True statement about Puetz Jegher syndrome is
Correct Answer: Increased risk of sex cord tumors
Description: Puetz Jegher syndrome
Autosomal dominant inheritence.
Jejunum is the most common site.
Caused by loss of function of STK 11.
Increased risk of sex cord tumours, small intestinal, pancreatic, breast, lung and ovarian cancer.
Category:
Pediatrics
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