Pineoblastoma presenting in familial adenomatous polyposis (FAP): random association, FAP variant or Turcot syndrome?

Brain tumours which arise in combination with adenomatous polyposis are usually astrocytic tumours or medulloblastomas. An adult women with a pineoblastoma associated with familial adenomatous polyposis is presented. In regard to the neuro-epithelial origin of this tumour we propose that it should be included in the second category of brain tumour polyposis syndrome.