APC and desmoid tumor: These include the second reported case of intracranial germ cell tumor in an APC carrier19, absence of profuse polyposis and early onset CRC in 3 of the 7 codon 1309 mutation carriers as is classically described20, attenuated phenotype with only 50 polyps at age 33 years in a codon 593 mutation carrier, desmoid tumor with codon 1228 mutation, papillary thyroid cancer with codon 1346 mutation and most interestingly CHRPE with codon 1483 mutation7, 8.