Fusion status in patients with lymph node‐positive (N1) alveolar rhabdomyosarcoma is a powerful predictor of prognosis: Experience of the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG)

Gallego, Soledad; Zanetti, Ilaria; Orbach, Daniel; Ranchère, Dominique; Shipley, Janet; Zin, Angelica; Bergeron, Christophe; Salvo, Gian Luca De; Chisholm, Julia; Ferrari, Andrea; Jenney, Meriel; Mandeville, Henry; Rogers, Timothy; Merks, Johannes H. M.; Múdrý, Peter; Glosli, Heidi; Milano, Giuseppe Maria; Ferman, Sima; Bisogno, Gianni

doi:10.1002/cncr.31553

Cited by 58 publications

(54 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…1,3,5,17,18 The local therapy approach differed per protocol. Treatment generally consisted of a combination of chemotherapy with surgery and/or radiotherapy, as described previously.…”

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

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

Vaarwerk

Mallebranche

Affinita

et al. 2019

Cancer

Self Cite

View full text Add to dashboard Cite

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.

show abstract

“…1,3,5,17,18 The local therapy approach differed per protocol. Treatment generally consisted of a combination of chemotherapy with surgery and/or radiotherapy, as described previously.…”

Section: Methodsmentioning

confidence: 99%

“…Treatment generally consisted of a combination of chemotherapy with surgery and/or radiotherapy, as described previously. 1,3,5,17,18 The local therapy approach differed per protocol. If possible, delayed surgery was performed in case of residual tumor.…”

Section: Methodsmentioning

confidence: 99%

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

Vaarwerk

Mallebranche

Affinita

et al. 2019

Cancer

Self Cite

View full text Add to dashboard Cite

show abstract

“…Histologic diagnosis of alveolar RMS (RMA) or metastatic tumors is a known poor prognostic factor in infants . Fusion status or mutations as MYOD1 in patients with RMS are recently reported as powerful predictors of prognosis . In contrast, spindle‐cell RMS in infancy with NCOA2 gene rearrangements is reported to be associated with an excellent prognosis…”

Section: Introductionmentioning

confidence: 99%

“…4 Fusion status or mutations as MYOD1 in patients with RMS are recently reported as powerful predictors of prognosis. 11,12 In contrast, spindle-cell RMS in infancy with NCOA2 gene rearrangements is reported to be associated with an excellent prognosis. 13 In this report, we describe the characteristics, outcome, prognostic factors, pattern of relapse, long-term sequelae, and second malignancies for infants with localized and metastatic RMS treated within five trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS).…”

Section: Introductionmentioning

confidence: 99%

Rhabdomyosarcoma diagnosed in the first year of life: Localized, metastatic, and relapsed disease. Outcome data from five trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS)

Sparber‐Sauer

Stegmaier

Vokuhl³

et al. 2019

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Background Rhabdomyosarcoma (RMS) diagnosed during the first year of life is reported to have poor outcome. Little is known about treatment and outcome data of relapsed disease (RD). Methods Characteristics, treatment, and outcome of 155 patients ≤ 12 months registered within the Cooperative Weichteilsarkom Studiengruppe (CWS) between 1981 and 2016 were evaluated. Results Localized disease (LD) was diagnosed in 144 patients and metastatic disease (MD) in 11. The histological diagnosis was alveolar (RMA) (n = 38, 23/25 examined patients PAX7/3:FOXO1‐positive), embryonal (RME) (n = 100), botryoid (n = 10), anaplastic (n = 1), and spindle‐cell RMS (n = 6). Multimodal treatment including conventional (age‐adjusted) chemotherapy (CHT) (n = 150), resection (n = 137), and radiotherapy (RT) (n = 37) was administered. Complete remission was achieved in 129 of 144 patients with LD. RD occurred in 51 infants at a median age of 1.7 years (range, 0.3–8.8). Sixty‐three percent of patients with RMA suffered RD, in contrast to 28% of patients with RME. Relapse treatment consisted of conventional CHT (n = 48), resection (n = 28), and RT (n = 21). The pattern of relapse and best resection were significant prognostic factors for patients with RD (P = 0.000 and P = 0.002). Late effects occurred as secondary malignancies in 6%, long‐term toxicity in 21%, and resection‐related impairment in 33% of the 105 surviving patients. The 5‐year event‐free survival and overall survival for infants with initial LD were 51% and 69%, 14% and 14% for patients with initial MD and 39% and 41% for relapsed patients, respectively. Conclusion Multimodal treatment including microscopically complete resection is strongly recommended to achieve a good prognosis in LD and RD of infants with RMS.

show abstract

“…a sonda mais frequentemente utilizada é a FOXO1 gene dual color break-apart que pode detectar ambas as translocações envolvendo o gene FOXO1 6,13 . atualmente, existe a proposta para a inclusão dessa alteração molecular como um dos fatores de prognóstico da doença 14 .…”

Section: Discussionunclassified

Alterações Citogenético-Moleculares no Gene FOXO1 em uma Criança com Rabdomiossarcoma Alveolar: Relato de Caso

Cunha

Suzuki

Lima

et al. 2019

Rev. Brasileira.De.Cancerologia

View full text Add to dashboard Cite

Introdução: O rabdomiossarcoma (RMS) é o tumor de tecidos moles mais comum da infância. Pode ser classificado em dois subtipos principais: o rabdomiossarcoma alveolar (RMSa) e o embrionário (RMSe). No RMSa, o prognóstico é desfavorável quando comparado ao RMSe, necessitando de tratamento intensificado; dessa forma, a distinção entre ambos os subtipos é fundamental. Citogeneticamente, o RMSa apresenta translocações cromossômicas envolvendo o gene FOXO1 em 80% dos casos. A metodologia de hibridização in situ por fluorescência (FISH) tem sido muito utilizada para caracterizar o RMSa. Relato do caso: Paciente do sexo feminino, com 7 anos de idade, apresentou ao diagnóstico RMSa parameníngeo, sem metástase ao diagnóstico. A análise por meio de FISH mostrou a translocação envolvendo o gene FOXO1 e uma cópia extra desse gene. A paciente foi incluída no protocolo de tratamento do EpSSG, classificada como grupo de alto risco e recebeu quimioterapia e radioterapia. No final do tratamento, foi observada resposta parcial e iniciada quimioterapia de segunda linha. Não houve resposta clinicorradiológica e a paciente evoluiu com progressão de doença local refratária ao tratamento e óbito após um ano do diagnóstico. Conclusão: De acordo com o nosso conhecimento, é a primeira descrição de um caso de RMSa apresentando a translocação do gene FOXO1 e uma cópia extra desse gene em clones separados. São necessários ainda novos estudos, a fim de compreender melhor o significado prognóstico da presença dessas alterações.

show abstract

Fusion status in patients with lymph node‐positive (N1) alveolar rhabdomyosarcoma is a powerful predictor of prognosis: Experience of the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG)

Cited by 58 publications

References 36 publications

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

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

Rhabdomyosarcoma diagnosed in the first year of life: Localized, metastatic, and relapsed disease. Outcome data from five trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS)

Alterações Citogenético-Moleculares no Gene FOXO1 em uma Criança com Rabdomiossarcoma Alveolar: Relato de Caso

Contact Info

Product

Resources

About