Local Therapy for Rhabdomyosarcoma of the Hands and Feet: Is Amputation Necessary? A Report From the Children's Oncology Group

Abstract

Purpose To evaluate the outcome of children with rhabdomyosarcoma (RMS) of the hand or foot treated with surgery and/or local radiotherapy (RT). Methods Forty-eight patients with non-metastatic RMS of the hand or foot were enrolled on Intergroup Rhabdomyosarcoma Study Group III, IV-Pilot, and IV. Patients received multi-agent chemotherapy with surgery and/or RT. Twenty-four patients (50%) underwent surgery without local RT, of whom 4 had complete resection and 20 had an amputation. The remaining 24 patients (50%) underwent local RT, of whom 2 required RT for microscopic residual disease following prior amputation. Median follow-up for surviving patients is 9.7 years. Results Actuarial 10-year local control is 100%; 10-year event-free (EFS) and overall survival (OS) are 62% and 63%, respectively. Poor prognostic factors for recurrence include gross residual (Group III) disease and nodal involvement (p=0.01 and 0.05, respectively). More patients in the RT group had alveolar histology, Group III disease, and nodal involvement, as compared to the surgery group. There is no difference in 10-year EFS (57% vs. 66%) or OS (63% vs. 63%) between patients who underwent surgery or local RT. Among relapsing patients, there are no long-term survivors. No secondary malignancies have been observed. Conclusions Despite having high-risk features, patients treated with local RT achieved excellent local control. Complete surgical resection without amputation is difficult to achieve in the hand or foot. Therefore, we recommend either definitive RT or surgical resection which maintains form and function as primary local therapy rather than amputation in patients with hand or foot rhabdomyosarcoma.

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

“…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].…”

Survival stratification in childhood rhabdomyosarcoma of the extremities: a derivation and validation study

“…Studies have tried to explore the associations of age with patient survival and have shown shorter median survival 11 , lower long-term survival probabilities and worse overall/cause-specific/failure-free/event-free survival 8,9,[12][13][14][15] in older patients. Furthermore, Archer 16 concluded that young children tend to have an increased incidence of second malignant neoplasms (SMNs), potentially as a result of a lower chance of death from RMS before developing SMNs and a longer survival time in which to develop SMN.…”

Soft Tissue Masses

“…Fortunately, with modern surgical techniques, combined with chemotherapy and radiation, 70-80% of patients achieve local control [ 76 , 77 ]. There is some evidence that primary radiotherapy to treat tumors in the hands and feet, where surgery would be severely disfi guring, is as effi cacious as surgery [ 78 ]. All patients are offered chemotherapy, as the tumor is assumed to be metastatic at presentation.…”