Document gLQk9xBEEKzOnZ9qMLQ4Zb0q

HFM -003514 ' Inheritance Clinic*! and pathological feature* Thyroid . Adrenal medulla Adrenal cortex Parathyroid Pancraet Pituitary Other phenotypic features seal Type 1 (Warmer)3 TypeII. (Sipple)31 Autosomal dominant with high degree of penetrance. Thyroid disorder In 20% usually adenoma, but may Include differentiated carci noma (not medullary), col loid goiter, thyroiditis or thyrotoxicosis. Autosomal dominant with high degree of penetrance. v Medullary carcinoma, frequently multifocal. Elevated serum calcitonin with exaggerated response to calcium or glucagon infusion may facilitate . diagnosis of carcinoma or C-cell hy- i; perplatia. Increased serum and tissue hlstamlnase activity can serve as bio-. . chemical marker for primary and mar" tastatic carcinoma. (Hlstamlnase Is found normally in human intestine, kidney end placenta.) Ectopic pro duction of serotonin and prostaglandint may give rise to carcinoid end dirrheal syndromes, respectively. . Pheoehromocytoma may be bF laterti. Diffuse or local hyper plasia may precede tumor forma tion. Adenoma, diffuse hyperpiasla or carcinoma. Cushing's syndrome may be secondary to ectopic secre tion of.ACTH. Aldocteronoma. Diffuse hyperplasia secondary to ACTH secretion by medullary thy roid carcinoma. Hyperparathyroidism due to adenoma or hyperplasia in most patients. Less common and characteristical ly hyperplasia rather th8n adeno ma. More likely a secondary responseto the hypocalcemfc action of calcitonin, than an expression of genetic plelotropism. Adenoma, hyperplasia (microadenomatosis) or car cinoma of non-beta islet cells. Accompanied frequently by elevated fasting serum gastrin and intractable peptic ulcer diathesis (Zoil)nger-EiHson syndrome). Glucagon and in sulin-secreting adenomas have also.been described. Adenomas in about 65% of patients. Frequently non functional, but may give rise to acromegaly or ForbesAlbrlght syndrome (amenor rhea and galactorrhea)! Bronchia) and Intestinal car Multiple mucosal neuromas of lips, cinoid tumors, multiple tongue, eyelids, segmental gang- lipomas, schwannomas and lloneuromatosls of large intestine thymomas. resulting In megacolon, neurofibro mas, cafe au lait spots and marfBn- oid body habitus. These pheno typic features in conjunction'with medullary carcinoma and pheo- -, chromocytoma are' now designa 0912 _ ted MEA III. patients, or four times that expected. The eoceTRH secretion.MittraW-** described, observed risk of thyroid cancer in women a prolactin-thyroxin antagonism in the : with breast cancer was statistically signi rat whereby, in the absence of thyroid ficant at the one percent level. The inci- - hormones, the maminotrophic effjjct of dence of breast cancer in 827 patients with endogenous prolactin was enhanced; thyroid cancer, although not statistically significant, was 0$ per year per 1,000 patients or 1.3 times expectation. The magnitude of increase in . the incidence of thyroid cancer in our breast cancer patients did not exceed that observed throughout our population of hospital cancer registry patients. We observed a 5.7-fold excess of second primary carci nomas of the thyroid in 41,341 cancer patients and after 123,531 person-years of foilow-up. Because the incidence of breast cancer was not seen to increase signifi cantly in thyroid cancer patients, and be cause of the generally observed increase of second primary thyroid cancers, we were unable to determine if a common etiology for both breast and thyroid can cer might have existed. Prolactin is a sustaining factor in the growth of mammary carcinoma in some laboratory animals. For example, in the absence of the ovaries and adrenals, pro lactin alone can maintain the growth of an existing mammary carcinoma in the rat. The role of prolactin in human breast cancer is less certain. Significant prolactin stimulation occurs during pregnancy and lactation, yet pregnancy before age 30 is relatively protective against breast cancer when compared with the risk noted in nulliparous women or in women whose first pregnancy is after age 30, and neither the duration nor the frequency of lactation is significantly correlated with the risk of breast cancer.*9Blood prolactin concen trations are not consistently aberrant in women with breast cancer, although ele Experimentally, ionizing radiation and * vated prolactin, estradiol and estrone hypothyroidism augment tumorigenesis levels have been detected in the daughters in the thyroid and breast. Previous case of breast cancer patients.90 control studies of women with breast can Mittra and Hayward86 studied the role cer that used conventional tests of thyroid of the thyroid in breast cancer by assessing function failed to provide unequivocal the adaptive alterations in the hypothal- evidence that hypothyroidism predisposes amic-pituitary-thyroid axis. By measuring to breast cancer.83 Although a positive levels of TSH before and after TRH;Stim correlation has been suggested for coun ulation; they observed evidence of hypo tries at increased risk of endemic goiter thyroidism in 10 percent of women with and breast cancer mortality, a simitar cor early breast cancer, 14 percent with ad relation is not evident between thyroid vanced breast cancer, and in none of their cancer and breast cancer incidence. For age-matched hospital controls. The plas example, the Chinese women living in ma concentration ofTSH was significantly Hawaii do not exhibit an increased risk of higher in patients with breast cancer. breast cancer. Although a study in Japan These recent studies are of interest if we suggested that the risk of breast cancer recall that Sommers in 1955 reported in a was significantly increased in women with controlled necropsy study that pituitary Hashimoto's thyroiditis, this was not con amphophil . hyperplasia ("thyrotropic firmed, at least in the population studied basophils") and thyroid atrophy were at the Mayo Clinic. **. present with significantly greater frequen Thyrotropin-releasing hormone (TRH) from the hypothalamus is not only required for the normal synthesis and secretion of thyroid stimulating hormone: (TSH), but also stimulates the secretion cy in breast cancer patients. Thyroid atro phy was interpreted as the most significant anatomical alteration in explaining an "endocrine imbalance that predisposed to breast cancer."91 of prolactin. The concentration ofplasma- ' The role of the thyroid in breast cancer free thyroxine regulates the responsive-- is a question that has been pursued since ness of TSH to TRH and may also influ- at least the time of Beatson in 1896. " VOL.28. NO. 2 MARCWfAPRIL1?78 *00.1 0913