Peutz-Jeghers Syndrome Associated With Functioning Ovarian Tumor

Abstract

PEUTZ,¹in 1921, reported the association of skin pigmentation and small bowel polyps in several members of a family. Jeghers²added two such patients to the literature in 1944 and, in 1949 along with McKusick and Katz,³he established the syndrome as an entity which appears to be inherited as a mendelian dominant. Since that time additional cases have brought the reported number to somewhat over 100.⁴ An additional patient, a 41/2-year-old girl with the rare syndrome of mucocutaneous pigmentation and gastrointestinal polyposis, is presented below because of the associated presence of a granulosatheca cell tumor of the ovary with resultant precocious puberty. The interest aroused by the coincidence of these two rare disorders has been further stimulated by the isolated reports^5,6of two other cases of Peutz-Jeghers syndrome associated granulosa cell tumors. <h3>Report of a Case</h3> A 41/2-year-old girl was admitted for