An 18-year-old woman was diagnosed 7 years earlier with precocious pseudopuberty secondary to ovarian tumor. Physical examination reveals oral and lingual dark pigmentation. (See Figure below). Which of the following is the most likely diagnosis?
Correct Answer: Peutz-Jegher syndrome
Description: Intestinal polyposis is a possible indication of Peutz-Jegher syndrome associated with dark brown spots on the lips and palate. There is characteristic distribution of pigment around lips, nose, eyes, and hands. Tumors of the ovary, breast, pancreas, and endometrium are associated with this syndrome. The other syndromes listed do not have the characteristic pigment changes described in this patient.
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