Kliniko-geneticheskoe issledovanie papillyarnogo i follikulyarnogo raka shchitovidnoy zhelezy

Hereditary predisposition to thyroid cancer (TC) was studied in 40 patients with this condition, out of whom 74% were observed to have papillary cancer; papillary-follicular and follicular cancers were found in 23 and 3%, respectively; and their 912 first-degree relatives. For the convenience of analysis, these histological forms were integrated into nonmedullar TC (NMTC). The risk of NMTC was ascertained to be higher in women than that in men (the male/female ratio was 1:5). The age at disease diagnosis averaged 47.7% years; however, in 78% of the patients the first signs of the disease occurred an average of 6-7 years earlier. There was a significant trend for various organs to be involved; the rate of primary-multiple malignant neoplasms were 5.4%, by exceeding the similar rate in the population manifold (0.003%). Analysis of the relatives' diseases revealed that 4% of them were affected by various neoplasias and 3.2% were by various benign thyroid tumor and nontumor diseases. NMTC was found to have a family component. The age at the manifestation of familial papillary TC (32.1% years) was much less than that in the total sample of patients with NMTC, indicating a severer phenotype. The detected familial cases of papillary TC were referred to as familial papillary TC, in which only thyroid tissue is involved. Papillary and follicular TC may be a component of hereditary syndromes. It was suggested that there were general regularities in tissue lesions of the thyroid, kidney, breast, skin (melanoma), nervous system, and large bowel in the family of patients with NMTC. Recommendations are proposed for the management of hereditary TC forms.

papillary and follicular (nonmedular) thyroid cancer, hereditary forms, genetic heterogeneity, associations