EPAS1 and somatostatinoma: Overall clinical manifestations and their frequency in the 62 published cases with HIF2A mutations are as follows: isolated polycythemia in 29 patients (45.0%); polycythemia and PHEO/PGL in nine patients (14.5%); polycythemia, PHEO/PGL, and somatostatinoma in six patients (9.6%); isolated PHEO/PGL in 14 patients (22.6%); brain hemangiomas in three patients (4.8%, one with a concomitant PGL); and duodenal gangliocytic PGL in two patients (3.2%) [50, 72–82].