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Molecular Pathogenesis Unit, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, Maryland 20892 [S. C. H., A. O. V., S. D. P., U. D. L., P. M., I. A. L., Z. Z.]; Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Disease, NIH, Bethesda, Maryland 20892 [C. A. K., G. P. C., K. P.]; and University of Texas, M. D. Anderson Cancer Center, Houston, Texas 77030 [R. F. G.]
Inherited mutations of the RET proto-oncogene are tumorigenic in patients with multiple endocrine neoplasia type 2 (MEN 2). However, it is not understood why only few of the affected cells in the target organs develop into tumors. Genetic analysis of nine pheochromocytomas from five unrelated patients with MEN 2 showed either duplication of the mutant RET allele in trisomy 10 or loss of the wild-type RET allele. Our results suggest a "second hit" causing a dominant effect of the mutant RET allele, through either duplication of the mutant allele or loss of the wild-type allele, as a possible mechanism for pheochromocytoma tumorigenesis in patients with MEN 2.
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