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Cavernous Malformation
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Cavernoma, cavernous hemangioma, cavernous angioma, "angiographically occult" vascular malformation
            • Definitions

              • Cavernous malformation (CM): Benign vascular lesion arising in CNS
              • Consists of dilated sinusoids containing blood products of various ages & lined by thin immature walls

            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

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Choquet H et al: Genetics of cerebral cavernous malformations: current status and future prospects. J Neurosurg Sci. 59(3):211-20, 2015
                                            2. Gross BA et al: The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 1-6, 2015
                                            3. Horne MA et al: Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol. ePub, 2015
                                            4. Kosnik-Infinger L et al: Management of cerebral cavernous malformations in the pediatric population: a literature review and case illustrations. J Neurosurg Sci. 59(3):283-94, 2015
                                            5. Nikoubashman O et al: Prospective hemorrhage rates of cerebral cavernous malformations in children and adolescents based on MRI appearance. AJNR Am J Neuroradiol. 36(11):2177-83, 2015
                                            6. Di Giannatale A et al: Natural history of cavernous malformations in children with brain tumors treated with radiotherapy and chemotherapy. J Neurooncol. 117(2):311-20, 2014
                                            7. Mikati AG et al: Dynamic permeability and quantitative susceptibility: related imaging biomarkers in cerebral cavernous malformations. Stroke. 45(2):598-601, 2014
                                            8. Nagy G et al: Stereotactic radiosurgery of intracranial cavernous malformations. Neurosurg Clin N Am. 24(4):575-89, 2013
                                            9. Al-Holou WN et al: Natural history and imaging prevalence of cavernous malformations in children and young adults. J Neurosurg Pediatr. 9(2):198-205, 2012
                                            10. Alexiou GA et al: Treatment options for central nervous system cavernous malformations in children. Eur J Paediatr Neurol. 15(3):281, 2011
                                            11. Chang EF et al: Supratentorial cavernous malformations in eloquent and deep locations: surgical approaches and outcomes. Clinical article. J Neurosurg. 114(3):814-27, 2011
                                            12. Ozgen B et al: Radiological features of childhood giant cavernous malformations. Neuroradiology. 53(4):283-9, 2011
                                            13. Abla AA et al: Cavernous malformations of the brainstem presenting in childhood: surgical experience in 40 patients. Neurosurgery. 67(6):1589-98; discussion 1598-9, 2010
                                            14. Smith ER et al: Cavernous malformations. Neurosurg Clin N Am. 21(3):483-90, 2010
                                            15. Chen L et al: Apoptotic functions of PDCD10/CCM3, the gene mutated in cerebral cavernous malformation 3. Stroke. 40(4):1474-81, 2009
                                            16. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part I. High-field imaging of excised human lesions. Neurosurgery. 63(4):782-9; discussion 789, 2008
                                            17. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part II. Imaging of lesions in murine models. Neurosurgery. 63(4):790-7; discussion 797-8, 2008
                                            18. Yun TJ et al: A T1 hyperintense perilesional signal aids in the differentiation of a cavernous angioma from other hemorrhagic masses. AJNR Am J Neuroradiol. 29(3):494-500, 2008
                                            19. Reich P et al: Molecular genetic investigations in the CCM1 gene in sporadic cerebral cavernomas. Neurology. 60(7):1135-8, 2003
                                            20. Kehrer-Sawatzki H et al: Mutation and expression analysis of the KRIT1 gene associated with cerebral cavernous malformations (CCM1). Acta Neuropathol (Berl). 104(3):231-40, 2002
                                            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

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Cavernoma, cavernous hemangioma, cavernous angioma, "angiographically occult" vascular malformation
                                                      • Definitions

                                                        • Cavernous malformation (CM): Benign vascular lesion arising in CNS
                                                        • Consists of dilated sinusoids containing blood products of various ages & lined by thin immature walls

                                                      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

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Choquet H et al: Genetics of cerebral cavernous malformations: current status and future prospects. J Neurosurg Sci. 59(3):211-20, 2015
                                                                                      2. Gross BA et al: The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 1-6, 2015
                                                                                      3. Horne MA et al: Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol. ePub, 2015
                                                                                      4. Kosnik-Infinger L et al: Management of cerebral cavernous malformations in the pediatric population: a literature review and case illustrations. J Neurosurg Sci. 59(3):283-94, 2015
                                                                                      5. Nikoubashman O et al: Prospective hemorrhage rates of cerebral cavernous malformations in children and adolescents based on MRI appearance. AJNR Am J Neuroradiol. 36(11):2177-83, 2015
                                                                                      6. Di Giannatale A et al: Natural history of cavernous malformations in children with brain tumors treated with radiotherapy and chemotherapy. J Neurooncol. 117(2):311-20, 2014
                                                                                      7. Mikati AG et al: Dynamic permeability and quantitative susceptibility: related imaging biomarkers in cerebral cavernous malformations. Stroke. 45(2):598-601, 2014
                                                                                      8. Nagy G et al: Stereotactic radiosurgery of intracranial cavernous malformations. Neurosurg Clin N Am. 24(4):575-89, 2013
                                                                                      9. Al-Holou WN et al: Natural history and imaging prevalence of cavernous malformations in children and young adults. J Neurosurg Pediatr. 9(2):198-205, 2012
                                                                                      10. Alexiou GA et al: Treatment options for central nervous system cavernous malformations in children. Eur J Paediatr Neurol. 15(3):281, 2011
                                                                                      11. Chang EF et al: Supratentorial cavernous malformations in eloquent and deep locations: surgical approaches and outcomes. Clinical article. J Neurosurg. 114(3):814-27, 2011
                                                                                      12. Ozgen B et al: Radiological features of childhood giant cavernous malformations. Neuroradiology. 53(4):283-9, 2011
                                                                                      13. Abla AA et al: Cavernous malformations of the brainstem presenting in childhood: surgical experience in 40 patients. Neurosurgery. 67(6):1589-98; discussion 1598-9, 2010
                                                                                      14. Smith ER et al: Cavernous malformations. Neurosurg Clin N Am. 21(3):483-90, 2010
                                                                                      15. Chen L et al: Apoptotic functions of PDCD10/CCM3, the gene mutated in cerebral cavernous malformation 3. Stroke. 40(4):1474-81, 2009
                                                                                      16. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part I. High-field imaging of excised human lesions. Neurosurgery. 63(4):782-9; discussion 789, 2008
                                                                                      17. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part II. Imaging of lesions in murine models. Neurosurgery. 63(4):790-7; discussion 797-8, 2008
                                                                                      18. Yun TJ et al: A T1 hyperintense perilesional signal aids in the differentiation of a cavernous angioma from other hemorrhagic masses. AJNR Am J Neuroradiol. 29(3):494-500, 2008
                                                                                      19. Reich P et al: Molecular genetic investigations in the CCM1 gene in sporadic cerebral cavernomas. Neurology. 60(7):1135-8, 2003
                                                                                      20. Kehrer-Sawatzki H et al: Mutation and expression analysis of the KRIT1 gene associated with cerebral cavernous malformations (CCM1). Acta Neuropathol (Berl). 104(3):231-40, 2002