Peutz--Jeghers syndrome with pseudoinvasion of hamartomatous polyps and multiple epithelial neoplasms.
The risk of malignant change developing in the hamartomatous polyps in Peutz-Jeghers syndrome is widely held to be negligible. However an association with tumours of the upper gastro-intestinal tract, ovary and other diverse multiple neoplasms is now recognized. Previously reported cases of malignant change in Peutz--Jeghers polyps may represent 'pseudoinvasion' and we report such a case. This was associated with carcinomas of the bile ducts, left tonsil and a papillary adenoma of the pancreatic duct. It lends further support to the view that there may be a genetic predisposition to the development of neoplasms in this condition. Pathologists and clinicians must be aware of this entity of 'pseudoinvasion' in order to avoid unnecessarily radical surgery in Peutz-Jeghers syndrome.
D000230:Adenocarcinoma / Q000473:pathology; D001650:Bile Duct Neoplasms / Q000473:pathology; D002294:Carcinoma, Squamous Cell / Q000473:pathology; D003537:Cystadenoma / Q000473:pathology; D006222:Hamartoma / Q000473:pathology*; D006801:Humans; D007414:Intestinal Neoplasms / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010183:Pancreatic Ducts; D010190:Pancreatic Neoplasms / Q000473:pathology; D010580:Peutz-Jeghers Syndrome / Q000473:pathology*; D014067:Tonsillar Neoplasms / Q000473:pathology