Cancer Research 65, , , . doi:
© 2009 American Association for Cancer Research
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Figure 1. Radiologic, morphologic, and immunohistochemical features of ELSTs. Serial magnetic resonance and computed tomography imaging of the temporal region from a VHL patient with hearing loss showing the development of a left endolymphatic sac tumor. A, axial, T1-weighed enhanced magnetic resonance imaging reveals enhancement in the region of the left endolymphatic duct (arrows). B, corresponding axial, nonenhanced computed tomography of the left temporal bone shows bony erosion confined to the left endolymphatic duct (arrowhead). Morphologic spectrum of ELSTs. Papillary structures were observed in all tumors (C), whereas cystic areas (D) were seen in half of the cases. E, one tumor had areas of epitheloid clear cell clusters, reminiscent of clear cell renal carcinoma. F, extensive hemosiderin deposits were evident in half of the tumors. G, a feature of all tumors was intensive vascularization (immunohistochemistry with anti-CD 34). Note the abundant vessels in papillary stroma (arrows) and the immediate contact of numerous small vessels with the cystic epithelium (arrowheads), which seems to be induced by expression of HIF and vascular endothelial growth factor by the epithelial tumor cells. Immunohistochemistry for NSE (H), MAK6 (I), and AE1/AE3 (J) was consistently positive.
