Which condition is characterized by pigmented macules on the lips and is associated with an increased risk of malignancies?

Peutz-Jeghers syndrome is a genetic condition that is characterized by the presence of pigmented macules on the lips, as well as on the mucous membranes and skin. These pigmented spots typically appear during childhood or adolescence. One of the significant concerns associated with Peutz-Jeghers syndrome is its association with an increased risk of various malignancies throughout an individual's life, particularly gastrointestinal cancers, such as colorectal cancer, as well as cancers of the pancreas, breast, and other organs.

The underlying genetic cause of Peutz-Jeghers syndrome is a mutation in the STK11 gene, which plays a role in controlling cell growth and division. The presence of these pigmented macules serves as an important clinical marker for healthcare providers to recognize the syndrome and the associated cancer risks early in the patient's life.

In contrast, other conditions listed, while they may also be linked to cancer risks, do not present with the distinct feature of pigmented macules on the lips. For instance, hereditary nonpolyposis colorectal cancer (Lynch syndrome) primarily involves increased risks of specific cancers but lacks the characteristic pigmentation. Familial adenomatous polyposis is identified by numerous polyps in the colon and rectum without the pigmentation