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Endolymphatic Sac Tumor
Surjith Vattoth, MD, FRCR; Hilda E. Stambuk, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Endolymphatic sac tumor (ELST)
            • Synonyms

              • Adenomatous tumor of ELS, Heffner tumor
            • Definitions

              • Papillary cystadenomatous tumor of ELS
                • Originates from epithelium of endolymphatic sac

            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. Mendenhall WM et al: Current treatment of endolymphatic sac tumor of the temporal bone. Adv Ther. 35(7):887-98, 2018
                                            2. Wang HQ et al: Clinicopathological features of low-grade malignant endolymphatic sac tumors. Pathol Res Pract. 214(3):431-35, 2018
                                            3. Bausch B et al: Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the international ELST registry. Head Neck. 38 Suppl 1:E673-9, 2016
                                            4. Shaigany K et al: Endolymphatic sac tumor in association with von Hippel-Lindau syndrome. Ear Nose Throat J. 95(3):96-8, 2016
                                            5. Du J et al: Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle. Neuropathology. 35(5):410-20, 2015
                                            6. Wick CC et al: Endolymphatic sac tumors. Otolaryngol Clin North Am. 48(2):317-30, 2015
                                            7. Nevoux J et al: Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease. Otol Neurotol. 35(5):899-904, 2014
                                            8. Carlson ML et al: Management of primary and recurrent endolymphatic sac tumors. Otol Neurotol. 34(5):939-43, 2013
                                            9. Bell D et al: Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): sine qua non radiology-pathology and the University of Texas MD Anderson Cancer Center experience. Ann Diagn Pathol. 15(2):117-23, 2011
                                            10. Lee KJ et al: Endolymphatic sac tumor presenting with Ménière's disease. Otolaryngol Head Neck Surg. 142(6):915-6, 2010
                                            11. Janse van Rensburg P et al: Magnetic resonance and computed tomography imaging of a grade IV papillary endolymphatic sac tumour. J Neurooncol. 89(2):199-203, 2008
                                            12. Diaz RC et al: Tumors and pseudotumors of the endolymphatic sac. Skull Base. 17(6):379-93, 2007
                                            13. Kang M et al: Imaging features of endolymphatic sac tumours: case report with review of literature. Australas Radiol. 51 Suppl:B199-201, 2007
                                            14. Lalwani AK: Endolymphatic sac tumors in von Hippel-Lindau disease. Arch Otolaryngol Head Neck Surg. 133(9):857, 2007
                                            15. Megerian CA et al: Evaluation and management of endolymphatic sac and duct tumors. Otolaryngol Clin North Am. 40(3):463-78, viii, 2007
                                            16. Bisceglia M et al: Endolymphatic sac papillary tumor (Heffner tumor). Adv Anat Pathol. 13(3):131-8, 2006
                                            17. Patel NP et al: The radiologic diagnosis of endolymphatic sac tumors. Laryngoscope. 116(1):40-6, 2006
                                            18. Saunders NC et al: Metastatic squamous carcinoma with features typical of an endolymphatic sac tumor. Otol Neurotol. 27(5):739-40, 2006
                                            19. Schipper J et al: Endolymphatic sac tumours: surgical management. J Otolaryngol. 35(6):387-94, 2006
                                            20. Hansen MR et al: Surgical outcomes in patients with endolymphatic sac tumors. Laryngoscope. 114(8):1470-4, 2004
                                            21. Jensen RL et al: Endolymphatic sac tumors in patients with and without von Hippel-Lindau disease: the role of genetic mutation, von Hippel-Lindau protein, and hypoxia inducible factor-1alpha expression. J Neurosurg. 100(3):488-97, 2004
                                            22. Cohen JE et al: Endolymphatic sac tumor: staged endovascular-neurosurgical approach. Neurol Res. 25(3):237-40, 2003
                                            23. Devaney KO et al: Endolymphatic sac tumor (low-grade papillary adenocarcinoma) of the temporal bone. Acta Otolaryngol. 123(9):1022-6, 2003
                                            24. Richards PS et al: Endolymphatic sac tumours. J Laryngol Otol. 117(8):666-9, 2003
                                            25. Baltacioglu F et al: MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases. Neuroradiology. 44(1):91-6, 2002
                                            26. Joy HM et al: Radiological considerations in the diagnosis of an endolymphatic sac tumour. Clin Radiol. 57(7):652-4, 2002
                                            27. Luff DA et al: Endolymphatic sac tumours. J Laryngol Otol. 116(5):398-401, 2002
                                            28. Horiguchi H et al: Endolymphatic sac tumor associated with a von Hippel-Lindau disease patient: an immunohistochemical study. Mod Pathol. 14(7):727-32, 2001
                                            29. Richard S et al: Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel-Lindau disease. Neurosurg Rev. 23(1):1-22; discussion 23-4, 2000
                                            30. Gaeta M et al: Sudden unilateral deafness with endolymphatic sac adenocarcinoma: MRI. Neuroradiology. 41(10):799-801, 1999
                                            31. Roche PH et al: Endolymphatic sac tumors: report of three cases. Neurosurgery. 42(4):927-32, 1998
                                            32. Mukherji SK et al: Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology. 202(3):801-8, 1997
                                            33. Ho VT et al: Low-grade adenocarcinoma of probable endolymphatic sac origin: CT and MR appearance. AJNR Am J Neuroradiol. 17(1):168-70, 1996
                                            34. Lo WW et al: Endolymphatic sac tumors: radiologic appearance. Radiology. 189(1):199-204, 1993
                                            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

                                                        • Endolymphatic sac tumor (ELST)
                                                      • Synonyms

                                                        • Adenomatous tumor of ELS, Heffner tumor
                                                      • Definitions

                                                        • Papillary cystadenomatous tumor of ELS
                                                          • Originates from epithelium of endolymphatic sac

                                                      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. Mendenhall WM et al: Current treatment of endolymphatic sac tumor of the temporal bone. Adv Ther. 35(7):887-98, 2018
                                                                                      2. Wang HQ et al: Clinicopathological features of low-grade malignant endolymphatic sac tumors. Pathol Res Pract. 214(3):431-35, 2018
                                                                                      3. Bausch B et al: Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the international ELST registry. Head Neck. 38 Suppl 1:E673-9, 2016
                                                                                      4. Shaigany K et al: Endolymphatic sac tumor in association with von Hippel-Lindau syndrome. Ear Nose Throat J. 95(3):96-8, 2016
                                                                                      5. Du J et al: Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle. Neuropathology. 35(5):410-20, 2015
                                                                                      6. Wick CC et al: Endolymphatic sac tumors. Otolaryngol Clin North Am. 48(2):317-30, 2015
                                                                                      7. Nevoux J et al: Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease. Otol Neurotol. 35(5):899-904, 2014
                                                                                      8. Carlson ML et al: Management of primary and recurrent endolymphatic sac tumors. Otol Neurotol. 34(5):939-43, 2013
                                                                                      9. Bell D et al: Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): sine qua non radiology-pathology and the University of Texas MD Anderson Cancer Center experience. Ann Diagn Pathol. 15(2):117-23, 2011
                                                                                      10. Lee KJ et al: Endolymphatic sac tumor presenting with Ménière's disease. Otolaryngol Head Neck Surg. 142(6):915-6, 2010
                                                                                      11. Janse van Rensburg P et al: Magnetic resonance and computed tomography imaging of a grade IV papillary endolymphatic sac tumour. J Neurooncol. 89(2):199-203, 2008
                                                                                      12. Diaz RC et al: Tumors and pseudotumors of the endolymphatic sac. Skull Base. 17(6):379-93, 2007
                                                                                      13. Kang M et al: Imaging features of endolymphatic sac tumours: case report with review of literature. Australas Radiol. 51 Suppl:B199-201, 2007
                                                                                      14. Lalwani AK: Endolymphatic sac tumors in von Hippel-Lindau disease. Arch Otolaryngol Head Neck Surg. 133(9):857, 2007
                                                                                      15. Megerian CA et al: Evaluation and management of endolymphatic sac and duct tumors. Otolaryngol Clin North Am. 40(3):463-78, viii, 2007
                                                                                      16. Bisceglia M et al: Endolymphatic sac papillary tumor (Heffner tumor). Adv Anat Pathol. 13(3):131-8, 2006
                                                                                      17. Patel NP et al: The radiologic diagnosis of endolymphatic sac tumors. Laryngoscope. 116(1):40-6, 2006
                                                                                      18. Saunders NC et al: Metastatic squamous carcinoma with features typical of an endolymphatic sac tumor. Otol Neurotol. 27(5):739-40, 2006
                                                                                      19. Schipper J et al: Endolymphatic sac tumours: surgical management. J Otolaryngol. 35(6):387-94, 2006
                                                                                      20. Hansen MR et al: Surgical outcomes in patients with endolymphatic sac tumors. Laryngoscope. 114(8):1470-4, 2004
                                                                                      21. Jensen RL et al: Endolymphatic sac tumors in patients with and without von Hippel-Lindau disease: the role of genetic mutation, von Hippel-Lindau protein, and hypoxia inducible factor-1alpha expression. J Neurosurg. 100(3):488-97, 2004
                                                                                      22. Cohen JE et al: Endolymphatic sac tumor: staged endovascular-neurosurgical approach. Neurol Res. 25(3):237-40, 2003
                                                                                      23. Devaney KO et al: Endolymphatic sac tumor (low-grade papillary adenocarcinoma) of the temporal bone. Acta Otolaryngol. 123(9):1022-6, 2003
                                                                                      24. Richards PS et al: Endolymphatic sac tumours. J Laryngol Otol. 117(8):666-9, 2003
                                                                                      25. Baltacioglu F et al: MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases. Neuroradiology. 44(1):91-6, 2002
                                                                                      26. Joy HM et al: Radiological considerations in the diagnosis of an endolymphatic sac tumour. Clin Radiol. 57(7):652-4, 2002
                                                                                      27. Luff DA et al: Endolymphatic sac tumours. J Laryngol Otol. 116(5):398-401, 2002
                                                                                      28. Horiguchi H et al: Endolymphatic sac tumor associated with a von Hippel-Lindau disease patient: an immunohistochemical study. Mod Pathol. 14(7):727-32, 2001
                                                                                      29. Richard S et al: Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel-Lindau disease. Neurosurg Rev. 23(1):1-22; discussion 23-4, 2000
                                                                                      30. Gaeta M et al: Sudden unilateral deafness with endolymphatic sac adenocarcinoma: MRI. Neuroradiology. 41(10):799-801, 1999
                                                                                      31. Roche PH et al: Endolymphatic sac tumors: report of three cases. Neurosurgery. 42(4):927-32, 1998
                                                                                      32. Mukherji SK et al: Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology. 202(3):801-8, 1997
                                                                                      33. Ho VT et al: Low-grade adenocarcinoma of probable endolymphatic sac origin: CT and MR appearance. AJNR Am J Neuroradiol. 17(1):168-70, 1996
                                                                                      34. Lo WW et al: Endolymphatic sac tumors: radiologic appearance. Radiology. 189(1):199-204, 1993