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CA: A Cancer Journal for Clinicians, Vol 29, 216-232, Copyright © 1979 by American Cancer Society

Hereditary Cancer: Ascertainment and Management

¹ Professor and Chairman, Department of Preventive Medicine/Public Health and the Oncology Clinic, Creighton School of Medicine, Omaha, Nebraska.
² Instructor, Department of Preventive Medicine/Public Health and the Oncology Clinic, Creighton School of Medicine, Omaha, Nebraska.
³ Assistant Professor, Departments of Surgery and Preventive Medicine/Public Health, Surgery, and the Oncology Clinic, Creighton School of Medicine, Omaha, Nebraska.
⁴ Surgical Oncology Fellow, American Cancer Society.
⁵ Professor and Chairman, Department of Surgery, Creighton School of Medicine, Omaha, Nebraska.
⁶ Instructor, Department of Preventive Medicine/Public Health, Creighton School of Medicine, Omaha, Nebraska.

The historical perspective provided by familial multiple adenomatous polyposis of the colon (Table 2) provides an excellent example of the advances in cancer epidemiology and characterization of hereditary precancerous disease(s), through multidisciplinary study over a long period of time. Concomitant advances in surgical management and prophylaxis have been witnessed. However, it has also been seen that even in this most intensively studied disorder(s), a great deal remains unknown about the variability in clinical features.

Those disorders lacking the readily discernable markers of the FPC, FAMMM and the MEA syndromes have been a particular concern in this discussion. Clinical research into these syndromes has been similar to the approach used in the study of FPC. This research has arrived at varying stages in the development of differential diagnostic and therapeutic protocols.

Due to the early ages of high-risk patients and their unfamiliarity with the role of genetic factors in cancer etiology, most would not otherwise be subject to screening for the diseases involved. A registry of such patients and their families could facilitate a greater recognition of their medical genetic significance. Our Registry-based program has clearly demonstrated the manner in which a carefully obtained family history can lead to the recognition of patients with exceedingly high cancer risk.

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