Clinical group and modified TNM stage for rhabdomyosarcoma: A review from the Children's Oncology Group

Crane, Jacquelyn; Xue, Wei; Qumseya, Amira; Gao, Zhengya; Arndt, Carola A.S.; Donaldson, Sarah S.; Harrison, Douglas J.; Hawkins, Douglas S.; Linardic, Corinne M.; Mascarenhas, Leo; Meyer, William H.; Rodeberg, David A.; Rudzinski, Erin R.; Shulkin, Barry L.; Walterhouse, David; Venkatramani, Rajkumar; Weiss, Aaron R.

doi:10.1002/pbc.29644

Cited by 21 publications

(21 citation statements)

References 90 publications

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“…However, patients aged <13 years, who accounted for 64.8% of the overall cohort, were unevenly distributed, which may potentially lead to bias. As for the tumor sites, according to the historical Children's Oncology Group risk stratification, bladder, or prostate sites are considered unfavorable, presenting a relatively low survival rate, therefore, cannot be treated as low risk ( 19 – 21 ). Our multivariate analysis of survival showed that the survival rate of bladder or prostate tumors was 1.86 times lower compared to other favorable sites, which is in accordance with other studies.…”

Section: Discussionmentioning

confidence: 99%

A Web-Based Prognostic Model for Pediatric Genitourinary Rhabdomyosarcoma: Analysis of Population-Based Cohort With External Validation

Huang

Liu

et al. 2022

Front. Public Health

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BackgroundWe conduct an analysis of data from the Surveillance, Epidemiology, and End Results (SEER) database, intending to identify prognostic factors of pediatric genitourinary rhabdomyosarcoma (PGU–RMS). Prognostic nomogram and web-based calculator were developed for potential clinical use.MethodsData of PGU–RMS patients were extracted from the SEER database as training and internal validation cohort, patients diagnosed as PGU–RMS from 2001 to 2015 in Beijing Children's Hospital were collected as an external validation cohort. We used log-rank tests to seek risk factors on the overall survival (OS) in the overall SEER cohort, tumor site subgroups, radiation subgroups, and metastasis subgroups. The univariable and multivariate Cox regression analyses were applied to establish the prognosis model.ResultsA total of 372 PGU-RMS patients in SEER and 84 patients from our center were included. 1-, 3-, and 5-year OS of the overall SEER cohort were 95.8, 82.1, and 78.8%. Subgroup analysis indicated that tumors located in the prostate/bladder were associated with a worse prognosis than the paratesticular, female genital system, and other sites (P < 0.001). Tumors of the T1/T2 stage, without regional lymph node, involvement or metastasis, can benefit from radiotherapy (P < 0.05). For patients without metastasis, younger age, T1/T2 stage, and undergoing radiation were associated with better prognosis (P < 0.05). The prognosis nomogram was well-calibrated, the concordance index (C-index) for the OS prediction was 0.823, 0.803, and 0.768 in training, internal and external validation cohort, the area under the receiver operating characteristic curve for 3-, and 5-year OS were 0.84, 0.84 in the training cohort, 0.90, 0.84 in internal validation cohort and 0.75, 0.80 in the external validation cohort. Decision curve analysis showed good clinical utility. The predictive performance of the nomogram was higher than the Intergroup Rhabdomyosarcoma Study Group (IRSG) pretreatment stage system based on the comparison of overtime C-index, net reclassification index, and integrated discriminatory index (P < 0.001).ConclusionA comprehensive analysis of OS for PGU–RMS patients was conducted based on population cohort. The established prognosis nomogram has been fully validated and evaluated, exhibits better performance than the IRSG pretreatment stage system. Furthermore, a web-based risk calculator was developed to optimize clinical decisions.

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Section: Discussionmentioning

confidence: 99%

A Web-Based Prognostic Model for Pediatric Genitourinary Rhabdomyosarcoma: Analysis of Population-Based Cohort With External Validation

Huang

Liu

et al. 2022

Front. Public Health

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“…The orbit is designated a favorable primary site by the Intergroup Rhabdomyosarcoma Study Group (IRSG) modified tumor, node, metastasis (TNM) staging system. [1][2][3] The standard treatment approach to ORMS on IRSG and subsequent Children's Oncology Group (COG) studies has been biopsy followed by chemotherapy and radiotherapy, so that, at presentation, most patients have group III disease. 4 The highest failure-free survival (FFS) rate reported for patients with group III ORMS occurred on Intergroup Rhabdomyosarcoma Study IV (IRS-IV) (3-year FFS, 94%; overall survival [OS], 98%); however, intensive therapy was administered with 26.4 g/m 2 cyclophosphamide or 126 g/m 2 ifosfamide and 50.4-59.4 Gray (Gy) of radiation, increasing the risks of long-term morbidity.…”

Section: Introductionmentioning

confidence: 99%

Survival of patients with orbital and eyelid rhabdomyosarcoma treated on Children's Oncology Group studies from 1997 to 2013: A report from the Children's Oncology Group

Metts

Xue

Gao

et al. 2023

Cancer

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Background Orbital rhabdomyosarcoma (ORMS) commonly presents as low‐risk disease (stage 1, group I–III, embryonal RMS) with excellent outcome. Long‐term follow‐up of patients with low‐risk ORMS and outcomes of less common subgroups of ORMS treated on recent Children's Oncology Group (COG) trials have not been reported. Methods Patients with ORMS enrolled on COG trials from 1997 to 2013 were identified. Demographic information and disease characteristics were collected. Outcomes were determined for the following subgroups: 1) low‐risk ORMS, 2) resected (group I/II) low‐risk ORMS, 3) non–low‐risk ORMS, and 4) recurrent ORMS. Event‐free survival (EFS) and overall survival (OS) were estimated using the Kaplan–Meier method. ResultsThe authors identified 218 patients with ORMS. Most tumors were embryonal/botryoid (n = 169; 77.5%), <5 cm (n = 213; 97.7%), group III (n = 170; 78.0%), and without lymph node involvement (N0; n = 215; 98.6%). For 192 patients with low‐risk ORMS, the 10‐year EFS and OS rates were 85.5% (95% confidence interval [CI], 77.0%–94.0%) and 95.6% (95% CI, 90.8%–100.0%), respectively. Those with group I/II low‐risk ORMS (n = 5 in group I; n = 39 in group IIA) had 10‐year EFS and OS rates of 88.0% (95% CI, 72.6%–100.0%) and 97.6% (95% CI, 90.0%–100.0%), respectively. Twenty‐six patients with non–low‐risk ORMS had 5‐year EFS and OS rates of 88.5% (95% CI, 75.6%–100.0%) and 95.8% (95% CI, 87.7%–100.0%), respectively. For patients with recurrent ORMS, the 10‐year OS rate from the time of recurrence was 69.4% (95% CI, 50.0%–88.8%). Conclusions Patients with ORMS had favorable long‐term survival outcomes on COG studies from 1997 to 2013, including those who had both low‐risk and non–low‐risk disease. A significant proportion of patients with recurrent ORMS may achieve long‐term survival.

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“…At the same time, despite aggressive combination therapy, no further significant improvements have been made for the treatment of children with high-risk disease or recurrent disease (5-year survival <30% and 17%, respectively) [ 2 ]. Favorable prognostic factors include a primary tumor site in the orbit and tumors that are located in the non-parameningeal head and neck region and genitourinary sites, with the exception of the bladder and prostate; from patients aged 1–9 years; a lack of distant metastases at diagnosis; a clinical stage based on the classification of the primary procedure; a maximum tumor diameter ≤5 cm; and embryonal histology [ 1 , 7 ]. According to a report from the COG (The Children’s Oncology Group), PAX-FOXO1 fusion results in unfavorable outcomes in children with RMS [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

Cancer Stem Cell Markers in Rhabdomyosarcoma in Children

Radzikowska

Czarnecka

Klepacka³

et al. 2022

Diagnostics

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(1) Background: The aim of the present study was to assess the cancer stem cell (CSC) markers CD24, CD44, CD133, and ALDH1A1 in rhabdomyosarcoma (RMS) in children and to define their prognostic role in this group of patients. (2) Methods: The study material was archival tissue specimens collected from 49 patients under 18 years of age and who had been diagnosed with RMS. Immunohistochemistry (IHC) was used to evaluate the expression of the selected CSC markers in the tumor tissue. Expression was evaluated using a semiquantitative IRS scale based on the one developed by Remmele and Stenger and was correlated with the clinical and pathomorphological parameters of prognostic importance in RMS. (3) Results: Expression of the selected CSC markers CD24, CD44, CD133, and ALDH1A1 was demonstrated in 83.7%, 55.1%, 81.6%, and 100% of the RMS patients, respectively. The expression of all of the assessed CSC markers was statistically significantly higher in the study group versus the control group. No significant correlation was found between the expression of the selected CSC markers and clinical and pathological prognostic factors that were analyzed. The expression of the CSC markers did not have a significant influence on RMS survival rates. (4) Conclusions: The results of the conducted study confirm the expression of selected CSC markers in rhabdomyosarcoma tissue in children. The study did not support the prognostic relevance of the expression of any of the assessed CSC markers. However, further studies are needed to fully understand the relevance of the selected CSC markers in RMS carcinogenesis.

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Clinical group and modified TNM stage for rhabdomyosarcoma: A review from the Children's Oncology Group

Cited by 21 publications

References 90 publications

A Web-Based Prognostic Model for Pediatric Genitourinary Rhabdomyosarcoma: Analysis of Population-Based Cohort With External Validation

A Web-Based Prognostic Model for Pediatric Genitourinary Rhabdomyosarcoma: Analysis of Population-Based Cohort With External Validation

Survival of patients with orbital and eyelid rhabdomyosarcoma treated on Children's Oncology Group studies from 1997 to 2013: A report from the Children's Oncology Group

Cancer Stem Cell Markers in Rhabdomyosarcoma in Children

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