title: Malignant extra-cranial germ cell tumors in children and adolescents. Results following the guidelines of SFOP/SFCE 95 Protocol.
abstract: Between September 1995 and December 2010, 99 new consecutive assessable patients with extra-cranial MGCT were treated according to SFOP/SFCE TGM95 Protocol. A "watch and wait" strategy for completely resected stage I-II was observed in cases with preoperative high tumor markers levels. Metastatic disease or alpha fetoprotein levels > 15 000 ng/ml cases were treated by VIP chemotherapy (etoposide, ifosfamide and CDDP) 4-6-courses. All other cases were treated by VBP (vinblastine, bleomycin, and CDDP) 3-5 courses. Median age for the whole group was 11.1 (r: 0-17) years. Males: 49, females: 50. Stage I: 19 patients, stage II: 16, stage III: 31 and stage IV: 3. Gonadal disease occurred in 77 cases. Of 21 completely resected stage I-II patients with MGCT who did not receive chemotherapy after surgery, 6 presented disease progression and were successfully treated by chemotherapy and remained disease-free. There were no significant differences in outcome according to age, gender, initial site, staging, and histological variant or high levels of alpha-fetoprotein. Initial non-responsiveness to VIP chemotherapy was the only significant unfavorable prognostic feature. With a median follow-up of 64 (r: 5-204) months, at 10 years EFS and OS estimates for the whole group were 0.82 (SE = 0.05) and 0.90 (SE = 0.03) respectively. Therapy results of MGCT treated with the SFOP/SFCE 95 strategy were excellent. Initial non-response to front line chemotherapy was the only significant adverse prognostic feature. The "watch and wait" strategy for completely resected disease with initial positive markers proved to be safe with optimal outcome.
authors.affiliation: Servicio de Hematología y Oncología, Hospital de Pediatría SAMIC Prof. Dr. Juan P Garrahan, Buenos Aires, Argentina. E-mail: pedro.zubizarreta@hotmail.com.
authors.forename: Pedro
authors.initials: P
authors.lastname: Zubizarreta
authors.affiliation: Servicio de Hematología y Oncología, Hospital de Pediatría SAMIC Prof. Dr. Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Ana
authors.initials: A
authors.lastname: Rossa
authors.affiliation: Servicio de Cirugía, 3Servicio de Endocrinología, Hospital de Pediatría SAMIC Prof. Dr. Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Marcela
authors.initials: M
authors.lastname: Bailez
authors.affiliation: Servicio de Endocrinología, Hospital de Pediatría SAMIC Prof. Dr. Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Silvia
authors.initials: S
authors.lastname: Gil
authors.affiliation: Servicio de Hematología y Oncología, Hospital de Pediatría SAMIC Prof. Dr. Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Adriana
authors.initials: A
authors.lastname: Rose
authors.affiliation: Servicio de Hematología y Oncología, Hospital de Pediatría SAMIC Prof. Dr. Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Walter
authors.initials: W
authors.lastname: Cacciavillano
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D018309:Neoplasms, Gonadal Tissue / Q000401:mortality / Q000473:pathology / Q000628:therapy; D010051:Ovarian Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D017410:Practice Guidelines as Topic; D011379:Prognosis; D011446:Prospective Studies; D015203:Reproducibility of Results; D018570:Risk Assessment; D012307:Risk Factors; D012445:Sacrococcygeal Region; D017678:Sex Distribution; D013736:Testicular Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D013997:Time Factors; D057832:Watchful Waiting / Q000379:methods
keywords: adolescence; childhood; malignant germ cell tumors