Papillary endolymphatic sac tumor: Case report with clinical study and therapeutic management

Abstract

INTRODUCTION AND IMPORTANCE: Endolymphatic sac tumor (ELST) is a rare, histologically benign tumor with potentially aggressive behavior. Its management depends on the stage and extent of the disease. CASE PRESENTATION: We report the case of a 34-year-old male, with no significant medical history, presenting with progressive right-sided hearing loss, high-pitched tinnitus, and recurrent vertigo. Radiological assessment with CT and MRI revealed a hypervascular lesion centered on the petrous apex, with erosion of the internal auditory canal. A retrolabyrinthine surgical approach was performed. Histopathological examination confirmed a papillary tumor of the endolymphatic sac. Postoperative recovery was favorable, with no immediate neurological complications. Histological and immunohistochemical studies confirmed the diagnosis of ELST. Genetic testing for Von Hippel-Lindau (VHL) disease was negative. CLINICAL DISCUSSION: ELSTs may occur sporadically or in association with Von Hippel-Lindau disease. Due to non-specific symptoms, imaging plays a crucial role in the diagnostic process. Surgery remains the mainstay of treatment to reduce the risk of recurrence, and the choice of surgical approach depends on several factors. The role of radiotherapy remains controversial. Long-term follow-up is essential to monitor for recurrence. CONCLUSION: This report underscores the rarity of ELST and supports early surgical intervention due to its locally aggressive nature. Given the potential association with VHL syndrome, genetic screening is recommended even in apparently sporadic cases.