Low-grade adenocarcinoma of endolymphatic sac origin

Saurabh Malhotra, Ravikala V. Rao, Manna Valiathan, Mary Mathew, Deepak Ranjan Nayak, A. Raja

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7 Citations (SciVal)


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.

Original languageEnglish
Pages (from-to)362-365
Number of pages4
JournalAmerican Journal of Otolaryngology - Head and Neck Medicine and Surgery
Issue number5
Publication statusPublished - 01-09-2006

All Science Journal Classification (ASJC) codes

  • Otorhinolaryngology


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