SERTOLI-CELL TUMOR CAUSING PRECOCIOUS PUBERTY IN A GIRL WITH PEUTZ-JEGHERS SYNDROME

Distinctive ovarian and cervical tumors are associated with Peutz-Jegh ers syndrome (PJS). The most common gynecological tumors in this syndr ome are adenoma malignum of the uterine cervix and ovarian sex cord tu mor, particularly sex cord tumor with annular tubules (SCTAT). Other k inds of ovarian tumors have been rarely reported in association of PJS , including Sertoli cell tumors. We report a case of a 4.5-year-old gi rl with PJS who presented with isosexual precocious puberty (IPP) due to ovarian lipid-rich Sertoli cell tumor. In addition to estrinizing e ffect of the tumor, the patient had decidual reaction secondary to tum or-derived progesterone secretion. The literature on gonadal tumors in PJS is reviewed, including one previous report of ovarian lipid-rich Sertoli cell tumor associated with this syndrome. (C) 1998 Academic Pr ess.