DISSEMINATED CERVICAL ADENOMA-MALIGNUM AND BILATERAL OVARIAN SEX CORDTUMORS WITH ANNULAR TUBULES ASSOCIATED WITH PEUTZ-JEGHERS SYNDROME

Adenoma malignum is a highly differentiated mucinous adenocarcinoma of the cervix with a deceptively innocent histologic appearance but a hi ghly aggressive behavior. We describe a patient who had adenoma malign um associated with Peutz-Jeghers syndrome (PJS) and bilateral ovarian sex cord tumor with annular tubules (SCTAT). The relatively frequent o ccurrence of cervical adenoma malignum in women with PJS warrants clos e surveillance by gynecologists for early detection and treatment of t his cancer. We report the 16th known case of PJS with adenoma malignum and only the 8th known case of PJS with adenoma malignum and bilatera l SCTAT. The highly aggressive nature of cervical adenoma malignum is exemplified, and the difficulties associated with early diagnosis are discussed. The clinical significance of cervical and ovarian tumors as sociated with PJS is reviewed. (C) 1994 Academic Press, Inc.