Abstract
BACKGROUND
Outcomes of patients with relapsed CNS NGGCTs are extremely poor compared to patients with relapsed germinomas. The rarity of these tumors and paucity of prospective clinical trials make it difficult to establish a standard treatment approach.
METHODS
A review of clinical trials that included patients with relapsed CNS NGGCTs was performed.
RESULTS
Seventy-four patients reported in one prospective clinical trial and three retrospective studies were identified; only 14 patients were reported as long-term survivors (19%). Of the 14 survivors, eight relapsed within 24 months of initial diagnosis; five of 14 survivors had elevated serum/ cerebrospinal fluid (CSF) alpha-fetoprotein (AFP) at primary diagnosis; none had elevated serum/CSF AFP at relapse. Of the 14 survivors, seven had elevated serum/CSF beta-human chorionic gonadotropin (HCGβ) at initial diagnosis or relapse. Eleven received radiotherapy at some time following relapse (craniospinal irradiation=9, focal radiotherapy=2). Twelve received high-dose chemotherapy (HDCx) followed by autologous hematopoietic cell rescue (AuHCR); of six with data, all achieved complete or continuing complete (two after surgical resection) remission (CR) prior to HDCx. Post-transplant, eleven out of twelve (one with stable disease) continued without residual or recurrent disease.
CONCLUSION
Serum/ CSF AFP status at relapse, achieving CR prior and post-HDCx, receiving radiotherapy and HDC may have more prognostic value compared to the time of relapse, tumor markers at initial diagnosis and achieving GTR prior to consolidation therapy. Future trials need to better stratify relapsed patients on the basis of AFP status prior to relapse and receiving radiotherapy and HDCx.