Consensus Document Outlines Optimal Pediatric Chest Wall Rhabdomyosarcoma Care

The International Soft-Tissue Sarcoma Consortium (INSTRuCT) has issued a new consensus document outlining optimal treatment strategies for pediatric and adolescent patients with rhabdomyosarcoma (RMS) of the chest wall. The recommendations were published in Pediatric Blood & Cancer.1

RMS is the most common pediatric soft tissue sarcoma, but RMS of the chest wall is rare. Approximately 2.5 to 4% of solid malignant tumors in children are RMS, but only 2% of RMS cases are those in which the cancer primarily arises from the chest wall.2

The new recommendations are the result of collaboration between several global pediatric soft tissue sarcoma groups. | Image credit: ColoredLights - stock.adobe.com

The authors explained that the chest wall is considered an unfavorable site for staging. A 2008 report found that 5-year overall survival (OS) is just 61%, and that about 4 in 10 patients develop metastatic disease.3 Metastatic disease is associated with a particularly poor prognosis, the authors noted.

The new recommendations are the result of collaboration between several global pediatric soft tissue sarcoma groups, including the Children’s Oncology Group, the European Pediatric Soft Tissue Sarcoma Group, and the Cooperative Weichteilsarkom Studiengruppe. The document covers everything from staging to therapy, reconstructive approach, and long-term follow-up.1

In terms of staging, the investigators noted that a series of imaging and diagnostic tests are required to identify and locate the cancer and possible lymph node metastasis. The list includes magnetic resonance imaging (MRI) of the thorax, computed tomography (CT) of the chest, and whole-body positron emission tomography (PET)/MRI or PET/CT when feasible. They added, however, that PET-CT should not be solely relied upon to detect lymph node metastases due to its low positive predictive value.

Biopsies should be used for histological confirmation of radiographically enlarged nodes, they said. The authors said both incisional biopsies and image-guided core-needle biopsies can be considered, with the choice of technique guided by the size and location of the tumor, as well as the experience of the clinician.

Upfront resection is rarely indicated in chest wall RMS, the authors said, so “virtually all” patients will require radiation therapy. They said high-dose definitive RT (50.4 Gy) or delayed primary excision (DPE) with reduced-dose adjuvant RT (36-45 Gy) can be considered for local control.

“However, with emerging data suggesting a potential local control advantage when R0 excision can be achieved, DPE should be considered if gross total excision can be achieved without excess morbidity or excision of vital structures,” they said.

The investigators next discussed radiation therapy approaches, surgery timing, and lymph node assessment before delving into the difficult issue of surgical reconstruction of the thoracic cage. They noted that reconstruction can include everything from rigid reconstruction with rods, plates, or bone grafts to flexible reconstruction with bioabsorbable meshes or no reconstruction at all if a defect can be covered by chest wall muscle.

“The reconstruction should have sufficient rigidity in order to avoid paradoxical movements, be malleable, have low infection rates, and be biologically inert, as well as affordable,” they wrote. “Additionally, the method of reconstruction should allow adjunctive radiotherapy if necessary.”

Initial chest wall reconstruction should occur directly after tumor resection in the same session and thus requires careful advanced planning, the authors wrote. Additionally, long-term follow-up care should be concerned not only with recurrence monitoring but also with the possible complications of surgery, such as scoliosis, thoracic asymmetry, or reduced mobility, among others.

The investigators noted that there is a limited number of studies specifically looking at chest wall RMS, so some of the recommendations in the document are extrapolated from data related to the larger RMS population, rather than based on direct evidence in patients with chest wall RMS. Still, they said the recommendations show that careful, thorough planning can lead to optimal results.

“Carefully coordinated multidisciplinary care at specialty centers with the expertise for complex en bloc resection and customized reconstruction is key to successful oncological and functional outcomes,” they wrote.

References

1. Schmidt A, Guillén G, Luo LY, et al. Management of pediatric and adolescent rhabdomyosarcoma of the chest wall: an expert consensus opinion from the International Soft Tissue Sarcoma Consortium (INSTRuCT). Pediatr Blood Cancer. Published online October 27, 2025. doi:10.1002/pbc.32117
2. Martin-Giacalone BA, Weinstein PA, Plon SE, Lupo PJ. Pediatric Rhabdomyosarcoma: epidemiology and genetic susceptibility. J Clin Med. 2021;10(9):2028. doi:10.3390/jcm10092028
3. Hayes-Jordan A, Stoner JA, Anderson JR, et al. The impact of surgical excision in chest wall rhabdomyosarcoma: a report from the Children's Oncology Group. J Pediatr Surg. 2008;43(5):831-836. doi:10.1016/j.jpedsurg.2007.12.021