Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: a report of Intergroup Rhabdomyosarcoma Studies I through IV, 1972 through 1997.
OBJECTIVE
Rhabdomyosarcoma (RMS) of the perineum or anus is a rare sarcoma of childhood with a poor prognosis. This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment guidelines.


METHODS
From 1972 through 1997, 71 eligible patients were treated and studied. The median patient age was 6 years. The majority (64%) were at an advanced stage (clinical group III and IV) at initial presentation and 50% had positive regional lymph node (LN) involvement.


RESULTS
The 5-year failure-free survival rate (FFS) for all patients was 45% and the overall survival rate (OS) was 49%. Characteristics that were associated with significantly improved survival rate were primary tumor size less than 5 cm, lower (less advanced) clinical group and stage, negative regional lymph node status, and age less than 10 years. When the extent of disease was controlled for in multivariate analysis, only age less than 10 predicted an improved outcome. The 5-year overall survival rate for patients less than 10 years of age was 71% versus 20% in older patients (P <.001). Histology (alveolar versus embryonal) and primary site (perineal versus anal) did not significantly affect outcome.


CONCLUSIONS
Because of the high incidence of regional LN involvement in these patients, a strategy of routine surgical evaluation of ilioinguinal lymph nodes in all patients with perineal or anal RMS is recommended.
Blakely
Martin L
ML
Soft-Tissue Sarcoma Committee of the Children's Oncology Group, Houston, Texas, USA.
Andrassy
Richard J
RJ
Raney
R Beverly
RB
Anderson
James R
JR
Wiener
Eugene S
ES
Rodeberg
David A
DA
Paidas
Charles N
CN
Lobe
Thom E
TE
Crist
William M
WM
D000293:Adolescent; D000367:Age Factors; D001005:Anus Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015993:Life Tables; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D009367:Neoplasm Staging; D010502:Perineum; D016016:Proportional Hazards Models; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery*; D016019:Survival Analysis; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide