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Endolymphatic Sac Tumor
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 endolymphatic sac (ELS), Heffner tumor
            • Definitions

              • Papillary cystadenomatous tumor of ELS
                • Originates from epithelium of ELS & duct

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Angiographic Findings

                    • Nuclear Medicine 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. Sykopetrites V et al: The endolymphatic sac tumor: challenges in the eradication of a localized disease. Eur Arch Otorhinolaryngol. 278(7):2297-304, 2021
                                              2. Shell J et al: The utility of 68Gallium-DOTATATE PET/CT in the detection of von Hippel-Lindau disease associated tumors. Eur J Radiol. 112:130-5, 2019
                                              3. Mendenhall WM et al: Current treatment of endolymphatic sac tumor of the temporal bone. Adv Ther. 35(7):887-98, 2018
                                              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. 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, 2015
                                              6. 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
                                              7. Wick CC et al: Endolymphatic sac tumors. Otolaryngol Clin North Am. 48(2):317-30, 2015
                                              8. Nevoux J et al: Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease. Otol Neurotol. 35(5):899-904, 2014
                                              9. Carlson ML et al: Management of primary and recurrent endolymphatic sac tumors. Otol Neurotol. 34(5):939-43, 2013
                                              10. 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
                                              11. Lee KJ et al: Endolymphatic sac tumor presenting with Ménière's disease. Otolaryngol Head Neck Surg. 142(6):915-6, 2010
                                              12. 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
                                              13. Diaz RC et al: Tumors and pseudotumors of the endolymphatic sac. Skull Base. 17(6):379-93, 2007
                                              14. Kang M et al: Imaging features of endolymphatic sac tumours: case report with review of literature. Australas Radiol. 51 Suppl:B199-201, 2007
                                              15. Lalwani AK: Endolymphatic sac tumors in von Hippel-Lindau disease. Arch Otolaryngol Head Neck Surg. 133(9):857, 2007
                                              16. Megerian CA et al: Evaluation and management of endolymphatic sac and duct tumors. Otolaryngol Clin North Am. 40(3):463-78, viii, 2007
                                              17. Bisceglia M et al: Endolymphatic sac papillary tumor (Heffner tumor). Adv Anat Pathol. 13(3):131-8, 2006
                                              18. Patel NP et al: The radiologic diagnosis of endolymphatic sac tumors. Laryngoscope. 116(1):40-6, 2006
                                              19. Saunders NC et al: Metastatic squamous carcinoma with features typical of an endolymphatic sac tumor. Otol Neurotol. 27(5):739-40, 2006
                                              20. Schipper J et al: Endolymphatic sac tumours: surgical management. J Otolaryngol. 35(6):387-94, 2006
                                              21. Hansen MR et al: Surgical outcomes in patients with endolymphatic sac tumors. Laryngoscope. 114(8):1470-4, 2004
                                              22. 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
                                              23. Cohen JE et al: Endolymphatic sac tumor: staged endovascular-neurosurgical approach. Neurol Res. 25(3):237-40, 2003
                                              24. Devaney KO et al: Endolymphatic sac tumor (low-grade papillary adenocarcinoma) of the temporal bone. Acta Otolaryngol. 123(9):1022-6, 2003
                                              25. Richards PS et al: Endolymphatic sac tumours. J Laryngol Otol. 117(8):666-9, 2003
                                              26. Baltacioglu F et al: MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases. Neuroradiology. 44(1):91-6, 2002
                                              27. Joy HM et al: Radiological considerations in the diagnosis of an endolymphatic sac tumour. Clin Radiol. 57(7):652-4, 2002
                                              28. Luff DA et al: Endolymphatic sac tumours. J Laryngol Otol. 116(5):398-401, 2002
                                              29. 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
                                              30. 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
                                              31. Gaeta M et al: Sudden unilateral deafness with endolymphatic sac adenocarcinoma: MRI. Neuroradiology. 41(10):799-801, 1999
                                              32. Roche PH et al: Endolymphatic sac tumors: report of three cases. Neurosurgery. 42(4):927-32, 1998
                                              33. Mukherji SK et al: Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology. 202(3):801-8, 1997
                                              34. 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
                                              35. 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 endolymphatic sac (ELS), Heffner tumor
                                                        • Definitions

                                                          • Papillary cystadenomatous tumor of ELS
                                                            • Originates from epithelium of ELS & duct

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Angiographic Findings

                                                                • Nuclear Medicine 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. Sykopetrites V et al: The endolymphatic sac tumor: challenges in the eradication of a localized disease. Eur Arch Otorhinolaryngol. 278(7):2297-304, 2021
                                                                                          2. Shell J et al: The utility of 68Gallium-DOTATATE PET/CT in the detection of von Hippel-Lindau disease associated tumors. Eur J Radiol. 112:130-5, 2019
                                                                                          3. Mendenhall WM et al: Current treatment of endolymphatic sac tumor of the temporal bone. Adv Ther. 35(7):887-98, 2018
                                                                                          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. 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, 2015
                                                                                          6. 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
                                                                                          7. Wick CC et al: Endolymphatic sac tumors. Otolaryngol Clin North Am. 48(2):317-30, 2015
                                                                                          8. Nevoux J et al: Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease. Otol Neurotol. 35(5):899-904, 2014
                                                                                          9. Carlson ML et al: Management of primary and recurrent endolymphatic sac tumors. Otol Neurotol. 34(5):939-43, 2013
                                                                                          10. 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
                                                                                          11. Lee KJ et al: Endolymphatic sac tumor presenting with Ménière's disease. Otolaryngol Head Neck Surg. 142(6):915-6, 2010
                                                                                          12. 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
                                                                                          13. Diaz RC et al: Tumors and pseudotumors of the endolymphatic sac. Skull Base. 17(6):379-93, 2007
                                                                                          14. Kang M et al: Imaging features of endolymphatic sac tumours: case report with review of literature. Australas Radiol. 51 Suppl:B199-201, 2007
                                                                                          15. Lalwani AK: Endolymphatic sac tumors in von Hippel-Lindau disease. Arch Otolaryngol Head Neck Surg. 133(9):857, 2007
                                                                                          16. Megerian CA et al: Evaluation and management of endolymphatic sac and duct tumors. Otolaryngol Clin North Am. 40(3):463-78, viii, 2007
                                                                                          17. Bisceglia M et al: Endolymphatic sac papillary tumor (Heffner tumor). Adv Anat Pathol. 13(3):131-8, 2006
                                                                                          18. Patel NP et al: The radiologic diagnosis of endolymphatic sac tumors. Laryngoscope. 116(1):40-6, 2006
                                                                                          19. Saunders NC et al: Metastatic squamous carcinoma with features typical of an endolymphatic sac tumor. Otol Neurotol. 27(5):739-40, 2006
                                                                                          20. Schipper J et al: Endolymphatic sac tumours: surgical management. J Otolaryngol. 35(6):387-94, 2006
                                                                                          21. Hansen MR et al: Surgical outcomes in patients with endolymphatic sac tumors. Laryngoscope. 114(8):1470-4, 2004
                                                                                          22. 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
                                                                                          23. Cohen JE et al: Endolymphatic sac tumor: staged endovascular-neurosurgical approach. Neurol Res. 25(3):237-40, 2003
                                                                                          24. Devaney KO et al: Endolymphatic sac tumor (low-grade papillary adenocarcinoma) of the temporal bone. Acta Otolaryngol. 123(9):1022-6, 2003
                                                                                          25. Richards PS et al: Endolymphatic sac tumours. J Laryngol Otol. 117(8):666-9, 2003
                                                                                          26. Baltacioglu F et al: MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases. Neuroradiology. 44(1):91-6, 2002
                                                                                          27. Joy HM et al: Radiological considerations in the diagnosis of an endolymphatic sac tumour. Clin Radiol. 57(7):652-4, 2002
                                                                                          28. Luff DA et al: Endolymphatic sac tumours. J Laryngol Otol. 116(5):398-401, 2002
                                                                                          29. 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
                                                                                          30. 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
                                                                                          31. Gaeta M et al: Sudden unilateral deafness with endolymphatic sac adenocarcinoma: MRI. Neuroradiology. 41(10):799-801, 1999
                                                                                          32. Roche PH et al: Endolymphatic sac tumors: report of three cases. Neurosurgery. 42(4):927-32, 1998
                                                                                          33. Mukherji SK et al: Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology. 202(3):801-8, 1997
                                                                                          34. 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
                                                                                          35. Lo WW et al: Endolymphatic sac tumors: radiologic appearance. Radiology. 189(1):199-204, 1993