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Allelic Loss on Chromosome 11 in Hereditary and Sporadic Tumors Related to Familial Multiple Endocrine Neoplasia Type 1¹

Department of Human Genetics, Yale University, New Haven, Connecticut 06510 [A. E. B., E. L. W., J. S. F.]; Surgery Branch, National Cancer Institute, [J. A. N.], Digestive Diseases Branch [P. N. M.], Metabolic Diseases Branch [E. S., G. D. A., S. J. M.], and Molecular Pathophysiology Branch [A. M. S, E. F.], National Institute of Arthritis, Diabetes, and Digestive and Kidney Diseases; and Surgical Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke [E. H. O.], NIH, Bethesda, Maryland 20892; Department of Molecular Biology and Genetics, Wayne State University, Detroit, Michigan 48201 [R. T. T.]; and Department of Physiopathology, University of Florence, 50139 Florence, Italy [M. L. B.]

Familial multiple endocrine neoplasia type 1 (FMEN1) is an autosomal dominant disorder characterized by tumors of the parathyroid glands, pancreatic islets, and anterior pituitary. The gene for this disease maps to chromosome 11q12–11q13, and allelic loss in this region has been shown in both sporadic and FMEN1-related parathyroid tumors, FMEN1-related pancreatic islet tumors, and rarely in sporadic anterior pituitary tumors. We tested for allelic loss at 7 loci on chromosome 11 in 17 tumors outside the parathyroid. We found loss of heterozygosity in 2 of 2 FMEN1-related benign pancreatic islet tumors but in none of 8 informative sporadic islet tumors (P = 0.02) including 5 malignant gastrinomas. Of 3 islet tumors from patients who had some but not all features of FMEN1, one showed alleic loss for 5 of 5 informative restriction fragment length polymorphisms, and the other 2 retained heterozygosity for all informative markers. A bronchial carcinoid from an FMEN1 patient and 3 sporadic anterior pituitary tumors showed no allelic loss. These data provide new evidence that many sporadic pancreatic islet neoplasms, even when malignant, do not develop through homozygous inactivation of the MEN1 gene.

¹ This investigation was supported by USPHS Grant CA-50497, the Swibelius Cancer Research Award, and the Charles E. Culpepper Award.

² To whom requests for reprints should be addressed, at Department of Human Genetics, Yale University School of Medicine, Box 3333, New Haven, CT 06510.

Received 8/17/90. Accepted 12/ 6/90.

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