In 2006, a review from the European Network for the Study of Adrenal Tumors (ENS@T) summarized germline variants (VHL, SDHB, SDHD, RET, and NF1) in 166 (25.9%) of 642 PPGL patients and VHL was reportedly the most frequently mutated gene (56/642, 8.7%) (8). The gene discussed is VHL; the disease is adrenal gland neoplasm.