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11/16. 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.
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12/16. Mesonephric adenocarcinoma of the uterine cervix with focal endocrine cell differentiation.

    A case of cervical adenocarcinoma arising in diffuse mesonephric hyperplasia is presented. The hyperplastic and neoplastic tubules showed focal endocrine cell differentiation. endocrine cells stained with the Grimelius technique, and were immunoreactive for chromogranin and serotonin. Antisera to additional specific hormones were negative. The natural history of this rare tumor is uncertain; the patient presented in this report is free of disease at 10-year follow-up.
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13/16. adenoma malignum of the uterine cervix detected by imaging methods in a patient with peutz-jeghers syndrome.

    adenoma malignum of the uterine cervix in a 25-year-old Japanese woman with peutz-jeghers syndrome (PJS) is described. A cervical multicystic mass was detected by CT scan, sonography, and MR imaging. These imaging findings strongly suggested the presence of adenoma malignum in spite of normal Pap smear, colposcopy, and cervical biopsy reports. Radical hysterectomy, bilateral salpingo-oophorectomy, pelvic lymphadenectomy, and para-aortic lymph node biopsy were performed after confirmation of adenoma malignum by conization. Both ovaries showed multicentric sex cord tumor with annular tubules. She is alive and well with no evidence of disease 23 months after surgery. Imaging methods including sonography, CT scan, and MR imaging may be useful aids in detecting the presence of adenoma malignum, especially in patients with PJS.
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14/16. Disseminated cervical adenoma malignum and bilateral ovarian sex cord tumors 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 highly aggressive behavior. We describe a patient who had adenoma malignum associated with peutz-jeghers syndrome (PJS) and bilateral ovarian sex cord tumor with annular tubules (SCTAT). The relatively frequent occurrence of cervical adenoma malignum in women with PJS warrants close surveillance by gynecologists for early detection and treatment of this cancer. We report the 16th known case of PJS with adenoma malignum and only the 8th known case of PJS with adenoma malignum and bilateral 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 associated with PJS is reviewed.
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keywords = tubule
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15/16. adenoma malignum of uterine cervix in peutz-jeghers syndrome: CT and US features.

    We report CT and ultrasound (US) features of adenoma malignum of the uterine cervix in a patient with peutz-jeghers syndrome (PJS) in whom bilateral ovarian mucinous cystadenomas and sex cord tumors with annular tubules were associated. adenoma malignum was shown as a hyperechoic mass mixed with multiple cysts on US and a low attenuated endocervical mass on CT. We think that imaging demonstration of an endocervical mass is important for the correct diagnosis of adenoma malignum in a female with PJS.
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16/16. peutz-jeghers syndrome: case presentation.

    The different aspects of the peutz-jeghers syndrome are discussed following a case presentation of a 36-year-old woman with peutz-jeghers syndrome, sex cord tumor with annular tubules of Scully, mucinous metaplasia of tubal epithelium, and adenocarcinoma of the endocervix. The latter two findings have not been described with the syndrome and an attempt is made to correlate them with peutz-jeghers syndrome.
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