Gender-Specific Aspects of Lynch Syndrome and Familial Adenomatous Polyposis.
BACKGROUND
The most frequent hereditary colorectal cancer (CRC) syndromes are Lynch syndrome and familial adenomatous polyposis (FAP), accounting for approximately 5% of the CRC burden. Both are characterized by an autosomal dominant mode of transmission and require an individualized approach of intensified screening and prophylactic surgery.


METHODS
In this review, we provide an overview of the literature regarding gene- and gender-specific aspects of Lynch syndrome and FAP. Based upon available data, a personalized approach when treating patients and families with these predisposition syndromes is increasingly warranted.


RESULTS
In Lynch syndrome patients, men have a significantly higher lifetime risk and earlier age of manifestation for CRC - especially in MSH6 mutation carriers. Moreover, incidence of gastric, bladder, and urothelial cancers is much higher in males. Females with an MSH6 mutation have to be aware of a very high risk especially for endometrial, but also for ovarian cancer. In FAP families, females are more prone to papillary thyroid cancers and also to desmoid tumors.


CONCLUSIONS
It is timely to suggest gene- and gender-based adapted screening and surgical recommendations for Lynch syndrome patients. In FAP patients, females should follow intensified screening recommendations for early detection of precursors or papillary cancer of the thyroid. Moreover, desmoid patients should primarily not be subjected to surgical resection but rather to a gender-specific conservative medical treatment.
Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Germany.
Ralph
R
Schneider
Department of General and Visceral Surgery, HELIOS St. Josefs-Hospital, Bochum-Linden, Germany.
Claudia
C
Schneider
Department of Gastroenterology, HELIOS St. Josefs-Hospital, Bochum-Linden, Germany.
Christian
C
Jakobeit
Department of Surgery, Caritas-Hospital St. Josef, Regensburg, Germany.
Alois
A
Fürst
Department of General and Visceral Surgery, HELIOS St. Josefs-Hospital, Bochum-Linden, Germany.
Gabriela
G
Möslein
FAP; Familial adenomatous polyposis; Gender-specific aspects; Lynch syndrome; Surgery