Recent Advances in Genetics, Diagnosis, Localization, and Treatment of Pheochromocytoma

Figure 1. Diploid cells have two copies of each gene. A tumor suppressor gene, such as the von Hippel–Lindau gene, is consistent
with the Knudson model. In the hereditary form of cancer, the patient inherits a mutation of one copy of the gene. In the
tumor, such as a pheochromocytoma in a patient with von Hippel–Lindau disease, the second copy is inactivated by a mechanism
such as mutation or deletion.
The Knudson two-hit model.
-
Ann Intern Med
February 20, 2001
vol. 134
no. 4
315-329