Endolymphatic sac tumor and angiomatous lesions of the nasal and pharyngeal mucosa as a first manifestation of von Hippel-Lindau disease.

Abstract

In the present article we report an endolymphatic sac tumor in a 15-year-old male who had additional angiomatous lesions in the nasal and pharyngeal mucosa and was diagnosed with von Hippel-Lindau disease postoperatively. Preoperative imaging excluded cholesteatoma, but did not provide sufficient information to distinguish between jugular paraganglioma and endolymphatic sac tumor. To the authors' knowledge this is the first description of angiomatous lesions in the mucosa of the upper respiratory tract in a von Hippel-Lindau disease patient, a potentially useful finding for future radiological differential diagnosis in cases presenting with endolymphatic sac tumor.