Peutz-Jeghers Syndrome Associated With Functioning Ovarian Tumor

C.D. Christian, MD, PhD; Thomas G. McLoughlin, MD; Edward R. Cathcart, MD; M. Michael Eisenberg, MD
JAMA. 1964;190(10):935-938. doi:10.1001/jama.1964.03070230071027.
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PEUTZ,1 in 1921, reported the association of skin pigmentation and small bowel polyps in several members of a family. Jeghers2 added two such patients to the literature in 1944 and, in 1949 along with McKusick and Katz,3 he 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 reports5,6 of two other cases of Peutz-Jeghers syndrome associated granulosa cell tumors.

Report of a Case  A 41/2-year-old girl was admitted for


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