Possible Considerations for the Management of Turcot’s Syndrome?

Abstract

Background: Turcot’s syndrome (TS) is a rare disease with known incidence of about 1-2 cases per year. It is, however, linked to high mortality due to the brain cancer. And because of this, we propose recommendations, aimed at preventing the mortality of the patients and to minimize the risk of undiagnosed Turcot’s syndrome. Methods: The authors collected the worldwide published data on TS, from the year of its definition till 2018, all of which was published on the search engines, such as Medline, Medknow, Cohraine and Wiley. Results: We included 97 patients, 57 from which are females and 40 males with median age of 22 years. The most common type of cancer is medulloblastoma, followed by glioblastoma and astrocytoma. We further divided the patients into two categories based on the first symptom of the disease and we made an algorithm of approaching these patients. Conclusion: TS is a disease that affects mostly members of families with multiple genetic mutations and types of cancers. And because of the unknown mechanisms of inheritance, it is useful to establish guidelines for the approach of those patients, in order to minimize the high mortality rate.