Zung A, Shoham Z, Open M, Altman Y, Dgani R, Zadik Z
Pediatric Endocrine Unit, Kaplan Medical Center, Jerusalem, Israel.
Gynecol Oncol. 1998 Sep;70(3):421-4. doi: 10.1006/gyno.1998.5063.
Distinctive ovarian and cervical tumors are associated with Peutz-Jeghers syndrome (PJS). The most common gynecological tumors in this syndrome are adenoma malignum of the uterine cervix and ovarian sex cord tumor, particularly sex cord tumor with annular tubules (SCTAT). Other kinds 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 girl with PJS who presented with isosexual precocious puberty (IPP) due to ovarian lipid-rich Sertoli cell tumor. In addition to estrinizing effect of the tumor, the patient had decidual reaction secondary to tumor-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.
独特的卵巢和宫颈肿瘤与黑斑息肉综合征（PJS）相关。该综合征最常见的妇科肿瘤是宫颈恶性腺瘤和卵巢性索肿瘤，尤其是环状小管性索肿瘤（SCTAT）。其他类型的卵巢肿瘤很少有与PJS相关的报道，包括支持细胞瘤。我们报告一例4.5岁患有PJS的女孩，因卵巢富含脂质的支持细胞瘤出现同性性早熟（IPP）。除了肿瘤的雌激素化作用外，患者因肿瘤分泌孕酮继发蜕膜反应。本文回顾了PJS中性腺肿瘤的文献，包括之前一篇关于与该综合征相关的卵巢富含脂质支持细胞瘤的报道。
