Endolymphatic sac tumors’: a surgical dilemma – to operate or not?

AIM: To aid decision making in Endolymphatic Sac Tumors (EST) with respect to surgical resection versus conservative care with stringent clinical observation. Methods: Our experience of a single case of EST managed conservatively after adequate pathological and radiological assessment. We have reviewed isolated case reports and case series in literature with most cases being managed with surgical resection. Results: Conservative treatment is an option in cystic variants of EST and where the morbidity with surgery outweighs the suspected future clinical deterioration. Surgical resection is the ideal treatment in papillary forms, aggressive variants and completely resectable lesions. Discussion: Endolymphatic sac tumor (EST) is a neuroectodermal tumor of the temporal bone and has high prevalence in von Hippel-Lindau (VHL) disease. ELST presents diagnostic challenge due to its rarity and the variety of other tumours potentially showing similar features. Conclusion: Treatment options in EST should be decided keeping in mind the relevant tumour and patient factors to avoid undue surgical morbidity.