TY - JOUR
T1 - Secreting Germ Cell Tumors of the Central Nervous System: A Long-Term Follow-up Experience.
AU - Biassoni, Veronica
AU - Schiavello, Elisabetta
AU - Gandola, Lorenza
AU - Pecori, Emilia
AU - Poggi, Geraldina
AU - Spreafico, Filippo
AU - Terenziani, Monica
AU - Meazza, Cristina
AU - Podda, Marta
AU - Ferrari, Andrea
AU - Luksch, Roberto
AU - Casanova, Michela
AU - Puma, Nadia
AU - Chiaravalli, Stefano
AU - Bergamaschi, Luca
AU - Cefalo, Graziella
AU - Simonetti, Fabio
AU - Gattuso, Giovanna
AU - Seregni, Ettore Cesare
AU - Pallotti, Federica
AU - Gianno, Francesca
AU - Diletto, Barbara
AU - Barretta, Francesco
AU - Massimino, Maura
PY - 2020/9/1
Y1 - 2020/9/1
N2 - INTRODUCTION: Due to the rarity of nongerminomatous germ cell tumors (NGGCT) with non-standard treatment as yet, we report retrospectively our 30 year experience with chemotherapy followed by craniospinal irradiation (CSI), plus a boost of whole ventricular irradiation (WVI)/tumor bed (TB), tailored to pre-radiation chemotherapy response. METHODS: Between 1988 and 2016, 28 patients received four cycles of PEB (cisplatin/etoposide/bleomycin), then CSI, and two further PEB cycles. Between 1988 and1994, CSI was 25.5 Gy for patients in complete remission (CR), 30 Gy if in partial remission (PR) or metastatic, with a boost to TB up to 45-54 Gy. In the period of 1995-2010, the boost included WVI and any extra-ventricular tumor sites up to 45 Gy. After 2010, CSI was reduced to 25.5 Gy for all non-metastatic patients, and a boost was given only to TB up to 40.5/45.5 Gy, depending on patients' CR/PR status. After 2003, patients with alfafetoprotein (αFP) textgreater 1000 ng/mL received intensified treatment, also including autologous stem cell transplantation. RESULTS: Among 28 patients (23 males; median age 12 years, 6 metastatic), 25 responded to PEB, and three progressed (PD) after one to four cycles; 26 received radiotherapy obtaining 13 CR, 7 PR and 5 stable disease (SD), 1 PD; 6 (21 died (5 for disease, 1 for pneumonia while in CR). Five-year overall survival (OS) and progression-free survival (PFS) were both 81 10 year OS and PFS 816 respectively (median follow-up 11 years). CONCLUSIONS: Survival for children with NGGCT, independently from disease extent, was encouraging. Further studies should elucidate which patients could benefit from reduced volume and dose irradiation.
AB - INTRODUCTION: Due to the rarity of nongerminomatous germ cell tumors (NGGCT) with non-standard treatment as yet, we report retrospectively our 30 year experience with chemotherapy followed by craniospinal irradiation (CSI), plus a boost of whole ventricular irradiation (WVI)/tumor bed (TB), tailored to pre-radiation chemotherapy response. METHODS: Between 1988 and 2016, 28 patients received four cycles of PEB (cisplatin/etoposide/bleomycin), then CSI, and two further PEB cycles. Between 1988 and1994, CSI was 25.5 Gy for patients in complete remission (CR), 30 Gy if in partial remission (PR) or metastatic, with a boost to TB up to 45-54 Gy. In the period of 1995-2010, the boost included WVI and any extra-ventricular tumor sites up to 45 Gy. After 2010, CSI was reduced to 25.5 Gy for all non-metastatic patients, and a boost was given only to TB up to 40.5/45.5 Gy, depending on patients' CR/PR status. After 2003, patients with alfafetoprotein (αFP) textgreater 1000 ng/mL received intensified treatment, also including autologous stem cell transplantation. RESULTS: Among 28 patients (23 males; median age 12 years, 6 metastatic), 25 responded to PEB, and three progressed (PD) after one to four cycles; 26 received radiotherapy obtaining 13 CR, 7 PR and 5 stable disease (SD), 1 PD; 6 (21 died (5 for disease, 1 for pneumonia while in CR). Five-year overall survival (OS) and progression-free survival (PFS) were both 81 10 year OS and PFS 816 respectively (median follow-up 11 years). CONCLUSIONS: Survival for children with NGGCT, independently from disease extent, was encouraging. Further studies should elucidate which patients could benefit from reduced volume and dose irradiation.
KW - chemotherapy
KW - radiotherapy
KW - CNS germ cell tumors
KW - craniospinal irradiation
KW - late effects
KW - NGGCT
U2 - 10.3390/cancers12092688
DO - 10.3390/cancers12092688
M3 - Article
VL - 12
JO - Cancers
JF - Cancers
SN - 2072-6694
IS - 9
ER -