Rhabdomyosarcoma in children: Retrospective analysis from a single tertiary care center in Saudi Arabia

Markiz, Samer; Khan, Safia; Wagley, Zainab B.; Viqaruddin, Mohammed K.; Khafaga, Yasser; AlFawaz, Ibrahim A.; Alanazi, Awatif; Al-Kofide, Amani; Khoja, Hatem; Ali, Ashraf

doi:10.1002/cnr2.1683

Cited by 2 publications

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References 29 publications

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“…Despite these similarities, the management strategies and prognoses for RMS and NB differ. Children diagnosed with RMS typically have surgery, while NB treatment varies by risk category and may include chemotherapy, surgery, radiation, stem cell transplant, immunotherapy, and retinoid therapy (3,11). In addition, the 5-year relative survival rate for low-risk and localized RMS in children exceeds 80% with surgery, radiation therapy, and chemotherapy.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of clinical and imaging features for differentiating rhabdomyosarcoma from neuroblastoma in pediatric soft tissue

Sheng,

Li,

et al. 2024

Front. Oncol.

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BackgroundIn this study, we developed a nomogram predictive model based on clinical, CT, and MRI parameters to differentiate soft tissue rhabdomyosarcoma (RMS) from neuroblastoma (NB) in children preoperatively.Materials and methodsA total of 103 children with RMS (n=37) and NB (n=66) were enrolled in the study from December 2012 to July 2023. The clinical and imaging data (assessed by two experienced radiologists) were analyzed using univariate analysis, and significant factors were further analyzed by multivariable logistic regression using the forward LR method to develop the clinical model, radiological model, and integrated nomogram model, respectively. The diagnostic performances, goodness of fit, and clinical utility of the integrated nomogram model were assessed using the area under the curve (AUC) of the receiver operator characteristics curve (ROC) with a 95% confidence interval (95% CI), calibration curve, and decision curve analysis (DCA) curves, respectively. Diagnostic efficacy between the model and radiologists’ interpretations was examined.ResultsThe median age at diagnosis in the RMS group was significantly older than the NB group (36.0 months vs. 14.5 months; P=0.003); the fever rates in RMS patients were significantly lower than in patients with NB (0.0% vs.16.7%; P=0.022), and the incidence of palpable mass was higher in patients with RMS compared with the NB patients (89.2% vs. 34.8%; P<0.001). Compare NB on image features: RMS occurred more frequently in the head and neck and displayed homogeneous density on non-enhanced CT than NB (48.6% vs. 9.1%; 35.3% vs. 13.8%, respectively; all P<0.05), and the occurrence of characteristics such as calcification, encasing vessels, and intraspinal tumor extension was significantly less frequent in RMS children compared to children with NB (18.9% vs. 84.8%; 13.5% vs. 34.8%; 2.7% vs. 50.0%, respectively; all P <0.05). Two, three, and four features were identified as independent parameters by multivariate logistic regression analysis to develop the clinical, radiological, and integrated nomogram models, respectively. The AUC value (0.962), calibration curve, and DCA showed that the integrated nomogram model may provide better diagnostic performance, good agreement, and greater clinical net benefits than the clinical model, radiological model, and radiologists’ subjective diagnosis.ConclusionThe clinical and imaging features-based nomogram has potential for helping radiologists distinguish between pediatric soft tissue RMS and NB patients preoperatively, and reduce unnecessary interventions.

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Section: Introductionmentioning

confidence: 99%

Evaluation of clinical and imaging features for differentiating rhabdomyosarcoma from neuroblastoma in pediatric soft tissue

Sheng,

Li,

et al. 2024

Front. Oncol.

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Rhabdomyosarcoma in children: Retrospective analysis from a single tertiary care center in Saudi Arabia

Markiz

Khan²,

Wagley

et al. 2022

Cancer Reports

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Background Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children occurring most commonly in the head and neck region. The treatment involves using a multimodality approach including chemotherapy, surgery, and radiation therapy. Survival for patients with localized disease has improved markedly, but the treatment of advanced disease remains a challenge. We report the clinical characteristics and outcome for patients treated at a tertiary care center in Saudi Arabia. Methods Patients aged 0–14 years diagnosed with RMS between 2005 and 2018 were included. Statistical analysis was performed using SPSS software. Kaplan–Meier method was used to calculate overall and event free survival. Cox proportional hazards model was used for multivariate analysis. Results One hundred and twenty‐four patients were analyzed. The median age was 5.7 years with male predominance (2.4:1). The most common primary sites were head/neck (30%) and the genitourinary tract (25%). Embryonal RMS was present in 81%; alveolar in 19%. Most patients had intermediate risk disease (60%). The 5‐year overall and event free survivals were 64.3% and 53.3%, respectively. Survival was influenced by primary tumor site, histology, and clinical risk group. Unfavorable primary site, high risk stratification, and poor initial response to therapy predicted a poor outcome. Conclusion This study provides an insight on the current management outcomes for our patients with RMS. Cytogenetics and molecular diagnostics need to be incorporated as standard of care in the therapeutic approach of our patients. In addition, there is a need for national collaborative efforts to improve the outcome of RMS in children and adolescents.

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Rhabdomyosarcoma in children: Retrospective analysis from a single tertiary care center in Saudi Arabia

Cited by 2 publications

References 29 publications

Evaluation of clinical and imaging features for differentiating rhabdomyosarcoma from neuroblastoma in pediatric soft tissue

Evaluation of clinical and imaging features for differentiating rhabdomyosarcoma from neuroblastoma in pediatric soft tissue

Rhabdomyosarcoma in children: Retrospective analysis from a single tertiary care center in Saudi Arabia

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