Histologic and clinical characteristics can guide staging evaluations for children and adolescents with rhabdomyosarcoma: a report from the Children's Oncology Group Soft Tissue Sarcoma Committee.
OBJECTIVE
To simplify the recommended staging evaluation by correlating tumor and clinical features with patterns of distant metastasis in newly diagnosed patients with embryonal rhabdomyosarcoma (ERMS) or alveolar rhabdomyosarcoma (ARMS).


METHODS
Patient data from the Intergroup Rhabdomyosarcoma Study Group and the Children's Oncology Group over two periods were analyzed: 1991 to 1997 and 1999 to 2004. We used recursive partitioning analyses to identify factors (including histology, age, regional nodal and distant metastatic status, tumor size, local invasiveness, and primary site) that divided patients into subsets with the most different rates of metastatic disease.


RESULTS
Of the 1,687 patients analyzed, 5.7% had lung metastases, 4.8% had bone involvement, and 6% had bone marrow (BM) involvement. Rhabdomyosarcoma (RMS) without local invasion (T1) had a low rate of metastasis for all distant sites, especially ERMS (0% bone, 0% BM). ARMS with local invasion (T2) had a higher rate of metastasis for all distant sites (13% lung, 18% bone, 23% BM). ERMS, T2 also had a higher rate of metastatic lung involvement (9%). The likelihood of bone or BM involvement increased in the presence of lung metastases (41% with, 6% without). Regional nodal metastases (N1) predicted a high rate of metastasis in all distant sites (14% lung, 14% bone, 18% BM). A staging algorithm was developed.


CONCLUSIONS
Staging studies in childhood RMS can be tailored to patients' presenting characteristics. Bone marrow aspirate and biopsy and bone scan are unnecessary in at least one third of patients with RMS.
The Soft Tissue Sarcoma Committee of the Children's Oncology Group, Monrovia, CA; Aaron R. Weiss, Maine Medical Center, Portland, ME; Elizabeth R. Lyden and James R. Anderson, University of Nebraska Medical Center, Omaha, NE; Douglas S. Hawkins, Seattle Children's Hospital, Fred Hutchinson Cancer Research Center, University of Washington, Seattle, WA; Sheri L. Spunt, St. Jude Children's Research Hospital and the University of Tennessee Health Science Center, Memphis, TN; David O. Walterhouse, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; Suzanne L. Wolden, Memorial Sloan-Kettering Cancer Center, New York, NY; David M. Parham, University of Oklahoma Health Sciences Center, Oklahoma City, OK; David A. Rodeberg, Children's Hospital of Pittsburgh, Pittsburgh, PA; Simon C. Kao, University of Iowa Hospitals and Clinics, Iowa City, IA; and Richard B. Womer, The Children's Hospital of Philadelphia, Philadelphia, PA.
Aaron R
AR
Weiss
Elizabeth R
ER
Lyden
James R
JR
Anderson
Douglas S
DS
Hawkins
Sheri L
SL
Spunt
David O
DO
Walterhouse
Suzanne L
SL
Wolden
David M
DM
Parham
David A
DA
Rodeberg
Simon C
SC
Kao
Richard B
RB
Womer
D000293:Adolescent; D000465:Algorithms; D019046:Bone Marrow Neoplasms / Q000556:secondary*; D001859:Bone Neoplasms / Q000556:secondary*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008175:Lung Neoplasms / Q000556:secondary*; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D018232:Rhabdomyosarcoma, Alveolar / Q000473:pathology*; D018233:Rhabdomyosarcoma, Embryonal / Q000473:pathology*