The challenging role of radiation therapy for very young children with rhabdomyosarcoma Journal Article


Authors: Puri, D. R.; Wexler, L. H.; Meyers, P. A.; La Quaglia, M. P.; Healey, J. H.; Wolden, S. L.
Article Title: The challenging role of radiation therapy for very young children with rhabdomyosarcoma
Abstract: Purpose: To evaluate local control and toxicity for very young children treated with multimodality therapy for rhabdomyosarcoma (RMS). Methods and Materials: From 1990 to 2004, 20 patients ≤36 months at diagnosis were treated at our institution. Nineteen underwent chemotherapy (CMT), surgery and/or intraoperative high-dose-rate brachytherapy (IOHDR), and external-beam radiation (EBRT). Median age was 17 months. Sites included extremity (7), trunk (5), parameningeal (4), orbit (1), head/neck (1), bladder/prostate (1). Histologies consisted of 10 embryonal (53%) and 9 alveolar/undifferentiated (47%). Ten had delayed gross total resection (GTR) at median time of 17 weeks after the start of CMT, and 8 of these underwent IOHDR. Median interval between start of CMT and EBRT was 18 weeks. Median EBRT dose was 36 Gy. EBRT technique was either intensity-modulated (11), three-dimensional (3), or two-dimensional (5). Functional outcome was assessed for patients alive ≥1 year after diagnosis (15) in terms of mild, moderate, or severe deficits. Results: Median follow-up was 33 months for survivors and 23 months for all patients. Two-year actuarial local control, event-free survival, disease-specific survival, and overall survival were 84%, 52%, 74%, and 62%, respectively. All patients who began EBRT ≤18 weeks after the start of CMT had their disease controlled locally. Five have mild deficits and 10 have no deficits. Conclusions: A reduced dose of 36-Gy EBRT after delayed GTR may maximize local control while minimizing long-term sequelae for very young children with RMS, but unresectable tumors (e.g., parameningeal) require higher doses. Normal-tissue-sparing techniques such as intensity-modulated radiation therapy and IOHDR are encouraged. Local control may be maximized when EBRT begins ≤18 weeks after initiation of CMT, but further study is warranted. Longer follow-up is required to determine the full extent of late effects. © 2006 Elsevier Inc. All rights reserved.
Keywords: adolescent; adult; cancer chemotherapy; cancer survival; child; clinical article; controlled study; child, preschool; functional assessment; survival rate; treatment failure; retrospective studies; three dimensional; cancer localization; intensity modulated radiation therapy; doxorubicin; cancer combination chemotherapy; multimodality cancer therapy; cancer patient; cancer radiotherapy; postoperative care; preoperative care; radiation dose; combined modality therapy; chemotherapy; drug megadose; follow up; cancer diagnosis; undifferentiated carcinoma; carboplatin; etoposide; antineoplastic combined chemotherapy protocols; radiotherapy; cyclophosphamide; melphalan; vincristine; age factors; radiation injury; oncology; bladder cancer; ifosfamide; childhood cancer; cancer survivor; irinotecan; prostate cancer; chronic disease; survival time; disease severity; evaluation; limb; infant; infant, newborn; tumors; medulloblastoma; newborn; radiation injuries; histochemistry; dactinomycin; brachytherapy; recurrent disease; cataract; radiation therapy; external beam radiotherapy; rhabdomyosarcoma; toxicity; drug dose regimen; trismus; embryonal carcinoma; peroperative care; rhabdomyosarcoma, embryonal; nephroblastoma; rhabdomyosarcoma, alveolar; autologous bone marrow transplantation; multimodality therapy; otitis media; gait disorder; trunk; walking difficulty; neck cancer; two dimensional; head cancer; infants; meninx; orbit cancer; very young children; intraoperative high-dose-rate brachytherapy (iohdr); parameningeal disorder
Journal Title: International Journal of Radiation Oncology, Biology, Physics
Volume: 65
Issue: 4
ISSN: 0360-3016
Publisher: Elsevier Inc.  
Date Published: 2006-07-15
Start Page: 1177
End Page: 1184
Language: English
DOI: 10.1016/j.ijrobp.2006.02.014
PUBMED: 16682130
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 13" - "Export Date: 4 June 2012" - "CODEN: IOBPD" - "Source: Scopus"
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Dev Raj Puri
    14 Puri
  2. Suzanne L Wolden
    560 Wolden
  3. Leonard H Wexler
    192 Wexler
  4. Paul Meyers
    311 Meyers
  5. John H Healey
    550 Healey