Clear cell adenocarcinoma of the vagina and cervix. A report of the central netherlands registry with emphasis on early detection and prognosis

Abstract

The Central Netherlands Registry (CNR) of women with vaginal or cervical clear cell adenocarcinoma (CCAC) was established in 1985. An overview is presented of clinical and pathologic data of 55 patients who were registered at the CNR until July 1, 1988. All Netherlands Departments of Pathology (NDP) maintain a patient registry and 95% of the Institutes are connected with a Central Archive via a computer network. The histologic slides and clinical status were reviewed at the CNR. Twenty-five tumors were classified as vaginal carcinoma and 30 as cervical carcinoma. The mean age of the patients was 22 years. Fifty-five percent of patients (63% of patients with known maternal history) were exposed to diethylstilbestrol (DES) in utero. The majority of cases was initially diagnosed after 1980. Cytologic examination before the initial histologic diagnosis indicated that cervical tumors were detected in 80% of cases, but vaginal tumors were detected only in 33% of cases. It was concluded that an examination of DES-exposed women should consist of colposcopic inspection of the cervix and vagina, cytologic examination of the cervix and four quadrants of the vagina, and careful palpation of the cervix and the entire vaginal wall. The most important prognostic parameter for patients with CCAC was stage and grade of nuclear atypia. The results of a statistical analysis showed that these features were the most effective to distinguish between nonsurvivors and patients surviving more than 5 years.

Clear cell adenocarcinoma of the uterine cervix with malignant pleural effusion in a 29-year old female- A case report

“…The young age group is largely composed of woman exposed to diethylstilbestrol, whereas the latter is constituted mostly by those without history of exposure. 6 Several studies showed better prognosis in diethylstilbestrol induced carcinoma than those with spontaneous clear cell carcinoma. 7,8 The treatment of clear cell carcinoma is similar to that of cervical cancer.…”

A case of clear cell adenocarcinoma in the uterine cervix of 52-year-old virgin

“…The first peak occurs in women who are 17 to 37 years of age (mean age 26 years), and the second peak occurs in women who are 44 to 88 years of age (mean age 71 years). The former group includes most of DESexposed women, and the latter is constituted mostly by those without a history of DES exposure [3]. DES has been considered as a teratogen which crosses placental barrier [4].…”

“…Most women had early stage (stage I-II) disease and our patient was stage IIA. In a Dutch series of 88 women, 76 (88.5%) had stage I-II disease [3]. The primary treatment of early disease usually involved radical surgery with pelvic±para-aortic lymphadenectomy, with outcomes comparable to other types of cervical adenocarcinoma [14,15].…”

“…All local recurrences occur within 3 years of initial diagnosis. Recently, a familial chromosome translocation has been described involving chromosome 3 and 6 [46, XX, t(3:6)(q29;q23)] in mother and her DES-exposed daughter [3]. Human papillomavirus (HPV) type 31 DNA also has been identifi ed in CCAC, but the oncogenic potential of this HPV subtype is low and, hence, it is thought unlikely to be associated with the development of CCAC [10].…”

Characteristic Cytological Features of Cervical Clear Cell Adenocarcinoma: A Report of 4 Cases

“…Cervical cytology showed clusters of adenocarcinoma cells of unequal sizes in which the nuclear/cytoplasmic ratio was high ( Figure 1 and Table 1). Sheet-like arrangements of cervical CCAC has been advocated in various publications [10], and doubts about its diagnostic value have also been expressed. One study found that 25% of cases of CCAC presented with false-negative smears [11].…”