Prognostic factors for children and adolescents with surgically resected nonrhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children's Research Hospital.
OBJECTIVE
The rarity and heterogeneity of pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) has precluded meaningful analysis of prognostic factors associated with surgically resected disease. To define a population of patients at high risk of treatment failure who might benefit from adjuvant therapies, we evaluated the relationship between various clinicopathologic factors and clinical outcome of children and adolescents with resected NRSTS over a 27-year period at our institution.


METHODS
We analyzed the records of 121 consecutive patients with NRSTS who underwent surgical resection between August 1969 and December 1996. Demographic data, tumor characteristics, treatment, and outcomes were recorded. Univariate and multivariate analyses of prognostic factors for survival, event-free survival (EFS), and local and distant recurrence were performed.


RESULTS
At a median follow-up of 9.2 years, 5-year survival and EFS rates for the entire cohort were 89% +/- 3% and 77% +/- 4%, respectively. In univariate models, positive surgical margins (P =.004), tumor size > or = 5 cm (P <.001), invasivene (P =.002), high grade (P =.028), and intra-abdominal primary tumor site (P =.055) adversely affected EFS. All of these factors except invasiveness remained prognostic of EFS and survival in multivariate models. Positive surgical margins (P =.003), intra-abdominal primary tumor site (P =.028), and the omission of radiation therapy (P =.043) predicted local recurrence, whereas tumor size > or = 5 cm (P <.001), invasiveness (P <.001), and high grade (P =.004) predicted distant recurrence.


CONCLUSIONS
In this largest single-institution analysis of pediatric patients with surgically resected NRSTS, we identified clinicopathologic features predictive of poor outcome. These variables should be prospectively evaluated as risk-adapted therapies are developed.
Spunt
S L
SL
Departments of Hematology/Oncology, Biostatistics and Epidemiology, Surgery, Radiation Oncology, and Pathology, St. Jude Children's Research Hospital, Memphis, TN 38105-2794, USA. sheri.spunt@stjude.org
Poquette
C A
CA
Hurt
Y S
YS
Cain
A M
AM
Rao
B N
BN
Merchant
T E
TE
Jenkins
J J
JJ
Santana
V M
VM
Pratt
C B
CB
Pappo
A S
AS
D000293:Adolescent; D000328:Adult; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D017063:Outcome Assessment, Health Care; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012509:Sarcoma / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality / Q000601:surgery; D016896:Treatment Outcome