Peutz-Jegher Syndrome PJS



- It's inherited in an Autosomal Dominant pattern

- Due to mutation in STK11 gene [ also known as LKB1 ] which is tumor suppressor gene. 

       →   its mutations disrupting its ability to restrain the cell division       
 

- Characterestic by development of non-cutaneous growths called hamartomatous polyps in the 

   gastrointestinal tract particulary stomach and intestine 

- The polyps have a great risk of develop cancer



features

   childern with :-
  
            * Small dark-colored spots on the lips, around and inside the mouth, near the eya, nostril and 
 
               around the anus. also may found on hands and feet and often fade as the person gets older 

           * Multiple polyps in the stomach and intestine during the childhood and adolescnce 

           * Polyps may cause recurrent bowel obstructions, chronic bleeding and abdominal pain

           * The patient has the risk of developing types of cancers as 

                    " GIT    " Pancreas       " Cervix      " Ovary      " Breast


The patient diagnosed by clinical picture