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 carci-noembryonic antigen in this tumor. The ovaries had no apparent abnormality. The diagnosis of the PJS was based on the presence of numerous hamarto-matous 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.