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Congenital Mastoid Cholesteatoma
William T. O'Brien, Sr., DO, FAOCR; H. Ric Harnsberger, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Congenital mastoid cholesteatoma (CMC)
              • Synonyms

                • Primary mastoid cholesteatoma or epidermoid
              • Definitions

                • Congenital cholesteatoma in mastoid secondary to epithelial rest

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Reporting Tips

                                        Selected References

                                        1. Annalisa P et al: Isolated congenital mastoid cholesteatoma with no involvement of aditus ad antrum and middle ear. Clin Med Insights Case Rep. ePub, 2021
                                        2. Misale P et al: Congenital cholesteatoma in adults-interesting presentations and management. Indian J Otolaryngol Head Neck Surg. 70(4):578-82, 2018
                                        3. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. Jun;37(6):1185-9, 2016
                                        4. Cvorović L et al: Congenital cholesteatoma of mastoid origin--a multicenter case series. Vojnosanit Pregl. 71(7):619-22, 2014
                                        5. Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
                                        6. Giannuzzi AL et al: Congenital mastoid cholesteatoma: case series, definition, surgical key points, and literature review. Ann Otol Rhinol Laryngol. 120(11):700-6, 2011
                                        7. Lee JH et al: Congenital cholesteatoma of mastoid origin. J Laryngol Otol. 121(11):e20, 2007
                                        8. Warren FM et al: Congenital cholesteatoma of the mastoid temporal bone. Laryngoscope. 117(8):1389-94, 2007
                                        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

                                                      • Congenital mastoid cholesteatoma (CMC)
                                                    • Synonyms

                                                      • Primary mastoid cholesteatoma or epidermoid
                                                    • Definitions

                                                      • Congenital cholesteatoma in mastoid secondary to epithelial rest

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Annalisa P et al: Isolated congenital mastoid cholesteatoma with no involvement of aditus ad antrum and middle ear. Clin Med Insights Case Rep. ePub, 2021
                                                                              2. Misale P et al: Congenital cholesteatoma in adults-interesting presentations and management. Indian J Otolaryngol Head Neck Surg. 70(4):578-82, 2018
                                                                              3. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. Jun;37(6):1185-9, 2016
                                                                              4. Cvorović L et al: Congenital cholesteatoma of mastoid origin--a multicenter case series. Vojnosanit Pregl. 71(7):619-22, 2014
                                                                              5. Más-Estellés F et al: Contemporary non-echo-planar diffusion-weighted imaging of middle ear cholesteatomas. Radiographics. 32(4):1197-213, 2012
                                                                              6. Giannuzzi AL et al: Congenital mastoid cholesteatoma: case series, definition, surgical key points, and literature review. Ann Otol Rhinol Laryngol. 120(11):700-6, 2011
                                                                              7. Lee JH et al: Congenital cholesteatoma of mastoid origin. J Laryngol Otol. 121(11):e20, 2007
                                                                              8. Warren FM et al: Congenital cholesteatoma of the mastoid temporal bone. Laryngoscope. 117(8):1389-94, 2007