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Multiple Endocrine Neoplasia Type 1: A 10-Year Prospective Screening Study in Four Kindreds^*

Department of Internal Medicine, University Hospital Uppsala, Sweden
Department of Clinical Chemistry, University Hospital Uppsala, Sweden
Department of Diagnostic Radiology, University Hospital Uppsala, Sweden
Department of Surgery, University Hospital Uppsala, Sweden
The Ludwig Institute for Cancer Research, Uppsala Branch Uppsala, Sweden

Address all correspondence and requests for reprints to: Britt Skogseid, M.D., Ph.D., Department of Internal Medicine, University Hospital, S-75185 Uppsala, Sweden.

A total of 80 individuals in 4 kindreds with multiple endocrine neoplasia type 1 (MEN 1) have been subjected to repeated biochemical screening during a 10-yr period with the principal aim being to analyze characteristics of the developing pancreatic lesion. Age at presentation of the MEN 1 trait averaged 18 yr in 7 previously unaffected individuals, and this effect of the screening procedure represented a lowering by almost 2 decades. Pancreatic endocrine involvement was recognized at a mean age of 25 yr and constituted the presenting lesion in a majority of the patients. A standardized meal test and basal values of serum pancreatic polypeptide, insulin, proinsulin, and gastrin were the most efficient markers for the pancreatic lesion and preceded signs of pancreatic tumors upon radiological examinations by a mean of 3.5 yr. A 75% penetrance of the islet cell disease and 90% for primary hyperparathyroidism within the affected individuals equalled the prevalences reported in autopsy studies. Two of the kindreds showed signs of intrafamilial homogeneity with respect to the profile of peptide excess (P < 0.05) and considerable discrepancy in the malignant potential of the pancreatic lesions. The results of early detection and surgical intervention of the pancreatic tumors in MEN 1 suggested an impact on morbidity, while any effect on the mortality of these individuals remains to be clarified.

^* This work was supported by Swedish Cancer Foundation Grant 1925.

Received July 16, 1990.

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