Alias: Familial pancreatic cancer Inheritance It has been estimated that as many as 10% of pancreatic cancers have a hereditary basis; five genetic syndromes have been identified that are associated with the familial aggregation of pancreatic cancer; these include: The second breast cancer syndrome (BRCA2), the familial atypical multiple mole melanoma (FAMMM), the Peutz-Jeghers Syndrome, the he...

Peutz Jegher’s syndrome is a mucocutaneous autosomal dominant condition associated with extensive melanin pigmentation and intestinal hamartomatous polyp formation. It is characterised by round, oval patches of blackish brown pigmentation, irregularly distributed over the oral mucosa, lips, tongue and the hard palate. This condition is associated with an increased risk towards carcinoma and sev...

We present a rare case of Peutz-Jeghers(P-J) syndrome in a 29-year-old woman who developed adenocarcinoma of the thyroid and polyps in the gastrointestinal tract. Polyps removed from the stomach, colon and ileum showed features typical of hamartomas. Histological examination indicated that the tumor resected from the right lobe of the thyroid was a papillary adenocarcinoma. This appears to be t...

The Peutz-Jeghers syndrome (PJS) is an autosomal-dominant hamartomatous polyposis syndrome characterized by mucocutaneous pigmentation, gastrointestinal polyps and the increased risk of multiple cancers. The causative point mutation in the STK11 gene of most patients accounts for about 30% of the cases of partial and complete gene deletion. This is a report on a girl with PJS features, learning...

Carney complex is a multiple endocrine neoplasia (MEN) syndrome that affects the adrenal cortex, the pituitary and thyroid glands, and the gonads. The complex is also associated with skin and mucosa pigmentation abnormalities and myxoid and other neoplasms of mesenchymal and neural crest origin. Thus, this syndrome also belongs to another group of genetic disorders, the lentiginoses (or lentige...

Peutz-Jeghers syndrome (PJS) is an extremely rare autosomal dominant hereditary disease characterized by the growth of hamartomatous polyps in gastrointestinal tract, mucocutaneous pigmented macules and increased risk malignant neoplasms various localizations. In most cases development PJS associated with presence a mutation STK11 gene, but not all patients have this mutation. This review prese...