With increasing age at tumor diagnosis in familial cancer: Cancer is limited to fewer organs

Abstract

Hereditary cancer that has monogenic inheritance affects every tenth patient, on average, who is diagnosed with cancer, and it has been suggested, based on twin studies, that approximately 30% of all cancer patients have a genetic predisposition to developing cancer. In this article, the author posited that familial syndromes become more organ specific with increasing age at tumor presentation to the point that very late in life, only a few organs are affected by tumors. The reason for this could be that the tumor originates from a more differentiated, organ-specific progenitor/stem cell later in life, while the progenitor/stem cell might be involved in organogenesis in different organs earlier in life. Examples are given for skin cancer, colon, endometrial and breast cancer. Patients with familial cancer who present with cancer at an older age have a more organ-restricted disease. This could be because the tumor has a more differentiated progenitor/stem cell origin. Examples are given for families with breast cancer, melanoma, non-melanoma skin cancer, endometrial and colon cancer. (Less)