PMID- 21357792 OWN - NLM STAT- MEDLINE DCOM- 20110527 LR - 20220321 IS - 1527-7755 (Electronic) IS - 0732-183X (Print) IS - 0732-183X (Linking) VI - 29 IP - 10 DP - 2011 Apr 1 TI - Prognostic significance and tumor biology of regional lymph node disease in patients with rhabdomyosarcoma: a report from the Children's Oncology Group. PG - 1304-11 LID - 10.1200/JCO.2010.29.4611 [doi] AB - PURPOSE: Regional lymph node disease (RLND) is a component of the risk-based treatment stratification in rhabdomyosarcoma (RMS). The purpose of this study was to determine the contribution of RLND to prognosis for patients with RMS. PATIENTS AND METHODS: Patient characteristics and survival outcomes for patients enrolled onto Intergroup Rhabdomyosarcoma Study IV (N = 898, 1991 to 1997) were evaluated among the following three patient groups: nonmetastatic patients with clinical or pathologic negative nodes (N0, 696 patients); patients with clinical or pathologic positive nodes (N1, 125 patients); and patients with a single site of metastatic disease (77 patients). RESULTS: Outcomes for patients with nonmetastatic alveolar N0 RMS were significantly better than for patients with N1 RMS (5-year failure-free survival [FFS], 73% v 43%, respectively; 5-year overall survival [OS], 80% v 46%, respectively; P < .001). Patients with a single site of alveolar metastasis had even worse FFS and OS (23% FFS and OS, P = .01) when compared with patients with N1 RMS; however, the differences was not as large as the differences between patients with N0 RMS and N1 RMS. For embryonal RMS, there was no statistically significant difference in FFS or OS (P = .41 and P = .77, respectively) for patients with N1 versus N0 RMS. Gene array analysis of primary tumor specimens identified that genes associated with the immune system and antigen presentation were significantly increased in N1 versus N0 alveolar RMS. CONCLUSION: RLND alters prognosis for alveolar but not embryonal RMS. For patients with N1 disease and alveolar histology, outcomes were more similar to distant metastatic disease rather than local disease. Current data suggest that more aggressive therapy for patients with alveolar N1 RMS may be warranted. FAU - Rodeberg, David A AU - Rodeberg DA AD - Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA. rodebergd@ecu.edu FAU - Garcia-Henriquez, Norbert AU - Garcia-Henriquez N FAU - Lyden, Elizabeth R AU - Lyden ER FAU - Davicioni, Elai AU - Davicioni E FAU - Parham, David M AU - Parham DM FAU - Skapek, Stephen X AU - Skapek SX FAU - Hayes-Jordan, Andrea A AU - Hayes-Jordan AA FAU - Donaldson, Sarah S AU - Donaldson SS FAU - Brown, Kenneth L AU - Brown KL FAU - Triche, Timothy J AU - Triche TJ FAU - Meyer, William H AU - Meyer WH FAU - Hawkins, Douglas S AU - Hawkins DS LA - eng GR - U10 CA24507/CA/NCI NIH HHS/United States GR - UL1 RR025014/RR/NCRR NIH HHS/United States GR - U10 CA098543/CA/NCI NIH HHS/United States GR - U10 CA024507/CA/NCI NIH HHS/United States GR - U10 CA98543/CA/NCI NIH HHS/United States PT - Clinical Trial PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural DEP - 20110228 PL - United States TA - J Clin Oncol JT - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JID - 8309333 SB - IM MH - Canada MH - Chi-Square Distribution MH - Child MH - Child, Preschool MH - Disease-Free Survival MH - Female MH - Gene Expression Profiling MH - Gene Expression Regulation, Neoplastic MH - Humans MH - Immunohistochemistry MH - Infant MH - Kaplan-Meier Estimate MH - Lymph Nodes/*pathology MH - Lymphatic Metastasis MH - Male MH - Neoplasm Staging MH - Proportional Hazards Models MH - Rhabdomyosarcoma, Alveolar/genetics/mortality/*secondary/therapy MH - Rhabdomyosarcoma, Embryonal/genetics/mortality/*secondary/therapy MH - Risk Assessment MH - Risk Factors MH - Time Factors MH - Treatment Outcome MH - United States PMC - PMC3083998 COIS- Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article. EDAT- 2011/03/02 06:00 MHDA- 2011/05/28 06:00 PMCR- 2012/04/01 CRDT- 2011/03/02 06:00 PHST- 2011/03/02 06:00 [entrez] PHST- 2011/03/02 06:00 [pubmed] PHST- 2011/05/28 06:00 [medline] PHST- 2012/04/01 00:00 [pmc-release] AID - JCO.2010.29.4611 [pii] AID - 94611 [pii] AID - 10.1200/JCO.2010.29.4611 [doi] PST - ppublish SO - J Clin Oncol. 2011 Apr 1;29(10):1304-11. doi: 10.1200/JCO.2010.29.4611. Epub 2011 Feb 28.