title: Low-grade adenocarcinoma of endolymphatic sac origin.
abstract: We present a case of papillary cystic low-grade adenocarcinoma of endolymphatic sac origin. These tumors are very rare and only a few cases have been reported in literature. They have a protracted clinical course causing progressive invasion and bony destruction. Radiologically, they appear as a heterogenous lobulated vascular mass, invading bone and compressing surrounding structures. Histologically, these tumors are composed of uniform population of cells, resembling normal endolymphatic sac epithelium. This causes considerable diagnostic difficulty. A strong index of suspicion along with clinical and radiological correlation is essential to arrive at a correct diagnosis. In some bilateral cases, association with von Hippel-Lindau disease has been noted. Radical mastoidectomy and temporal bone resection, which may sometimes necessitate sacrifice of cranial nerves, is the treatment of choice.
authors.affiliation: Department of Pathology, Kasturba Medical College, Manipal, Karnataka, India. ssaurabh10@hotmail.com
authors.forename: Saurabh
authors.initials: S
authors.lastname: Malhotra
authors.forename: Ravikala V
authors.initials: RV
authors.lastname: Rao
authors.forename: Manna
authors.initials: M
authors.lastname: Valiathan
authors.forename: Mary
authors.initials: M
authors.lastname: Mathew
authors.forename: Deepak Ranjan
authors.initials: DR
authors.lastname: Nayak
authors.forename: A
authors.initials: A
authors.lastname: Raja
mesh_terms: D000230:Adenocarcinoma / Q000145:classification / Q000175:diagnosis* / Q000473:pathology; D000328:Adult; D018450:Disease Progression; D004428:Ear Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000473:pathology; D004712:Endolymphatic Sac / Q000473:pathology; D006261:Headache; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D014012:Tinnitus; D014057:Tomography, X-Ray Computed