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. 2024 Jan;71(1):e30742.
doi: 10.1002/pbc.30742. Epub 2023 Oct 25.

Radiotherapy and long-term sequelae in pediatric patients with parameningeal rhabdomyosarcoma: Results of two Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry

Monika Sparber-Sauer  1   2 , Maximilian Dietzschold  3 , Anton Schönstein  4 , Amadeus Heinz  1 , Christian Vokuhl  5 , Kristian W Pajtler  6   7   8 , Semi Harrabi  9   10   11   12   13   14 , Yi-Lan Lin  15 , Thekla von Kalle  16 , Rudolf Hagen  17 , Ruth Ladenstein  18 , Bernarda Kazanowska  19 , Gustaf Ljungman  20 , Thomas Klingebiel  21 , Martin Ebinger  22 , Ewa Koscielniak  1   2 , Marc Münter  23 , Beate Timmermann  15   24 ; CWS Study Group
Affiliations

Radiotherapy and long-term sequelae in pediatric patients with parameningeal rhabdomyosarcoma: Results of two Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry

Monika Sparber-Sauer et al. Pediatr Blood Cancer. 2024 Jan.

Abstract

Background: Parameningeal location of rhabdomyosarcoma (PM RMS) is known to be an unfavorable prognostic factor. Scarce data are available on radiotherapy (RT) concepts with regard to outcome.

Methods: Treatment and outcome of 395 children with PM RMS registered within two Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry (1995-2021) were evaluated.

Results: Patients were IRS group II (n = 15) and III (n = 380) and received systemic treatment according to the enrolled protocols: I2VA (n = 172), VAIA/CEVAIE (n = 223). Delayed resection was performed in 88/395 (22%) patients, and RT was additionally given in 79/88 (90%) resected patients. RT was the predominant local treatment in 355/395 (90%) patients: hyperfractionated accelerated photon (HART; n = 77), conventionally fractionated photon (n = 91) or proton beam (n = 126), brachytherapy (n = 4), heavy ions (n = 1), not available (n = 56). In the subgroup of RT as only local treatment (n = 278), no intracranial tumor extension and complete remission at end of treatment were significant positive prognostic factors. No significant difference on tumor outcome was seen between different radiotherapy concepts. Long-term toxicity with mostly endocrinological and visual deficiencies was reported in 161/279 (58%) surviving patients with a lower trend after proton beam RT (48%) when compared to HART or conventionally fractionated photon RT (71% and 72%, respectively). Ten-year event-free and overall survival in the overall group were 62% (±5, 95% confidence interval [CI]) and 67% (±5, 95% CI); in the RT-only group 67% (±6, 95% CI) and 71% (±6, 95% CI), respectively.

Conclusion: CWS data confirm the recent RT concept in PM RMS. Long-term sequelae as endocrinological and visual deficiencies need to be addressed.

Keywords: CWS Group; children and adolescents; parameningeal rhabdomyosarcoma; proton beam irradiation.

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References

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