Tumor variation in the cancer family syndrome: Ovarian cancer

The Cancer Family Syndrome is a hereditary disorder (autosomal dominant), characterized by early onset cancer of the colon (particularly the proximal colon) and endometrium, with an excess of multiple primary cancers. Recent evidence reflects the possibility of an even broader tumor spectrum consisting of carcinoma of the stomach, the ovary, the kidney, and possibly other organs. A family with the cardinal features of the Cancer Family Syndrome is described, including two sisters and their mother who had ovarian carcinoma at early ages (38, 46, and 49 years). Two of these three women have shown unusual tolerance to cancer, despite invasion of the primary tumors. Implications for cancer surveillance and management are discussed.