The identification of an increasing variety of ''cancer genes,'' there
by signifying the cancer destiny of patients from hereditary cancer fa
milies, offer numerous opportunities for cancer surveillance and manag
ement, including prophylactic surgery in selected circumstances. This
new science and technology has emerged at a rapid pace, making it diff
icult for many physicians to grasp its full impact. This article revie
ws the genetics and molecular genetics of cancer and updates its genet
ic counseling implications. Several hereditary cancer syndromes are re
viewed as cancer genetic models, including multiple endocrine neoplasi
a types 2A and 2B, neurofibromatosis, familial pancreatic carcinoma, f
amilial atypical multiple mole melanoma syndrome, and von Hippel-Linda
u disease. Special attention is given to the distinctive pathology fea
tures that may appear in certain hereditary cancer syndromes, with her
editary nonpolyposis colorectal carcinoma discussed as a prototypic mo
del. Genetic testing issues including variable positions relevant to w
hether and/or when to test and whom to test, as well as the position o
f the American Society of Clinical Oncology, are also discussed. (C) 1
997 American Cancer Society.