Objective: Endolymphatic sac tumor (Heffner tumor/ELST) is benign, indolent but invasive arising in the posterior petrous ridge. ELST could occur sporadically or as a part of von Hippel–Lindau (VHL) disease. Morphologically, these tumors showed a papillary and glandular architecture. We report a case of endolymphatic sac tumor to discuss the diagnostic and therapeutic features of this rare entity.
Observation: A 58-year-old woman presented with one year history of slowly progressing unilateral hearing loss, tinnitus and vertigo. The patient was diagnosed with an endolymphatic sac tumor and was managed with a radiation therapy.
Conclusion: Endolymphatic sac tumor (ELST) is a rare low-grade adenocarcinoma of the skull base. Diagnosis is based on clinical, radiological, and pathological correlation and the optimal treatment is surgical resection with security respected margins. Radiotherapy remains controversial. A long term follow up is recommended.
Key-words: Endolymphatic sac tumor; Papillary tumor; Von Hippel-Lindau disease.
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