Through nationwide surveys, we collected and analyzed 242 patients of medullary thyroid carcinoma (MTC). Included were 40 patients with multiple endocrine neoplasia type 2A (MEN-2A), six patients with MEN-2B, and 36 patients with only MTC having a positive family history (82 total patients in the hereditary group). Ten-year survival rates were 81.5% for all cases, and 97.5% and 76.1% for the hereditary and the sporadic group, respectively. Epinephrine/norepinephrine ratio in the urine was found to be a good indicator of the adrenomedullary hyperfunction in patients with hereditary MTC. At least one patient in each family with hereditary MTC had overt pheochromocytoma or latent hyperfunction of adrenal medulla. Doubling times of plasma calcitonin levels correlated with life expectancy and recurrence rates. We carried out linkage analysis between the MEN-2 locus and several genetic markers in our MEN-2 families, but so far we have been unable to demonstrate or exclude a linkage.
Takai, Shin-ichiro; Miyauchi, Akira; Matsumoto, Hideo; Ikeuchi, Tatsuro; Miki, Tetsuro; Kuma, Kanji; and Kumahara, Yuichi
"Multiple Endocrine Neoplasia Type 2 Syndromes in Japan,"
Henry Ford Hospital Medical Journal
: Vol. 32
Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol32/iss4/10