Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Medline ® Abstract for Reference 59

of 'Clinical features, diagnosis, and management of von Hippel-Lindau disease'

Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients.
Mukherji SK, Albernaz VS, Lo WW, Gaffey MJ, Megerian CA, Feghali JG, Brook A, Lewin JS, Lanzieri CF, Talbot JM, Meyer JR, Carmody RF, Weissman JL, Smirniotopoulos JG, Rao VM, Jinkins JR, Castillo M
Radiology. 1997;202(3):801.
PURPOSE: To determine the computed tomographic (CT), magnetic resonance (MR) imaging, and angiographic findings of papillary endolymphatic sac tumors.
MATERIALS AND METHODS: Clinical and imaging studies in 20 patients (aged 17-65 years) with histopathologically proved papillary endolymphatic sac tumors were retrospectively reviewed. Patients underwent CT (n = 18), MR imaging (n = 15), or angiography (n = 12). CT scans were evaluated for bone erosion and calcification; MR images, for signal intensity, enhancement patterns, and flow voids; and angiograms, for tumoral blood supply.
RESULTS: All tumors were destructive and contained calcifications centered in the retrolabyrinthine region at CT. The MR imaging appearance varied with lesion size; 12 of 15 tumors showed increased signal intensity at T1-weighted imaging. The high-signal-intensity area was circumferential in lesions 3 cm or smaller and was scattered throughout the lesion in advanced tumors. Only tumors larger than 2 cm had flow voids. The blood supply arose predominantly from the external carotid artery. Large tumors had additional supply from the internal carotid and posterior circulation.
CONCLUSION: Papillary endolymphatic sac tumors are destructive, hypervascular lesions that arise from the temporal bone retrolabyrinthine region. Increased signal intensity at unenhanced T1-weighted MR imaging is common and may help distinguish these lesions from more common, aggressive temporal bone tumors.
Department of Radiology, University of North Carolina School of Medicine, Chapel Hill 27599-7510, USA.