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Congenital Petrous Apex Cholesteatoma
Philip R. Chapman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Petrous apex (PA) cholesteatoma (PA-Chol)
            • Synonyms

              • Congenital cholesteatoma, epidermoid or epidermoid cyst
            • Definitions

              • PA cholesteatoma (epidermoid): Ectopic ectodermal tissue in petrous bone ultimately gives rise to expansile, cystic mass lined by stratified squamous epithelium and filled with keratinous debris

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        • Reporting Tips

                                          Selected References

                                          1. Maxwell AK et al: Congenital cholesteatoma of the sphenoid, occipital, and temporal bones: 54-year follow up. Otol Neurotol. 41(5):e593-6, 2020
                                          2. Casazza GC et al: Primary petrous apex epidermoids with skull base erosion. Otol Neurotol. 40(5):e556-61, 2019
                                          3. Orhan KS et al: Endoscope-assisted surgery for petrous bone cholesteatoma with hearing preservation. J Int Adv Otol. 15(3):391-5, 2019
                                          4. Sharma SC et al: Petrous bone cholesteatoma: radical excision with an endeavour for hearing preservation. Indian J Otolaryngol Head Neck Surg. 71(Suppl 2):1572-9, 2019
                                          5. Iannella G et al: Giant petrous bone cholesteatoma: combined microscopic surgery and an adjuvant endoscopic approach. J Neurol Surg Rep. 77(1):e46-9, 2016
                                          6. MacKeith SA et al: Recurrent aseptic meningitis as a rare but important presentation of congenital petrous apex cholesteatoma: the value of appropriate imaging. BMJ Case Rep. 2014
                                          7. Chapman PR et al: Petrous apex lesions: pictorial review. AJR Am J Roentgenol. 196(3 Suppl):WS26-37 Quiz S40-3, 2011
                                          8. De Foer B et al: Diffusion-weighted magnetic resonance imaging of the temporal bone. Neuroradiology. 52(9):785-807, 2010
                                          9. Connor SE et al: Imaging of the petrous apex: a pictorial review. Br J Radiol. 81(965):427-35, 2008
                                          10. Moffat D et al: Petrous temporal bone cholesteatoma: a new classification and long-term surgical outcomes. Skull Base. 18(2):107-15, 2008
                                          11. Kojima H et al: Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol. 27(5):299-305, 2006
                                          12. Mattox DE: Endoscopy-assisted surgery of the petrous apex. Otolaryngol Head Neck Surg. 130(2):229-41, 2004
                                          13. Profant M et al: Petrous apex cholesteatoma. Acta Otolaryngol. 120(2):164-7, 2000
                                          14. Chang P et al: Imaging destructive lesions of the petrous apex. Laryngoscope. 108(4 Pt 1):599-604, 1998
                                          15. Mafee MF: MRI and CT in the evaluation of acquired and congenital cholesteatomas of the temporal bone. J Otolaryngol. 22(4):239-48, 1993
                                          16. Atlas MD et al: Petrous apex cholesteatoma: diagnostic and treatment dilemmas. Laryngoscope. 102(12 Pt 1):1363-8, 1992
                                          17. Ishii K et al: Middle ear cholesteatoma extending into the petrous apex: evaluation by CT and MR imaging. AJNR Am J Neuroradiol. 12(4):719-24, 1991
                                          18. Glasscock ME 3rd et al: Petrous apex cholesteatoma. Otolaryngol Clin North Am. 22(5):981-1002, 1989
                                          19. Rosenberg RA et al: Cholesteatoma vs. cholesterol granuloma of the petrous apex. Otolaryngol Head Neck Surg. 94(3):322-7, 1986
                                          20. Horn KL et al: Congenital cholesteatoma of the petrous pyramid. Arch Otolaryngol. 111(9):621-2, 1985
                                          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

                                                    • Abbreviations

                                                      • Petrous apex (PA) cholesteatoma (PA-Chol)
                                                    • Synonyms

                                                      • Congenital cholesteatoma, epidermoid or epidermoid cyst
                                                    • Definitions

                                                      • PA cholesteatoma (epidermoid): Ectopic ectodermal tissue in petrous bone ultimately gives rise to expansile, cystic mass lined by stratified squamous epithelium and filled with keratinous debris

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              • Image Interpretation Pearls

                                                                                • Reporting Tips

                                                                                  Selected References

                                                                                  1. Maxwell AK et al: Congenital cholesteatoma of the sphenoid, occipital, and temporal bones: 54-year follow up. Otol Neurotol. 41(5):e593-6, 2020
                                                                                  2. Casazza GC et al: Primary petrous apex epidermoids with skull base erosion. Otol Neurotol. 40(5):e556-61, 2019
                                                                                  3. Orhan KS et al: Endoscope-assisted surgery for petrous bone cholesteatoma with hearing preservation. J Int Adv Otol. 15(3):391-5, 2019
                                                                                  4. Sharma SC et al: Petrous bone cholesteatoma: radical excision with an endeavour for hearing preservation. Indian J Otolaryngol Head Neck Surg. 71(Suppl 2):1572-9, 2019
                                                                                  5. Iannella G et al: Giant petrous bone cholesteatoma: combined microscopic surgery and an adjuvant endoscopic approach. J Neurol Surg Rep. 77(1):e46-9, 2016
                                                                                  6. MacKeith SA et al: Recurrent aseptic meningitis as a rare but important presentation of congenital petrous apex cholesteatoma: the value of appropriate imaging. BMJ Case Rep. 2014
                                                                                  7. Chapman PR et al: Petrous apex lesions: pictorial review. AJR Am J Roentgenol. 196(3 Suppl):WS26-37 Quiz S40-3, 2011
                                                                                  8. De Foer B et al: Diffusion-weighted magnetic resonance imaging of the temporal bone. Neuroradiology. 52(9):785-807, 2010
                                                                                  9. Connor SE et al: Imaging of the petrous apex: a pictorial review. Br J Radiol. 81(965):427-35, 2008
                                                                                  10. Moffat D et al: Petrous temporal bone cholesteatoma: a new classification and long-term surgical outcomes. Skull Base. 18(2):107-15, 2008
                                                                                  11. Kojima H et al: Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol. 27(5):299-305, 2006
                                                                                  12. Mattox DE: Endoscopy-assisted surgery of the petrous apex. Otolaryngol Head Neck Surg. 130(2):229-41, 2004
                                                                                  13. Profant M et al: Petrous apex cholesteatoma. Acta Otolaryngol. 120(2):164-7, 2000
                                                                                  14. Chang P et al: Imaging destructive lesions of the petrous apex. Laryngoscope. 108(4 Pt 1):599-604, 1998
                                                                                  15. Mafee MF: MRI and CT in the evaluation of acquired and congenital cholesteatomas of the temporal bone. J Otolaryngol. 22(4):239-48, 1993
                                                                                  16. Atlas MD et al: Petrous apex cholesteatoma: diagnostic and treatment dilemmas. Laryngoscope. 102(12 Pt 1):1363-8, 1992
                                                                                  17. Ishii K et al: Middle ear cholesteatoma extending into the petrous apex: evaluation by CT and MR imaging. AJNR Am J Neuroradiol. 12(4):719-24, 1991
                                                                                  18. Glasscock ME 3rd et al: Petrous apex cholesteatoma. Otolaryngol Clin North Am. 22(5):981-1002, 1989
                                                                                  19. Rosenberg RA et al: Cholesteatoma vs. cholesterol granuloma of the petrous apex. Otolaryngol Head Neck Surg. 94(3):322-7, 1986
                                                                                  20. Horn KL et al: Congenital cholesteatoma of the petrous pyramid. Arch Otolaryngol. 111(9):621-2, 1985