Which of the following is not a feature of Peutz – Jegher Syndrome ?
**Core Concept**
Peutz-Jeghers Syndrome (PJS) is a rare genetic disorder characterized by the development of hamartomatous polyps in the gastrointestinal tract and an increased risk of various cancers. It is caused by mutations in the STK11 gene, which encodes a serine/threonine kinase involved in cell cycle regulation and tumor suppression.
**Why the Correct Answer is Right**
The correct answer is a feature that is not typically associated with Peutz-Jeghers Syndrome. To determine this, we must consider the characteristic features of PJS, which include:
* Gastrointestinal polyps, particularly in the small intestine
* Mucocutaneous melanin deposits, leading to characteristic blue or brown macules on the skin and mucous membranes
* Increased risk of various cancers, including gastrointestinal, breast, and ovarian cancers
* Other systemic features, such as developmental abnormalities and metabolic disorders
**Why Each Wrong Option is Incorrect**
**Option A:** Gastrointestinal polyps are a hallmark feature of PJS, so this option is incorrect.
**Option B:** Mucocutaneous melanin deposits are a characteristic feature of PJS, making this option incorrect.
**Option C:** Increased risk of various cancers is a well-established feature of PJS, so this option is also incorrect.
**Clinical Pearl / High-Yield Fact**
To remember the characteristic features of Peutz-Jeghers Syndrome, use the mnemonic "G-M-P-C," which stands for Gastrointestinal polyps, Mucocutaneous melanin deposits, Polypoid lesions, and Cancer risk.
**Correct Answer:** D.