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Local treatment in initially unresected non-rhabdomyosarcoma soft-tissue sarcomas of children and adolescents: A retrospective single-center experience.

Author information

Affiliations

  • 1. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
      Authors
    • Ferrari A 1
    • Bergamaschi L 1
    • Chiaravalli S 1
    • Puma N 1
    • Luksch R 1
    • Terenziani M 1
    • Spreafico F 1
    • Meazza C 1
    • Podda M 1
    • Biassoni V 1
    • Schiavello E 1
    • Massimino M 1
    • Casanova M 1
    (13 authors)
  • 2. Pediatric Radiotherapy Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.
      Authors
    • Vennarini S 2
    • Pecori E 2
    (2 authors)
  • 3. Department of Surgery, Fondazione IRCCS, Istituto Nazionale dei Tumori, Milan, Italy.
      Authors
    • Fiore M 3
    • Colombo C 3
    (2 authors)
  • 4. Radiology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
      Authors
    • Morosi C 4
    (1 author)

ORCIDs linked to this article

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Pediatric Blood & Cancer, 31 Jan 2024, 71(4):e30901
https://doi.org/10.1002/pbc.30901 PMID: 38296840 

Abstract 

Background

Pediatric non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) are a heterogeneous group of aggressive tumors. Patients with locally advanced/initially unresected disease represent a subset of patients with unsatisfactory outcome: limited data are available on the best treatment approach, in particular regarding local therapy.

Methods

This retrospective analysis concerned 71 patients < 21 years old with nonmetastatic, initially unresected adult-type NRSTS, treated at a referral center for pediatric sarcomas from 1990 to 2021. Patients were treated using a multimodal approach, based on the protocols adopted at the time of their diagnosis.

Results

The series included a selected group of patients with unfavorable clinical characteristics, i.e., most cases had high-grade and large tumors, arising from axial sites in 61% of cases. All patients received neoadjuvant chemotherapy, 58 (82%) had delayed surgery (R0 in 45 cases), and 50 (70%) had radiotherapy. Partial response to chemotherapy was observed in 46% of cases. With a median follow-up of 152 months (range, 18-233), 5-year event-free survival (EFS) and overall survival (OS) were 39.9% and 56.5%, respectively. Survival was significantly better for patients who responded to chemotherapy, and those who had a delayed R0 resection. Local relapse at 5 years was 7.7% for patients who did not undergo delayed surgery.

Conclusions

Our series underscores the unsatisfactory outcome of initially unresected NRSTS patients. Improving the outcome of this patient category requires therapeutic strategies able to combine novel effective systemic therapies with a better-defined local treatment approach to offer patients the best chances to have R0 surgery.

Full text links 

Read article at publisher's site: https://doi.org/10.1002/pbc.30901

Read article for free, from open access legal sources, via Unpaywall: https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/pbc.30901Unpaywall

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