Henry Ford Hospital Medical Journal


In a large family with multiple endocrine neoplasia rype 2 A (MEN-2 A), 20 patients were identified by the diagnosis of medullary thyroid carcinoma (MTC) and/or pheochromocytomas. Another five subjects had neck surgery on the basis of slightly increased results of a C-cell provocative test. Retrospectively, however, although the immunohistochemical diagnosis of C-cell hyperplasia was confirmed, the diagnosis of MEN-2 was doubtful in these five subjects, and the C-cell hyperplasia observed was probably within the limits of normal variation. The occurrence of C-cell hyperplasia in a normal population was investigated by a C-cell provocative test, as well as in random postmortem material. An increased number of C-cells in normal subjects is fairly common and may represent a physiologic condition rather than a neoplastic phenomenon. Identification of early expression of MEN-2 is difficult on the basis of slightly increased C-cell-provocative test results. To avoid total thyroidectomy in normal subjects, it seems reasonable to postpone surgery until the peak stimulated level of pure extractable calcitonin increases to more than 1,000 pg/mL.