The Parochial Meddle-Endolymphatic Sac Tumour
Journal Title: Journal of Clinical Immunology AND Microbiology
Endolymphatic sac tumour is an exceptional, benign neoplasm arising from epithelium of endolymphatic duct or sac. Endolymphatic sac tumour is associated with von Hippel –Lindau disease in around 11% to 30% of subjects. The gradually progressive endolymphatic sac tumour manifests with acute or progressive deafness, tinnitus, episodic vertigo, otalgia, otorrhoea, disequilibrium, aural fullness, occipital headache, neurological deficits, facial paresis or hypoglossus muscle paralysis. The papillary neoplasm is layered with simple, cuboidal to columnar epithelial cells with minimal nuclear atypia, infrequent mitotic activity and absence of tumour necrosis. Endolymphatic sac tumour requires a segregation from neoplasms such as middle ear adenoma, middle ear carcinoma, choroid plexus papilloma, para-ganglioma, papillary ependymoma, metastatic renal cell carcinoma, metastatic thyroid papillary carcinoma, jugulotympanic paraganglioma, glomus jugulare tumour, enlarged vestibular aqueduct, petrous apicitis, cholesterol granuloma, meningioma or secondary metastasis within bone. Upon computerized tomography, erosion of petrous bone displays an infiltrative or “moth-eaten” configuration along with centric calcified spicules, intra-tumour and posterior rim calcification and intense, homogeneous enhancement of the tumefaction. Comprehensive surgical excision of the neoplasm is an optimal treatment strategy.