Therapy and prognostic significance of regional lymph node involvement in embryonal rhabdomyosarcoma: a report from the European paediatric Soft tissue sarcoma Study Group

• DOI: 10.1016/j.ejca.2022.05.033
• Publication Date: 2022-06-25T08:45:06Z
• Authors: Myriam Ben-Arush; Veronique Minard-...; Giovanni Scarzello; Raquel D. Fajardo; Sheila Terwisscha...; Valérie Bernier; Meriel Jenney; Soledad Gallego; Ilaria Zanetti; Maja Cesen; Johannes H.M. Merks; Gianni Bisogno
• Keywords: Rhabdomyosarcoma, Alveolar/drug therapy; Cancer Research; Lymph node involvement; Research Support, Non-U.S. Gov't; Rhabdomyosarcoma, Embryonal/therapy; Infant; Pediatric oncology; Chemotherapy; Embryonal rhabdomyosarcoma; Lymph node involvement; Pediatric oncology; Prognosis; Disease-Free Survival; 3. Good health; Oncology; Rhabdomyosarcoma/drug therapy; Antineoplastic Combined Chemotherapy Protocols; Rhabdomyosarcoma; Lymph Nodes/pathology; Journal Article; Chemotherapy; Humans; Rhabdomyosarcoma, Embryonal; Antineoplastic Combined Chemotherapy Protocols/therapeutic use; Lymph Nodes; Child; Rhabdomyosarcoma, Alveolar; Embryonal rhabdomyosarcoma

Regional lymph node disease (N1) is a component of the risk-based treatment stratification in rhabdomyosarcoma (RMS). The purpose of this study was to determine the contribution of nodal disease to the prognosis of patients with non-metastatic embryonal RMS (ERMS) and analyse their outcome by treatment received.Between 2005 and 2016, 1294 children with ERMS were enrolled in the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 protocol, 143 patients with N1. Treatment comprised 9 cycles of ifosfamide, vincristine and dactinomycin. Some patients also received doxorubicin and/or maintenance if enrolled in the randomised studies. Local treatment was planned after 4 cycles of chemotherapy and included surgery to remove macroscopic residual tumour and/or radiotherapy (primary tumour and involved nodes).N1 patients were older and presented with tumours of unfavourable size, invasiveness, site and resectability. Unlike alveolar RMS, nodal involvement was more frequent in the head and neck area and rare in extremity sites. The 5-year event-free and overall survival were 75.5% and 86.3% for patients with N0, and 65.2% and 70.7% for patients with N1, respectively. The nodal involvement and the result of surgery at diagnosis (Intergroup Rhabdomyosarcoma Study group) were independent prognostic factors on multivariate analysis. Considering only patients with N1 ERMS, we were not able to identify any treatment variables which correlated with the outcome.In the case of nodal involvement, patients with ERMS present different characteristics and a better outcome than alveolar RMS. Regional nodal involvement is an independent prognostic factor in ERMS, therefore it is appropriate to include this population in the high-risk category.