Multidisciplinary approach to the endolymphatic sac tumour

• Published in: Neurocirugía (Asturias, Spain) Vol. 22; no. 6; pp. 567 - 573
• Main Authors: González-Bonet, L G, Amoros-Sebastiá, L I, Piquer-Belloch, J, Riesgo, P, Llacer, J L, Gisbert-Aguilar, J, Morell-Quadreny, L
• Format: Journal Article
• Language: Spanish
• Published: Spain 01.12.2011
• Subjects: Adolescent; Diagnosis, Differential; Ear Neoplasms - complications; Ear Neoplasms - etiology; Ear Neoplasms - pathology; Ear Neoplasms - surgery; Endolymphatic Sac - pathology; Humans; Male; Meniere Disease - etiology; Treatment Outcome; von Hippel-Lindau Disease - complications
• ISSN: 1130-1473
• DOI: 10.4321/S1130-14732011000600011

Papilar adenocarcinoma of endolymphatic sac is related with Von Hippel Lindau disease at 15% of cases, has a slow growing with a high local aggressiveness, and doesn't metastasize. It causes symptoms of Meniere's syndrome due to the compression that produces at endolymphatic duct. When it presents with hearing loss is usually sudden and irreversible manner. The diagnostic is made with image tests and analysis of its structure with immunohistochemical tests. The elective treatment is surgical remove, and its main complication the perioperative bleeding it can be avoided with preoperative embolization or stereotactic radiation. A case of endolymphatic sac tumour is presented, in a 17-years-old male with unilateral deafness and crisis of rotate vertigo, with family history of Von Hippel-Lindau disease. Perceptive deafness and right vestibular arreflexia are detected at technical exploration. In a petrous bone computer tomography appears a mass at vestibular aqueduct. We performed a petrosectomy with presigmoidal approach and saving of inner ear. Pathological analysis revealed an endolymphatic sac tumour. In patients with a family history of Von Hippel Lindau disease and clinical symptoms of vertigo and normal hearing or with slight hearing loss we should suspect the presence of endolymphatic sac tumor. The clinical presentation of hearing loss can be sudden and irreversible even with negative or inconclusive images. Therefore, a quick action is important for the preservation of this function.