Synovial sarcoma in children and adolescents: the St Jude Children's Research Hospital experience.

Abstract

PURPOSE AND METHODS:

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).

CONCLUSION:

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.

• Authors: A S Pappo, J Fontanesi, X Luo, B N Rao, D M Parham, C Hurwitz, L Avery, C B Pratt
• Journal: Journal of clinical oncology : official journal of the American Society of Clinical Oncology (J Clin Oncol) Vol. 12 Issue 11 Pg. 2360-6 (Nov 1994) ISSN: 0732-183X [Print] United States
• PMID: 7964951 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Topics

• Adolescent
• Adult
• Amputation, Surgical
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Child
• Child, Preschool
• Combined Modality Therapy
• Female
• Hospitals, Pediatric
• Humans
• Male
• Neoplasm Invasiveness
• Neoplasm Recurrence, Local
• Prognosis
• Sarcoma, Synovial (mortality, pathology, physiopathology, therapy)
• Survival Analysis