Endolymphatic Sac Tumors: MR Imaging Findings in Two Siblings

Endolymphatic sac tumors are aggressive papillary tumors of the temporal bone that could be associated with von Hippel-Lindau disease (VHL). We present imaging findings of a VHL family with two cases of endolymphatic sac tumors. These tumors spread to the cerebellum and the cerebellopontine cistern and may mimic more frequent tumors of this region. They show predominantly high signals on T1- and T2-weighted images and enhance heterogeneously. There are some difficulties in its differential diagnosis from other vascular and nonvascular tumors of the temporal bone. Increased signal intensity at unenhanced T1-weighted MR imaging is common and may help distinguish these lesions from more common aggressive temporal bone tumors.