Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma

Dantonello, Tobias; Int-Veen, Christoph; Schuck, Andreas; Seitz, Guido; Leuschner, Ivo; Nathrath, Michaela; Schlegel, Paul‐Gerhardt; Kontny, Udo; Behnisch, Wolfgang; Veit-Friedrich, Iris; Kube, Stefanie; Hallmen, Erika; Kazanowska, Bernarda; Ladenstein, Ruth; Paulussen, Michael; Ljungman, Gustaf; Bielack, Stefan; Klingebiel, Thomas; Kościelniak, Ewa; Studiengruppe, Cooperative Weichteilsarkom

doi:10.1002/pbc.24488

Cited by 48 publications

(41 citation statements)

References 32 publications

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“…We speculated that after events, salvage RT took the place of initial RT and became a high-weight prognostic factor, and this speculation was supported by our results that salvage RT was a statistically significant prognostic factor for OS in patients with events. It's reported that adequate local therapy is an important factor for survival after relapse [29], RT can further improve OS in relapsed patients undergone a repeat surgery [30], and local treatment such as RT and repeat surgery should be systemically considered even in previously irradiated patients [31]. The cure of parameningeal RMS is unlikely without RT [11].…”

Section: Discussionmentioning

confidence: 99%

“…These patients' survival is affected by many factors and some of them may be higher-weight prognostic factors, such as tumor size, primary site, regional nodal involvement, metastasis, repeat surgery, previous chemo and RT plan, multi-relapse, etc. [3,19,27,29,32]. All these factors make salvage RT unlikely to be an independent prognostic factor.…”

Section: Discussionmentioning

confidence: 99%

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Radiation therapy is an important factor to improve survival in pediatric patients with head and neck rhabdomyosarcoma by enhancing local control: a historical cohort study from a single center

et al. 2020

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Background: The purpose of this study is to analyze the influence of radiation therapy on survival in a historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma. Methods: A historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma from June 1st, 2013 to June 30th, 2019 was chosen. Clinical data and follow up results were collected including all diagnosis, treatment and prognosis information. Overall survival (OS) and event free survival (EFS) as time-to-event distributions were estimated with Kaplan-Meier method, and univariate analysis was performed with log rank test to detect differences between groups. Multivariate analysis was performed to explore the risk factors for survival with Cox proportional hazard model. Results: The media follow up time of all 56 patients was 31.8 months (range 3.5-74.6 months). There were 26 events during follow up, including 14 disease progressions and 12 relapses. The estimated 5-year OS of all patients was 69.9%, and the estimated 5-year EFS was 48.8%. Patients with radiation therapy as a component of the initial treatment plan had better 5-year OS and EFS compared with those without radiation therapy (OS 80.3% vs. 49.7%, p = 0.003 and EFS 63.9% vs. 21.9%, p < 0.001). In patients with events, those who received salvage radiation therapy had better 5-year OS compared with those who didn't (OS 66.0% vs. 31.2%, p = 0.033). On multivariate analysis, tumor size > 5 cm and non-initial radiation therapy were independent risk factors for OS in all patients, non-initial radiation therapy was an independent risk factor for EFS in all patients, and tumor size > 5 cm was an independent risk factor for OS in patients with events. Conclusions: Radiation therapy as a component of initial treatment can improve the OS and EFS in pediatric head and neck rhabdomyosarcoma patients by enhancing local control, and non-initial radiation therapy is an independent risk factor for OS and EFS. Salvage radiation therapy still can improve OS in patients with disease progression and relapse. Tumor size > 5 cm is an independent risk factor for OS in pediatric HNRMS patients with or without disease progression/relapse.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Radiation therapy is an important factor to improve survival in pediatric patients with head and neck rhabdomyosarcoma by enhancing local control: a historical cohort study from a single center

et al. 2020

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“…Three-year survival after relapse is approximately 37%, and it is associated with several factors such as histology, the initial tumor site, the pattern of relapse (local or metastatic), and prior radiotherapy. [4][5][6][7][8] The recommended surveillance after treatment, according to the European Paediatric Soft Tissue Sarcoma Study Group Rhabdomyosarcoma 2005 (EpSSG-RMS 2005) protocol, includes a clinical examination together with a magnetic resonance imaging (MRI) or computed tomography (CT) scan of the primary tumor site and a chest x-ray, which should be performed every 3 months in the first year and every 4 months in the second and third years after treatment. The recommended surveillance is once a year in the fourth and fifth years after treatment.…”

Section: Introductionmentioning

confidence: 99%

Is surveillance imaging in pediatric patients treated for localized rhabdomyosarcoma useful? The European experience

Vaarwerk

Mallebranche

Affinita

et al. 2019

Cancer

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BACKGROUND: After the completion of therapy, patients with localized rhabdomyosarcoma (RMS) are subjected to intensive radiological tumor surveillance. However, the clinical benefit of this surveillance is unclear. This study retrospectively analyzed the value of off-therapy surveillance by comparing the survival of patients in whom relapse was detected by routine imaging (the imaging group) and patients in whom relapse was first suspected by symptoms (the symptom group). METHODS: This study included patients with relapsed RMS after the completion of therapy for localized RMS who were treated in large pediatric oncology hospitals in France, the United Kingdom, Italy, and the Netherlands and who were enrolled in the International Society of Paediatric Oncology Malignant Mesenchymal Tumor 95 (1995)(1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004) study, the Italian Paediatric Soft Tissue Sarcoma Committee Rhabdomyosarcoma 96 (1996-2004) study, or the European Paediatric Soft Tissue Sarcoma Study Group Rhabdomyosarcoma 2005 (2005-2013) study. The survival times after relapse were compared with a log-rank test between patients in the imaging group and patients in the symptom group. RESULTS: In total, 199 patients with relapsed RMS were included: 78 patients (39.2%) in the imaging group and 121 patients (60.8%) in the symptom group. The median follow-up time after relapse was 7.4 years (interquartile range, 3.9-11.5 years) for survivors (n = 86); the 3-year postrelapse survival rate was 50% (95% confidence interval [CI], 38%-61%) for the imaging group and 46% (95% CI, 37%-55%) for the symptom group (P = .7). CONCLUSIONS: Although systematic routine imaging is the standard of care after RMS therapy, the majority of relapses were detected as a result of clinical symptoms. This study found no survival advantage for patients whose relapse was detected before the emergence of clinical symptoms. These results show that the value of off-therapy surveillance is controversial, particularly because repeated imaging may also entail potential harm. Cancer 2020;126:823-831.

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“…Patients with disease progression respond poorly to the initial treatment, and primary tumor enlargement, local invasion, regional nodal involvement, and even metastasis can be anticipated. Disease relapse represents late treatment failure [19,20], which means recurrence of RMS in any form after last treatment during off therapy surveillance. Patients with disease relapse usually respond well to the initial treatment and end with complete remission.…”

Section: Discussionmentioning

confidence: 99%

Risk Factors for Disease Progression and Prognosis Analysis in Pediatric Head and Neck Rhabdomyosarcoma Patients: a Historical Cohort Study From a Single Center

Wen¹,

Zhang²,

Zhang³

et al. 2020

Preprint

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Background: The purposes of this study are to explore risk factors for disease progression during primary treatment, and to analyze disease prognosis of different event groups in a historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma.Methods: From June 1st, 2016 to June 30th, 2019, a historical cohort of 56 pediatric patients diagnosed as head and neck rhabdomyosarcoma was chosen. Clinical data were collected including general information about diagnosis, treatment, prognostic factors, and follow up results. Patients were divided into non-event group, disease-progression group and disease-relapse group according different types of events. Comparison among the three groups was made to reveal different clinical features and prognosis. Patients were further divided into disease-progression group and non-progression group to explore risk factors for disease progression, with univariate and multivariate analysis.Results: The median follow-up time of all 56 patients was 31.8 months (range 3.5-74.6 months). There were 30 non-event patients, 12 disease relapse patients, and 14 disease-progression patients. The estimated 5-year overall survival rates were 100.0%, 82.5%, and 11.9% for non-event, disease-relapse and disease-progression patients respectively (p<0.001). Compared with non-event patients, disease-progression patients had higher rates of tumor size>5cm (p=0.008) and non-initial radiation therapy (p=0.001). Compared with disease-relapse patients, disease-progression patients had a higher rate of tumor size>5cm (p=0.002), and higher ranks of surgical-pathologic group (p=0.009) and risk group (p<0.001). Compare with non-progression patients, univariate analysis demonstrated that tumor size>5cm, alveolar histology, non-initial radiation therapy, and higher ranks of surgical-pathologic group as well as risk group were statistically significant risk factors for disease progression. The multivariate analysis result showed tumor size>5cm (OR=6.75, 95%CIs 1.02-44.78), alveolar histology (OR=7.10, 95%CIs 1.08-46.57), and non-initial radiation therapy (OR=15.26, 95%CIs 2.38-97.70) to be independent risk factors for disease progression.Conclusions: Pediatric head and neck rhabdomyosarcoma patients with disease progression have significantly worse overall survival, and the prognosis is obviously different from patients with disease relapse and patients without events. Tumor size>5cm, alveolar histology and non-initial radiation therapy are independent risk factors for disease progression. Patients with disease progression tend to have higher ranks of surgical-pathologic group and risk group.

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Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma

Cited by 48 publications

References 32 publications

Radiation therapy is an important factor to improve survival in pediatric patients with head and neck rhabdomyosarcoma by enhancing local control: a historical cohort study from a single center

Radiation therapy is an important factor to improve survival in pediatric patients with head and neck rhabdomyosarcoma by enhancing local control: a historical cohort study from a single center

Is surveillance imaging in pediatric patients treated for localized rhabdomyosarcoma useful? The European experience

Risk Factors for Disease Progression and Prognosis Analysis in Pediatric Head and Neck Rhabdomyosarcoma Patients: a Historical Cohort Study From a Single Center

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