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Diagnosis
Pediatric Neuroradiology
Brain
Pathology-Based Diagnoses
Neoplasms
Choroid Plexus Tumors
Choroid Plexus Carcinoma
Choroid Plexus Carcinoma
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Choroid plexus carcinoma (CPC)
                • Choroid plexus tumor (CPT)
                • Choroid plexus papilloma (CPP)

              • Definitions

                • Malignant tumor originating from epithelium of choroid plexus

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic Findings

                        • Nuclear Medicine Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Staging, Grading, & Classification

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      • Immunochemistry

                                        • Genetic Alterations

                                          CLINICAL ISSUES

                                          • Presentation

                                            • Demographics

                                              • Natural History & Prognosis

                                                • Treatment

                                                  DIAGNOSTIC CHECKLIST

                                                  • Consider

                                                    • Image Interpretation Pearls

                                                      Selected References

                                                      1. Crawford JR et al: Perinatal (fetal and neonatal) choroid plexus tumors: a review. Childs Nerv Syst. 35(6):937-44, 2019
                                                      2. Sasaki T et al: Roles of the apparent diffusion coefficient and tumor volume in predicting tumor grade in patients with choroid plexus tumors. Neuroradiology. 60(5):479-86, 2018
                                                      3. Siegfried A et al: A French retrospective study on clinical outcome in 102 choroid plexus tumors in children. J Neurooncol. 135(1):151-60, 2017
                                                      4. Thomas C et al: Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups. Neuro Oncol. 18(6):790-6, 2016
                                                      5. Cannon DM et al: Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 121(1):151-7, 2015
                                                      6. Dangouloff-Ros V et al: Choroid plexus neoplasms: toward a distinction between carcinoma and papilloma using arterial spin-labeling. AJNR Am J Neuroradiol. 36(9):1786-90, 2015
                                                      7. Dudley RW et al: Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neurooncol. 121(1):201-7, 2015
                                                      8. Merino DM et al: Molecular characterization of choroid plexus tumors reveals novel clinically relevant subgroups. Clin Cancer Res. 21(1):184-92, 2015
                                                      9. Zaky W et al: Choroid plexus carcinoma in children: the head start experience. Pediatr Blood Cancer. 62(5):784-9, 2015
                                                      10. Ruland V et al: Choroid plexus carcinomas are characterized by complex chromosomal alterations related to patient age and prognosis. Genes Chromosomes Cancer. 53(5):373-80, 2014
                                                      11. Korchi AM et al: Pseudoprogression after proton beam irradiation for a choroid plexus carcinoma in pediatric patient: MRI and PET imaging patterns. Childs Nerv Syst. 29(3):509-12, 2013
                                                      12. Gonzalez KD et al: Beyond Li Fraumeni syndrome: clinical characteristics of families with p53 germline mutations. J Clin Oncol. 27(8):1250-6, 2009
                                                      13. Gopal P et al: Choroid plexus carcinoma. Arch Pathol Lab Med. 132(8):1350-4, 2008
                                                      14. Wrede B et al: Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol. 85(3):345-51, 2007
                                                      15. Krieger MD et al: Differentiation of choroid plexus tumors by advanced magnetic resonance spectroscopy. Neurosurg Focus. 18(6A):E4, 2005
                                                      16. Levy ML et al: Choroid plexus tumors in children: significance of stromal invasion. Neurosurgery. 48(2):303-9, 2001
                                                      17. Malkin D et al: Tissue-specific expression of SV40 in tumors associated with the Li-Fraumeni syndrome. Oncogene. 20(33):4441-9, 2001
                                                      18. Poussaint TY: Magnetic resonance imaging of pediatric brain tumors: state of the art. Top Magn Reson Imaging. 12(6):411-33, 2001
                                                      19. Wolff JE et al: Detection of choroid plexus carcinoma with Tc-99m sestamibi: case report and review of the literature. Med Pediatr Oncol. 36(2):323-5, 2001
                                                      20. Sévenet N et al: Constitutional mutations of the hSNF5/INI1 gene predispose to a variety of cancers. Am J Hum Genet. 65(5):1342-8, 1999
                                                      Related Anatomy
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                                                      Related Differential Diagnoses
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                                                      References
                                                      Tables

                                                      Tables

                                                      KEY FACTS

                                                      • Terminology

                                                        • Imaging

                                                          • Top Differential Diagnoses

                                                            • Pathology

                                                              • Clinical Issues

                                                                • Diagnostic Checklist

                                                                  TERMINOLOGY

                                                                  • Abbreviations

                                                                    • Choroid plexus carcinoma (CPC)
                                                                    • Choroid plexus tumor (CPT)
                                                                    • Choroid plexus papilloma (CPP)

                                                                  • Definitions

                                                                    • Malignant tumor originating from epithelium of choroid plexus

                                                                  IMAGING

                                                                  • General Features

                                                                    • CT Findings

                                                                      • MR Findings

                                                                        • Ultrasonographic Findings

                                                                          • Angiographic Findings

                                                                            • Nuclear Medicine Findings

                                                                              • Imaging Recommendations

                                                                                DIFFERENTIAL DIAGNOSIS

                                                                                  PATHOLOGY

                                                                                  • General Features

                                                                                    • Staging, Grading, & Classification

                                                                                      • Gross Pathologic & Surgical Features

                                                                                        • Microscopic Features

                                                                                          • Immunochemistry

                                                                                            • Genetic Alterations

                                                                                              CLINICAL ISSUES

                                                                                              • Presentation

                                                                                                • Demographics

                                                                                                  • Natural History & Prognosis

                                                                                                    • Treatment

                                                                                                      DIAGNOSTIC CHECKLIST

                                                                                                      • Consider

                                                                                                        • Image Interpretation Pearls

                                                                                                          Selected References

                                                                                                          1. Crawford JR et al: Perinatal (fetal and neonatal) choroid plexus tumors: a review. Childs Nerv Syst. 35(6):937-44, 2019
                                                                                                          2. Sasaki T et al: Roles of the apparent diffusion coefficient and tumor volume in predicting tumor grade in patients with choroid plexus tumors. Neuroradiology. 60(5):479-86, 2018
                                                                                                          3. Siegfried A et al: A French retrospective study on clinical outcome in 102 choroid plexus tumors in children. J Neurooncol. 135(1):151-60, 2017
                                                                                                          4. Thomas C et al: Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups. Neuro Oncol. 18(6):790-6, 2016
                                                                                                          5. Cannon DM et al: Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 121(1):151-7, 2015
                                                                                                          6. Dangouloff-Ros V et al: Choroid plexus neoplasms: toward a distinction between carcinoma and papilloma using arterial spin-labeling. AJNR Am J Neuroradiol. 36(9):1786-90, 2015
                                                                                                          7. Dudley RW et al: Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neurooncol. 121(1):201-7, 2015
                                                                                                          8. Merino DM et al: Molecular characterization of choroid plexus tumors reveals novel clinically relevant subgroups. Clin Cancer Res. 21(1):184-92, 2015
                                                                                                          9. Zaky W et al: Choroid plexus carcinoma in children: the head start experience. Pediatr Blood Cancer. 62(5):784-9, 2015
                                                                                                          10. Ruland V et al: Choroid plexus carcinomas are characterized by complex chromosomal alterations related to patient age and prognosis. Genes Chromosomes Cancer. 53(5):373-80, 2014
                                                                                                          11. Korchi AM et al: Pseudoprogression after proton beam irradiation for a choroid plexus carcinoma in pediatric patient: MRI and PET imaging patterns. Childs Nerv Syst. 29(3):509-12, 2013
                                                                                                          12. Gonzalez KD et al: Beyond Li Fraumeni syndrome: clinical characteristics of families with p53 germline mutations. J Clin Oncol. 27(8):1250-6, 2009
                                                                                                          13. Gopal P et al: Choroid plexus carcinoma. Arch Pathol Lab Med. 132(8):1350-4, 2008
                                                                                                          14. Wrede B et al: Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol. 85(3):345-51, 2007
                                                                                                          15. Krieger MD et al: Differentiation of choroid plexus tumors by advanced magnetic resonance spectroscopy. Neurosurg Focus. 18(6A):E4, 2005
                                                                                                          16. Levy ML et al: Choroid plexus tumors in children: significance of stromal invasion. Neurosurgery. 48(2):303-9, 2001
                                                                                                          17. Malkin D et al: Tissue-specific expression of SV40 in tumors associated with the Li-Fraumeni syndrome. Oncogene. 20(33):4441-9, 2001
                                                                                                          18. Poussaint TY: Magnetic resonance imaging of pediatric brain tumors: state of the art. Top Magn Reson Imaging. 12(6):411-33, 2001
                                                                                                          19. Wolff JE et al: Detection of choroid plexus carcinoma with Tc-99m sestamibi: case report and review of the literature. Med Pediatr Oncol. 36(2):323-5, 2001
                                                                                                          20. Sévenet N et al: Constitutional mutations of the hSNF5/INI1 gene predispose to a variety of cancers. Am J Hum Genet. 65(5):1342-8, 1999