Figure 1

A paraganglioma from patient 10 (a) was composed of a nested to whorled arrangement of cells with amphophilic cytoplasm. The paraganglioma cells exhibited absence of immunohistochemical staining for SDHB (b), whereas endothelial cells and other stromal cells exhibited normal positive labeling for SDHB. An oncocytoma from patient 2 (c) was resected after the SDH-deficient RCC. In contrast to the SDH-deficient RCC, normal SDHB labeling was present in the oncocytoma (d).