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Miscellaneous Malignant Germ Cell Neoplasms
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Germ cell tumor (GCT)
            • Synonyms

              • Malignant GCT
              • Primary GCT (PGCT)
              • Intracranial GCT
            • Definitions

              • Malignant tumor composed of undifferentiated epithelial cells
              • Morphologic, immunophenotypic homologues of gonadal/extraneuraxial GCTs but in CNS
              • 2 major types of GCTs
                • Germinoma (50-70%)
                • Nongerminomatous GCTs (NGGCTs)
                  • Embryonal carcinoma
                  • Yolk sac tumor (YST)
                  • Choriocarcinoma
                  • Teratoma
                  • Mixed GCT

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Angiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                • Special Immunohistochemical Markers

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Lo AC et al: Intracranial germ cell tumors in adolescents and young adults: a 40-year multi-institutional review of outcomes. Int J Radiat Oncol Biol Phys. 106(2):269-78, 2020
                                              2. Jiang T et al: Predictive factors of overall survival in primary intracranial pure choriocarcinoma. J Clin Neurosci. 61:93-101, 2019
                                              3. Morana G et al: T2*-based MR imaging (gradient echo or susceptibility-weighted imaging) in midline and off-midline intracranial germ cell tumors: a pilot study. Neuroradiology. 60(1):89-99, 2018
                                              4. Takada A et al: Long-term follow-up of intensive chemotherapy followed by reduced-dose and reduced-field irradiation for intracranial germ cell tumor. J Neurosurg Pediatr. 23(3):317-24, 2018
                                              5. Yoneoka Y et al: Third ventricle germ cell tumor originating from the infundibulum with rapidly expansive enlargement. Pediatr Neurosurg. 53(1):49-54, 2018
                                              6. Wu CC et al: MRI features of pediatric intracranial germ cell tumor subtypes. J Neurooncol. 134(1):221-30, 2017
                                              7. Rosenblum MK et al: Germ cell tumours. In Louis DN et al: WHO Classification of Tumours of the Central Nervous System.  IARC. 286-91, 2016
                                              8. Ben Nsir A et al: Primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle: a case report. Pediatr Neurosurg. 50(2):76-9, 2015
                                              9. Lai IC et al: Treatment results and prognostic factors for intracranial nongerminomatous germ cell tumors: single institute experience. Childs Nerv Syst. 31(5):683-91, 2015
                                              10. Ogiwara H et al: Apparent diffusion coefficient of intracranial germ cell tumors. J Neurooncol. 121(3):565-71, 2015
                                              11. Ogiwara H et al: Second-look surgery for intracranial germ cell tumors. Neurosurgery. 76(6):658-62, 2015
                                              12. Takami H et al: Human chorionic gonadotropin is expressed virtually in all intracranial germ cell tumors. J Neurooncol. 124(1):23-32, 2015
                                              13. Liu Z et al: Imaging characteristics of primary intracranial teratoma. Acta Radiol. 55(7):874-81, 2014
                                              14. Wang L et al: Novel somatic and germline mutations in intracranial germ cell tumours. Nature. 511(7508):241-5, 2014
                                              15. Zhao S et al: Intracranial pure yolk sac tumor in the anterior third ventricle of an adult: a case report. Exp Ther Med. 8(5):1471-2, 2014
                                              16. Sethi RV et al: Delayed diagnosis in children with intracranial germ cell tumors. J Pediatr. 163(5):1448-53, 2013
                                              17. Isaacs H: Fetal brain tumors: a review of 154 cases. Am J Perinatol. 26(6):453-66, 2009
                                              18. Sato K et al: Pathology of intracranial germ cell tumors. Prog Neurol Surg. 23:59-75, 2009
                                              19. Echevarría ME et al: Pediatric central nervous system germ cell tumors: a review. Oncologist. 13(6):690-9, 2008
                                              20. Vuillermet P et al: Simultaneous suprasellar and pineal germ cell tumors in five late stage adolescents: endocrinological studies and prolonged follow-up. J Pediatr Endocrinol Metab. 21(12):1169-78, 2008
                                              21. Kageji T et al: Successful neoadjuvant synchronous chemo- and radiotherapy for disseminated primary intracranial choriocarcinoma: case report. J Neurooncol. 83(2):199-204, 2007
                                              22. Cuccia V et al: Pure pineal germinomas: analysis of gender incidence. Acta Neurochir (Wien). 148(8):865-71; discussion 871, 2006
                                              23. Kamakura Y et al: C-kit gene mutation: common and widely distributed in intracranial germinomas. J Neurosurg. 104(3 Suppl):173-80, 2006
                                              24. Gömöri E et al: Cytogenetic profile of primary pituitary germinoma. J Neurooncol. 50(3):251-5, 2000
                                              25. Sano K: Pathogenesis of intracranial germ cell tumors reconsidered. J Neurosurg. 90(2):258-64, 1999
                                              26. Sawamura Y et al: Germ cell tumours of the central nervous system: treatment consideration based on 111 cases and their long-term clinical outcomes. Eur J Cancer. 34(1):104-10, 1998
                                              27. Salzman KL et al: Primary intracranial germ cell tumors: clinicopathologic review of 32 cases. Pediatr Pathol Lab Med. 17(5):713-27, 1997
                                              28. Fujimaki T et al: CT and MRI features of intracranial germ cell tumors. J Neurooncol. 19(3):217-26, 1994
                                              29. Smirniotopoulos JG et al: Pineal region masses: differential diagnosis. Radiographics. 12(3):577-96, 1992
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Germ cell tumor (GCT)
                                                        • Synonyms

                                                          • Malignant GCT
                                                          • Primary GCT (PGCT)
                                                          • Intracranial GCT
                                                        • Definitions

                                                          • Malignant tumor composed of undifferentiated epithelial cells
                                                          • Morphologic, immunophenotypic homologues of gonadal/extraneuraxial GCTs but in CNS
                                                          • 2 major types of GCTs
                                                            • Germinoma (50-70%)
                                                            • Nongerminomatous GCTs (NGGCTs)
                                                              • Embryonal carcinoma
                                                              • Yolk sac tumor (YST)
                                                              • Choriocarcinoma
                                                              • Teratoma
                                                              • Mixed GCT

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Angiographic Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            • Special Immunohistochemical Markers

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Lo AC et al: Intracranial germ cell tumors in adolescents and young adults: a 40-year multi-institutional review of outcomes. Int J Radiat Oncol Biol Phys. 106(2):269-78, 2020
                                                                                          2. Jiang T et al: Predictive factors of overall survival in primary intracranial pure choriocarcinoma. J Clin Neurosci. 61:93-101, 2019
                                                                                          3. Morana G et al: T2*-based MR imaging (gradient echo or susceptibility-weighted imaging) in midline and off-midline intracranial germ cell tumors: a pilot study. Neuroradiology. 60(1):89-99, 2018
                                                                                          4. Takada A et al: Long-term follow-up of intensive chemotherapy followed by reduced-dose and reduced-field irradiation for intracranial germ cell tumor. J Neurosurg Pediatr. 23(3):317-24, 2018
                                                                                          5. Yoneoka Y et al: Third ventricle germ cell tumor originating from the infundibulum with rapidly expansive enlargement. Pediatr Neurosurg. 53(1):49-54, 2018
                                                                                          6. Wu CC et al: MRI features of pediatric intracranial germ cell tumor subtypes. J Neurooncol. 134(1):221-30, 2017
                                                                                          7. Rosenblum MK et al: Germ cell tumours. In Louis DN et al: WHO Classification of Tumours of the Central Nervous System.  IARC. 286-91, 2016
                                                                                          8. Ben Nsir A et al: Primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle: a case report. Pediatr Neurosurg. 50(2):76-9, 2015
                                                                                          9. Lai IC et al: Treatment results and prognostic factors for intracranial nongerminomatous germ cell tumors: single institute experience. Childs Nerv Syst. 31(5):683-91, 2015
                                                                                          10. Ogiwara H et al: Apparent diffusion coefficient of intracranial germ cell tumors. J Neurooncol. 121(3):565-71, 2015
                                                                                          11. Ogiwara H et al: Second-look surgery for intracranial germ cell tumors. Neurosurgery. 76(6):658-62, 2015
                                                                                          12. Takami H et al: Human chorionic gonadotropin is expressed virtually in all intracranial germ cell tumors. J Neurooncol. 124(1):23-32, 2015
                                                                                          13. Liu Z et al: Imaging characteristics of primary intracranial teratoma. Acta Radiol. 55(7):874-81, 2014
                                                                                          14. Wang L et al: Novel somatic and germline mutations in intracranial germ cell tumours. Nature. 511(7508):241-5, 2014
                                                                                          15. Zhao S et al: Intracranial pure yolk sac tumor in the anterior third ventricle of an adult: a case report. Exp Ther Med. 8(5):1471-2, 2014
                                                                                          16. Sethi RV et al: Delayed diagnosis in children with intracranial germ cell tumors. J Pediatr. 163(5):1448-53, 2013
                                                                                          17. Isaacs H: Fetal brain tumors: a review of 154 cases. Am J Perinatol. 26(6):453-66, 2009
                                                                                          18. Sato K et al: Pathology of intracranial germ cell tumors. Prog Neurol Surg. 23:59-75, 2009
                                                                                          19. Echevarría ME et al: Pediatric central nervous system germ cell tumors: a review. Oncologist. 13(6):690-9, 2008
                                                                                          20. Vuillermet P et al: Simultaneous suprasellar and pineal germ cell tumors in five late stage adolescents: endocrinological studies and prolonged follow-up. J Pediatr Endocrinol Metab. 21(12):1169-78, 2008
                                                                                          21. Kageji T et al: Successful neoadjuvant synchronous chemo- and radiotherapy for disseminated primary intracranial choriocarcinoma: case report. J Neurooncol. 83(2):199-204, 2007
                                                                                          22. Cuccia V et al: Pure pineal germinomas: analysis of gender incidence. Acta Neurochir (Wien). 148(8):865-71; discussion 871, 2006
                                                                                          23. Kamakura Y et al: C-kit gene mutation: common and widely distributed in intracranial germinomas. J Neurosurg. 104(3 Suppl):173-80, 2006
                                                                                          24. Gömöri E et al: Cytogenetic profile of primary pituitary germinoma. J Neurooncol. 50(3):251-5, 2000
                                                                                          25. Sano K: Pathogenesis of intracranial germ cell tumors reconsidered. J Neurosurg. 90(2):258-64, 1999
                                                                                          26. Sawamura Y et al: Germ cell tumours of the central nervous system: treatment consideration based on 111 cases and their long-term clinical outcomes. Eur J Cancer. 34(1):104-10, 1998
                                                                                          27. Salzman KL et al: Primary intracranial germ cell tumors: clinicopathologic review of 32 cases. Pediatr Pathol Lab Med. 17(5):713-27, 1997
                                                                                          28. Fujimaki T et al: CT and MRI features of intracranial germ cell tumors. J Neurooncol. 19(3):217-26, 1994
                                                                                          29. Smirniotopoulos JG et al: Pineal region masses: differential diagnosis. Radiographics. 12(3):577-96, 1992