Desmoid tumours:

Desmoid tumors are troublesome in about 10% of patients with FAP . They may be lethal in 10% of those affected. There is an association with particular APC mutations. It is apparantly necessary for both alleles of the APC gene to be knocked out in desmoids. They are are aggrevated by trauma and oestrogen.

From the management point of view desmoids can be divided into those in the abominal wall and those that are intra-abdominal. Resection is advised for abdominal wall desmoids but it is recommended to avoid surgery for intra-abdominal desmoids as this may be lethal and usually aggravates the desmoid rather than curing it.

Of interest, desmoids may respond to NSAID and anti-oestrogen drug therapy.