Peutz-Jeghers Syndrome (PJS) is a rate autosomal dominant disorder characterized by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. The literatures have described that patients exhibiting PJS were considered to be at increased risk of malignant lesions of several organ systems. Patients with PJS have often associated with neoplastic changes of the uterine cervix and ovary, especially with well-differentiated cervical adenocarcinoma and ovarian sex cord tumor with annular tubules (SCTAT). This paper describes a case of PJS associated with cervical adenocarcinoma and SCTAT. The patient was a 30-year-old woman, who had been diagnosed as PJS 10 years ago after melanin spots was noted. She visited a local hospital with a complaint of abnormal vaginal bleeding in December, 1989. Cervical biopsy suggested invasive squamous cell carcinoma and radical hysterectomy was performed on January 12, 1990. Post-operative pathological diagnosis was well-differentiated cervical adenocarcinoma with squamoid differentiation and bilateral ovarian sex cord tumor with annular tubules (SCTAT). Review of the literatures indicated that PJS with cervical adenocarcinoma and SCTAT was very rare.
