Extremely well-differentiated adenocarcinoma ("adenoma malignum") of the cervix in a patient with Peutz-Jeghers syndrome.

In a 29-year-old woman with the Peutz-Jeghers syndrome (PJS), an extremely well differentiated adenocarcinoma (adenoma malignum) of the uterine cervix was detected. The cervical lesion consisted of a polypoid mass, measuring 3.5 cm in greatest diameter, composed of extremely well differentiated tubules resembling those of the endocervical glands, yet containing a few Paneth cells. Immunohistochemical stains displayed cytoplasmic carcinoembryonic antigen in this tumor. The ovaries had no apparent abnormality. The diagnosis of the PJS was based on the presence of numerous hamartomatous polyps of the rectum and cutaneous pigmentation around the lips, fingers, and toes. The patient underwent a simple total hysterectomy and was subsequently treated with chemotherapy. In an 11 year follow-up, there has been no recurrence of the cervical tumor and she is currently well. The clinicohistopathologic differences of this cervical tumor in patients with and without PJS are briefly discussed.