Regional Nodal Involvement and Patterns of Spread Along In-Transit Pathways in Children With Rhabdomyosarcoma of the Extremity: A Report From the Children's Oncology Group

Abstract

Purpose-To evaluate the incidence and prognostic factors for regional failure, with attention to in-transit pathways of spread, in children with non-metastatic rhabdomyosarcoma (RMS) of the extremity.Materials/Methods-Intergroup Rhabdomyosarcoma Studies III, IV-Pilot, and IV enrolled 226 children with RMS of the extremity. Failure at in-transit (epitrochlear/brachial and popliteal) and proximal (axillary/infraclavicular and inguinal/femoral) nodes were evaluated. Median follow-up for surviving patients is 10.4 years.Results-Fifty-five (24%) of 226 children had clinical or pathologic evidence of either in-transit and/or proximal lymph node involvement (LNI) at diagnosis. The actuarial 5-year risk of regional failure is 12%. The prognostic factors for poor regional control are female gender and LNI at diagnosis. In the 116 patients with a distal extremity primary tumor, 5% had in-transit LNI at diagnosis. The estimated 5-year incidences of in-transit and proximal nodal failure are 12% and 8%, respectively. The in-transit failure rate is 0% in patients who received radiation therapy and/or underwent lymph node sampling of the in-transit nodal site, but 15% in those who did not (p=0.07), although 5-year EFS did not differ between these two groups (64% vs. 55%, p=0.47). Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Conclusions-The NIH Public Access

Prognostic Factors for Outcome in Localized Extremity Rhabdomyosarcoma. Pooled Analysis from Four International Cooperative Groups

“…Nodal involvement was common (21%) and more common for alveolar than for non‐alveolar tumors. This is in line with data in a detailed study of patterns of nodal involvement in extremity RMS which also raised the issue of managing potential/confirmed involvement of in‐transit nodes . In that study, patients who underwent complete lymph node staging with appropriate radiotherapy to the in‐transit nodal site were at slightly lower risk of in‐transit failure.…”

“…This is in line with data in a detailed study of patterns of nodal involvement in extremity RMS which also raised the issue of managing potential/confirmed involvement of in-transit nodes. [21] In that study, patients who underwent complete lymph node staging with appropriate radiotherapy to the in-transit nodal site were at slightly lower risk of in-transit failure. However, the delivery of optimal local therapy to limit failure in in-transit nodes in patients with extremity tumors remains a challenge.…”

Children's Oncology Group's 2013 blueprint for research: Soft tissue sarcomas

“…Any future phase III study will use PAX-FOXO1 fusion status rather than ARMS/ERMS for treatment allocation. In addition, future phase III studies will incorporate standard requirements for lymph node evaluation, since regional lymph node involvement is associated with outcome in ARMS [114] and is an important site of relapse [115]. Phase III studies will include local treatment pathways that maximize local control and minimize morbidity [116][117][118][119][120].…”

Detection of lymph node metastases in pediatric and adolescent/young adult sarcoma: Sentinel lymph node biopsy versus fludeoxyglucose positron emission tomography imaging—A prospective trial

“…Although it is less common, nodal involvement can also be seen in clear cell sarcoma, epithelioid sarcoma, angiosarcoma, and occasionally synovial sarcoma . The accurate detection of nodal metastases allows patients to receive appropriate additional therapy such as irradiation or completion lymphadenectomy, which can reduce subsequent recurrence rates …”