Synovial Sarcoma in Pediatric Age Group: Single Center Experience

INTRODUCTION: Synovial sarcoma (SS) is generally observed in adults, however it is the most common non-rhabdomyosarcoma soft tissue sarcoma during childhood. In our study we reviewed pediatric patients with SS followed in our clinic retrospectively.
METHODS: Patients with SS diagnosed between 1993 and 2017 in Ankara Oncology Training and Research Hospital included into the study. Their files were reviewed retrospectively, clinical presentation, treatments and response of patients to treatment were investigated.
RESULTS: We analyzed data of 15 pediatric patients. Median age of patients was 14.5 (13 – 17) years, in 14 patients tumor was located in extremities. When we grouped patients based on IRS staging; five patients (33.3%) were in group I, eight patients (53.3%) were in group II, and two patients (13.3%) were in group III. Four (33%) of the 12 patients that in first remission relapse occurred; in three patients there was isolated lung relapse and in one patient relapse was from lung and external iliac lymph node. Patients were followed median 28 (5 – 85) months, three years event free survival was 53.6%, and overall survival was 73%. When we compared three years overall survival of patients based on IRS staging; 75% in IRS group I; 100 % in IRS group II; and 0% in IRS group III (p; <0.001).
DISCUSSION AND CONCLUSION: Prognosis of synovial sarcoma during childhood is very bad if surgery is not performed. In patients with remission lung is the most common region for relapse, and patients should be followed carefully for relapse if unspecified small pulmonary nodules are detected. Multi centric studies with more patients should be performed to get more valid information about our country.

Çocukluk Çaðý Döneminde Sinoviyal Sarkom: Tek Merkez Deneyimi

GÝRÝÞ ve AMAÇ: Sinoviyal sarkom (SS) çocukluk çaðýnýn en sýk rabdomyosarkom dýþý yumuþak doku sarkomu olmakla birlikte eriþkin yaþ grubunda daha sýk görülür. Çalýþmamýzda merkezimizde izlenen SS tanýlý çocuk hastalarýn klinik bulgu ve tedavi sonuçlarýný geriye dönük olarak incelemeyi amaçladýk.
YÖNTEM ve GEREÇLER: Ankara Onkoloji Eðitim ve Araþtýrma Hastanesi Çocuk Hematoloji Onkoloji Kliniði’nde 1993 ile 2017 yýllarý arasýnda SS tanýsý almýþ hastalar deðerlendirildi. Hastalarýn klinik özellikleri, aldýklarý tedaviler ve tedaviye yanýtlarý incelendi.
BULGULAR: Kliniðimizde izlenen 15 çocuk hastanýn verilerine ulaþýldý. Hastalarýn yaþ ortancasý 14,5 (13 – 17) yýldý, 14 hastada tümör ekstremite kökenliydi. Hastalar IRS gruplama sistemine göre deðerlendirildiðinde beþ hasta (%33,3) grup I, sekiz hasta (%53,3) grup II ve iki hasta (%13,3) grup III’tü. Ýlk remisyon saðlanan 12 hastanýn dördünde (%33) rölaps geliþti, üç hastada sadece akciðer, bir hastada akciðer ve eksternal iliak lenf düðümünde hastalýk tespit edildi. Hastalar ortanca 28 (5 – 85) ay izlendi, üç yýllýk olaysýz sað kalým %53,6, üç yýllýk genel sað kalým %73 olarak hesaplandý. Hastalarýn IRS gruplarýna göre üç yýllýk genel sað kalýmlarý karþýlaþtýrýldýðýnda IRS grup I; %75, IRS grup II; %100 ve IRS grup III’te %0’dý (p; <0,001).
TARTIÞMA ve SONUÇ: Çocukluk çaðýnda SS yeterli cerrahi yapýlamadýðýnda prognozu oldukça kötü bir tümördür. Remisyon saðlanan hastalarda bile akciðer en sýk relaps yeridir, kontrollerde küçük akciðer nodülleri tespit edildiðinde akciðer relapsý açýsýndan dikkatli izlenmelidir. Ülkemizde yapýlacak çok merkezli çalýþmalarla çocukluk çaðý SS hakkýnda genel geçerliliði olan veriler elde edilebilir.