Synovial sarcoma in children and adolescents: the St Jude Children's Research Hospital experience.
OBJECTIVE
We reviewed the clinical records and pathologic findings of 37 children and adolescents with synovial sarcoma treated at our institution over a 30-year period to evaluate the prognostic significance of tumor size, invasiveness, histology, and other features.


RESULTS
The 20 male and 17 female patients with synovial sarcoma had a median age of 13.7 years at diagnosis. Primary tumor sites were the extremities (n = 27), trunk (n = 8), and head and neck (n = 2). Disease stage (clinical group) was as follows: group I, n = 21; group II, n = 7; group III, n = 4; and group IV, n = 5. Nineteen patients had invasive (T2) lesions, 20 had tumors more than 5 cm in diameter, and 14 had histologic grade 3 lesions. The estimated 5-year survival rate (+/- SE) for patients with group I or II disease was 80% +/- 9%, compared with 17% +/- 15% for those with group III or IV tumors (P = .0003). An exact log-rank test, adjusted for clinical group, showed that tumor invasiveness and grade independently predicted overall and progression-free survival (P < .05); tumor size was significantly correlated with progression-free survival. A borderline significant relationship with overall survival was found for both tumor size and histologic subtype (P = .09).


CONCLUSIONS
A controlled trial of adjuvant chemotherapy is merited in children with resected synovial sarcoma (clinical group I or II) who present with unfavorable clinicopathologic features such as large, invasive, or grade 3 lesions. Children with unresected or metastatic disease fare poorly despite multimodality therapy and require novel treatment approaches.
Department of Hematology-Oncology, St Jude Children's Research Hospital, Memphis, TN 38101-0318.
A S
AS
Pappo
J
J
Fontanesi
X
X
Luo
B N
BN
Rao
D M
DM
Parham
C
C
Hurwitz
L
L
Avery
C B
CB
Pratt
D000293:Adolescent; D000328:Adult; D000671:Amputation, Surgical; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D008297:Male; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D013584:Sarcoma, Synovial / Q000401:mortality / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy; D016019:Survival Analysis