Metastatic rhabdomyosarcoma with exclusive distant lymph node involvement: A European Pediatric Soft tissue sarcoma Study Group (EpSSG) report

Mercolini F, Merks JHM, Minard-Colin V, Cameron A, Terwisscha van Scheltinga S, Sher O, Fichera G, Orbach D, Glosli H, Coppadoro B, Gallego S, Chisholm J, Bisogno G. Pediatr Blood Cancer
. 2023 Mar;70(3):e30143. doi: 10.1002/pbc.30143.

Prognosis of patients with metastatic rhabdomyosarcoma is still poor. However, efforts must be made to stratify patients thus to tailor treatments.
In this study, patients affected by rhabdomyosarcoma with the presence of metastases to distant lymph nodes, in the absence of other metastatic sites of disease, were analysed. These patients have been found to have markedly superior prognoses to other patients with metastatic disease and comparable to those with localized disease and locoregional lymph node involvement. In particular patients with fusion-negative (PAX3/7-FOXO1) tumors showed excellent prognosis.
This data, which will have to be confirmed on even larger series, could suggest that some rhabdomyosarcomas may have a tendency to spread exclusively by lymphatic route (and not by hematogenous route) and present a minor biological aggressiveness.

Keywords: EpSSG; children; nodal extension; rhabdomyosarcoma; staging.