Synovial sarcoma of the extremities and trunk: A long lasting disease

Abstract

10073 Background: Synovial sarcoma (SS) of an extremity or trunk is a relatively rare type of soft tissue sarcoma. SS most commonly affects adolescents and young adults. SS usually originates at an extremity, carries a t(X;18)(p11;q11) translocation, and approached by limb sparing surgery, radiation therapy, and chemotherapy. Methods: A retrospective analysis of clinical and histopathological data of 73 patients with proven SS, treated at the National Unit of Orthopedic Oncology, from January 1991 through December 2004 was performed. Results: At a median follow-up time of 6 years, a local-recurrence was observed in 17.8% of the patients, while systemic recurrence in 35.6% (local-only in 6.8 %, systemic-only in 24.6 %, and combined in 11%). Accumulation of events of local and systemic recurrence following a limb sparing approach, did not reach a plateau even after 192 months from diagnosis. The 10-year local recurrence free survival (LRFS), the 10-year systemic recurrence-free survival (SRFS), and the 10-year overall survival (OS) were 78%, 68%, and 61%, respectively. The median SRFS time was 180 months, while the median LRFS and OS have not been reached yet. LRFS was significantly better for ILP treated patients; SRFS was influenced by a shorter delay in diagnosis. Conclusions: The practical aspects of our observations are the need for long-term follow-up for diagnosis of recurrence, the fact that not all local or distant recurrences are necessarily associated with shortening of overall survival, and the important role of induction ILP with TNF in cases of extremity SS. No significant financial relationships to disclose.