mol. Biosci.

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Background: Malignant rhabdoid tumor of the kidney (MRTK) is an infrequent malignant tumor in childhood, accounting for approximately 2% of all childhood kidney tumors. Although the development of current treatments, the overall survival (OS) rate of MRTK patients is only 25%. The aim of this research was to explore the prognostic value of genes associated with the mTORC1 signaling pathway in MRTK.Methods: The transcriptome data of MRTK samples were downloaded from the TARGET database. The 200 genes of HALLMARK_MTORC1_SIGNALING were downloaded from the Molecular Signatures Database (MSigDB). Furthermore, we applied gene set variation analysis (GSVA) to screen differentially expressed gene sets between the MRTK and normal samples. The 200 genes were combined with differentially expressed genes (DEGs) identified from differentially expressed gene sets. Then, a gene signature of mTORC1 pathway-related genes (mTRGs) was constructed in MRTK. The molecular mechanism of prognostic factors in MRTK was further analyzed using gene set enrichment analysis (GSEA). The target drugs based on these prognostic factors were explored from The Comparative Toxicogenomics Database (CTD). Moreover, six paired fresh tumor tissues and paraneoplastic tissues from children with MRTK were collected to validate the expressions of P4HA1, MLLT11, AURKA, and GOT1 in clinical samples via real-time fluorescence quantitative PCR and Western blot.Results: A four-gene signature (P4HA1, MLLT11, AURKA, and GOT1) related to the mTORC1 pathway was developed in MRTK, which divided the MRTK patients into high-risk and low-risk groups. The patients with high-risk scores were strongly associated with reduced OS. Receiver operating characteristic (ROC) analysis indicated a good prediction performance of the four biomarker signatures. GSEA revealed that the mTOR signaling pathway was significantly enriched. The risk score was demonstrated to be an independent predictor for MRTK outcome. According to the correlation of tumor stem cell index and prognostic factors, the target drugs were obtained for the treatment of MRTK patients. Furthermore, the expressions of RT-qPCR and Western blot were consistent with RNA-sequencing data such that their expressions were significantly elevated in tumor tissues.Conclusion: A total of four genes (P4HA1, MLLT11, AURKA, and GOT1) were screened as prognostic markers, further providing a new understanding for the treatment of patients with MRTK.

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Clinical and Molecular Differentiation Between Malignant Rhabdoid Tumor of the Kidney and Normal Tissue: A Two-Case Report

Cited by 7 publications

References 26 publications

A Web-Based Prediction Model for Cancer-Specific Survival of Elderly Patients With Clear Cell Renal Cell Carcinoma: A Population-Based Study

A Web-Based Prediction Model for Cancer-Specific Survival of Elderly Patients With Clear Cell Renal Cell Carcinoma: A Population-Based Study

Malignant Rhabdoid Tumor of the Kidney in a Child With Xeroderma Pigmentosum: Incidence or Coincidence?

Identification of Prognostic Biomarkers in Patients With Malignant Rhabdoid Tumor of the Kidney Based on mTORC1 Signaling Pathway-Related Genes

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