Synovial sarcoma in patients under 20 years of age: A multicenter study with a minimum follow-up of 10 years

Abstract

Purpose Synovial sarcoma (SS) is an aggressive softtissue tumor noted for late local recurrence and metastasis. This study investigates the long-term outcome of SS in patients of pediatric age and evaluates potential prognostic factors for SS. Methods We performed a retrospective review of 13 SS cases in patients younger than 20 years at the time of diagnosis who had a minimum follow-up of 10 years. The mean follow-up for living patients (n = 8) was 20.1 years (12.1-27.6) and for nonsurvivors (n = 5) 4.9 years (range: 2.6-9.3). Nine patients had unplanned excisions (69%), of which 6 (67%) were performed prior to their referral. Re-excisions were necessary in all 13 patients. The factors sex, tumor site, tumor size, tumor grade, histological subtype, fusion type, and type of treatment were evaluated for their prognostic value. Results Only 2 patients (15%) met the criteria of adequate tumor treatment. Overall, the 5-and 10-year survival rates were 77 and 61%, respectively. The mean time until a local recurrence (n = 5) was 3.2 years (range: 0.7-10.2), while there was a mean time of 2.1 years until the occurrence of late metastases (n = 5; range: 0.8-4.8). A high tumor grade and having a tumor in the trunk were adverse factors in terms of overall, local recurrence-free, and metastasis-free survival. Patients with wide resections or amputations had fewer local recurrences than patients with marginal or intralesional resections. Conclusion Inadequate primary excision of SS results in incomplete excision in the majority of cases. The tumor site, size, and histological grade should be considered when determining a risk-adapted treatment for SS, and wide surgical excision is the surgical intervention of choice. While local recurrence and late metastases appear to occur after a shorter time period in pediatric patients than in adults, in view of the tendency for late recurrence and metastasis with SS, follow-up should be at least 10 years.

Prognosis of T1 synovial sarcoma depends upon surgery by oncologic surgeons

“…The observation could be also explained by the slower growth rate in early stage T1 tumors compared to larger ones. In concordance with several previous reports [8,10,15,16,25], we also found local recurrence predicted metastasis. Four of six (67%) patients with local recurrence developed metastasis whereas only 1 of 57 (2%) patients without local recurrence developed metastasis at some point during their disease (P < 0.001).…”

Targeting regulation of cyclin dependent kinase 9 as a novel therapeutic strategy in synovial sarcoma

“…Synovial sarcoma is a high‐grade, aggressive malignant mesenchymal neoplasm that accounts for 5–10% of soft tissue sarcomas in childhood and adolescence . A specific balanced reciprocal translocation, t(X;18) (p11.2; q11.2), has been found in over 90% of human synovial sarcomas, involving the SS18 gene on chromosome 18 and the SSX1, SSX2, or SSX4 genes on chromosome X at Xp11.2, resulting in the generation of a SS18‐SSXfusion oncoprotein .…”

A sarcoma synoviale kezelési lehetőségei

“…Több munkacsoport vizsgálta az SS-ben szenvedő pá-ciensek túlélését befolyásoló prognosztikai faktorokat, amelyek közül az 5 cm vagy annál kisebb daganatméret korrelált a hosszabb túléléssel [4,5,6,7,8,9,10,11]. Wushou és Miao 26 vizsgálat (n = 93) metaanalízisében tekintették át a fej-nyak régióban elhelyezkedő SS prognosztikai faktorait.…”

“…Tömegspektrometriával felfedezett szecernin-1 fehérje expressziója SS-ben jó prognosztikai markernek ígérkezik a teljes és az áttétmentes túlélés tekintetében [18]. Mivel évekkel később kiújulhat az SS, ezért több mint 10 éves utánkövetés javasolt [10,11].…”