MULTIPLE ENDOCRINE NEOPLASIA TYPE-1 ASSOCIATED WITH MULTIPLE LIPOMAS

Background: Multiple endocrine neoplasia type 1 (MEN-1) is characteriz ed by tumors of the parathyroids, the neuroendocrine pancreas-duodenum and the anterior pituitary, but shows also a wide clinical variety of other symptoms. Case Report: We present a case of a 68-year-old woman with a 18 year history of MEN-1 consisting of gastrinoma and primary hyperparathyroidism. Beside these typical symptoms, the patient suffer ed from thyroid adenoma, malignant kidney tumor and multiple subcutane ous lipomas. Result: While the number of autopsies declined from 113 i n 1977 to 66 in 1984, the number of diagnostic techniques used increas ed continuously (94, 107, 118 and 140, amounting to 0.83, 1.34, 1.76 a nd 2.12 per patient). The pre-mortal detection of abdominal abnormalit ies increased globally from 16.8 to 32.5%. This increase was largely d ue to better diagnosis of liver and gallbladder abnormalities which we re in most cases of little relevance. Conclusion: Whether these non-en docrine tumors are associated to MEN-1 is unclear and has to be tested by examining the chromosomal regions 11q13 and 11q24/25 of the tumors sample, in which the possible MEN-1 involved tumor suppressor genes a re located.