Kyung Taek Hong1, Jung Woo Han2, Enrica Ee Kar Tan3, Tai-Tong Wong4, Joo-Young Kim5, Hiroshi Fuji6, Hwa Kyung Byun7, Kyung-Nam Koh8, Ru Xin Wong9, Hsin-Lun Lee10, Hong In Yoon7, Joo Ho Lee11, Ji Hoon Phi12, Seung-Ki Kim12, Dong-Seok Kim13, Chuhl Joo Lyu2, Jung Yoon Choi1, Hyoung Jin Kang1, Yi-Wei Chen14, Yi-Yen Lee15, Ho Joon Im8, Young-Shin Ra16, Seung Do Ahn17, Sharon Yin Yee Low18, Wen Shen Looi9, Hyeon Jin Park19, Yang-Gun Suh20, Chang-Ok Suh21, Kyu-Chang Wang22. 1. Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. 2. Division of Pediatric Hematology and Oncology, Department of Pediatrics, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea. 3. Department of Pediatric Subspecialties, Pediatric Hematology/Oncology Service, KK Women's and Children's Hospital, Singapore, Singapore. enrica.tan.e.k@singhealth.com.sg. 4. Pediatric Brain Tumor Program, Taipei Cancer Center, and Taipei Medical University Hospital, Taipei Medical University, Taipei, Taiwan. ttwong99@gmail.com. 5. Department of Radiation Oncology, Research Institute and Hospital, National Cancer Center, Goyang, Korea. jooyoungcasa@ncc.re.kr. 6. Department of Radiation Oncology, National Center for Child Health and Development, Tokyo, Japan. 7. Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea. 8. Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. 9. Department of Radiation Oncology, National Cancer Centre, Singapore, Singapore. 10. Department of Radiation Oncology, Taipei Medical University Hospital, Taipei Medical University, Taipei, Taiwan. 11. Department of Radiation Oncology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. 12. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. 13. Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea. 14. Division of Radiation Oncology, Department of Oncology, Taipei Veterans General Hospital, Taipei, Taiwan. 15. Division of Pediatric Neurosurgery, Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan. 16. Division of Pediatric Neurosurgery, Department of Neurosurgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. 17. Department of Radiation Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. 18. National Neuroscience Institute, Singapore, Singapore. 19. Center for Pediatric Oncology, National Cancer Center, Goyang, Korea. 20. Department of Radiation Oncology, Research Institute and Hospital, National Cancer Center, Goyang, Korea. 21. Department of Radiation Oncology, CHA Bundang Medical Center, CHA University, Seongnam, South Korea. 22. Neuro-Oncology Clinic, National Cancer Center, Goyang, Korea.
Abstract
PURPOSE: Non-germinomatous germ cell tumors (NGGCTs) are rare pediatric conditions. This multicenter study using Asian multinational patient data investigated treatment outcomes and prognostic factors for NGGCTs. METHODS: Medical records of 251 patients with NGGCTs treated from 1995 to 2015 were retrospectively analyzed from participating centers in Asian countries (Korea, Taiwan, Singapore, and Japan). RESULTS: The median follow up was 8.5 years (95% CI 7.8-9.9). In the total cohort, 5-year event-free survival (EFS) and overall survival (OS) rates were 78.2% and 85.4%, respectively. In 17.9% of the patients, diagnosis was determined by tumor markers alone (alpha-fetoprotein ≥ 10 ng/mL (Korea) or > 25 ng/mL (Taiwan and Singapore), and/or β-human chorionic gonadotropin (β-hCG) ≥ 50 mIU/mL). Patients with immature teratomas and mature teratomas comprised 12.0% and 8.4%, respectively. The 5-year EFS rate was higher in patients with histologically confirmed germinoma with elevated β-hCG (n = 28) than those in patients with malignant NGGCTs (n = 127). Among malignant NGGCTs, patients with choriocarcinoma showed the highest 5-year OS of 87.6%, while yolk sac tumors showed the lowest OS (68.8%). For malignant NGGCT subgroups, an increase in serum β-hCG levels by 100 mIU/mL was identified as a significant prognostic factor associated with the EFS and OS. CONCLUSION: Our result shows excellent survival outcomes of overall CNS NGGCT. However, treatment outcome varied widely across the histopathologic subgroup of NGGCT. Hence, this study suggests the necessity for accurate diagnosis by surgical biopsy and further optimization of diagnosis and treatment according to the histopathology of NGGCTs. Future clinical trials should be designed for individualized treatments for different NGGCTs subsets.
PURPOSE: Non-germinomatous germ cell tumors (NGGCTs) are rare pediatric conditions. This multicenter study using Asian multinational patient data investigated treatment outcomes and prognostic factors for NGGCTs. METHODS: Medical records of 251 patients with NGGCTs treated from 1995 to 2015 were retrospectively analyzed from participating centers in Asian countries (Korea, Taiwan, Singapore, and Japan). RESULTS: The median follow up was 8.5 years (95% CI 7.8-9.9). In the total cohort, 5-year event-free survival (EFS) and overall survival (OS) rates were 78.2% and 85.4%, respectively. In 17.9% of the patients, diagnosis was determined by tumor markers alone (alpha-fetoprotein ≥ 10 ng/mL (Korea) or > 25 ng/mL (Taiwan and Singapore), and/or β-human chorionic gonadotropin (β-hCG) ≥ 50 mIU/mL). Patients with immature teratomas and mature teratomas comprised 12.0% and 8.4%, respectively. The 5-year EFS rate was higher in patients with histologically confirmed germinoma with elevated β-hCG (n = 28) than those in patients with malignant NGGCTs (n = 127). Among malignant NGGCTs, patients with choriocarcinoma showed the highest 5-year OS of 87.6%, while yolk sac tumors showed the lowest OS (68.8%). For malignant NGGCT subgroups, an increase in serum β-hCG levels by 100 mIU/mL was identified as a significant prognostic factor associated with the EFS and OS. CONCLUSION: Our result shows excellent survival outcomes of overall CNS NGGCT. However, treatment outcome varied widely across the histopathologic subgroup of NGGCT. Hence, this study suggests the necessity for accurate diagnosis by surgical biopsy and further optimization of diagnosis and treatment according to the histopathology of NGGCTs. Future clinical trials should be designed for individualized treatments for different NGGCTs subsets.
Authors: Stewart Goldman; Eric Bouffet; Paul G Fisher; Jeffrey C Allen; Patricia L Robertson; Paul J Chuba; Bernadine Donahue; Cynthia S Kretschmar; Tianni Zhou; Allen B Buxton; Ian F Pollack Journal: J Clin Oncol Date: 2015-06-22 Impact factor: 44.544
Authors: S E Schild; M G Haddock; B W Scheithauer; L B Marks; M G Norman; P C Burger; W W Wong; M K Lyons; P J Schomberg Journal: Int J Radiat Oncol Biol Phys Date: 1996-10-01 Impact factor: 7.038
Authors: H J Hoffman; H Otsubo; E B Hendrick; R P Humphreys; J M Drake; L E Becker; M Greenberg; D Jenkin Journal: J Neurosurg Date: 1991-04 Impact factor: 5.115
Authors: David N Louis; Arie Perry; Pieter Wesseling; Daniel J Brat; Ian A Cree; Dominique Figarella-Branger; Cynthia Hawkins; H K Ng; Stefan M Pfister; Guido Reifenberger; Riccardo Soffietti; Andreas von Deimling; David W Ellison Journal: Neuro Oncol Date: 2021-08-02 Impact factor: 13.029