A young woman with vagina cancer exposed to clinical case diethylstilbestrol and review of the literature. We analyzed the case of a 20-year-old patient, who was exposed in utero to diethylstilbestrol, as a probable predisposing factor to manifest vaginal cancer. The diagnostic pillar, in addition to the family history, was the taking of incisional biopsy, with histopathological report of clear cell carcinoma, stage III, extended to the pelvic wall, with metastasis to the lymph nodes. regional and absence of distant metastasis. Surgical management was decided with protocolized laparotomy, peritoneal lavage, retroperitoneal biopsies and radical hysterectomy, Piver III, with involvement of two thirds of the vagina. Factors of poor prognosis were coadjuvant management with systemic chemotherapy.
The objectives of this article are: to analyze the case of a patient with vaginal cancer who was exposed in utero to diethylstilbestrol, as a probable predisposing factor, and discuss the treatment, aggressive but necessary, for a young woman with a high risk of relapse adenocarcinoma of clear vaginal cells.
A 20-year-old female patient who started her condition three months before her first medical visit. She manifested opsomenorrheal menstrual cycles and moderate intensity abdominal pain, colic, of a month of evolution. It was attended by a particular gynecologist, who detected a profuse tumor that was seen in the vaginal introitus. His gynecological and obstetric history were: menarche at 12 years of age, rhythm 30 x 3, dysmenorrheic, amenorrhea of two months of patient evolution.
Laboratory studies were taken: biometrics blood count, blood chemistry, liver function tests, coagulation times, general urinalysis, all with results within normal parameters. Tumor markers: CA-125 and beta fraction of chorionic gonadotropin, both negative. The chest radiograph showed no findings of pulmonary or mediastinal metastases.
In abdominal-pelvic ultrasound, a normal image of the uterine body was seen, although displaced to the left of the midline. The right ovary had simple cysts. A solid tumor, perhaps of vaginal or adnexal origin, of 10 x 7 x 5 cm was observed. The Liver, kidneys, pancreas and bile ducts were normal (Figures 1 and 2).
Abdominal-pelvic computed tomography showed left ganglionar growth of the aorta at the height of L3, approximately 2 cm in diameter greater, lobed, with heterogeneous enhancement, which gave a topographic appearance of ganglionic conglomerate. The left ovary was enlarged and had small hypodense images in the parenchyma, less than 1 cm. In the right ovary a heterogeneous image with mixed density was observed, a mixed solid component of 7 x 6 cm in the major axes and free liquid in the bottom of bag (figures 3 and 4). In the office an incisional biopsy of the tumor was taken, which was observed in the vaginal introitus. The histopathological report confirmed moderate adenocarcinoma and little differentiated,
With clear cell component, compatible with papillary serous adenocarcinoma. The preoperative diagnosis was solid tumor, probably originated in the ovary. Due to the above, and in view of the suspicion of adnexal tumor, laparotomy was performed protocolized, peritoneal lavage, retroperitoneal biopsies and radical hysterectomy, without annexes, which were sutured above the true pelvis; Piver III, with two thirds of the vagina. The retroperitoneal lymph nodes were sent for a transoperative study and were reported as positive for malignancy.
The histopathological report was a solid tumor originating in the upper third of the vagina, which corresponded to clear cell adenocarcinoma, moderately little differentiated from it. The neoplasm formed glandular, papillary structures and small solid nests of polyhedral cells, with loss of the nucleus-cytoplasm relationship (Figures 5 and 6). The nuclei were large, there were apparent nucleoli and multiple atypical mitoses. In the study corresponding to the retroperitoneal, para-aortic and iliac ganglia ganglia left, malignant metastatic neoplasm of the vagina was observed (figure 7). The uterus was reported normal and the cytological diagnosis of the peritoneal lavage fluid was negative to malignancy. Healthy cervix.
The neoplasm was classified in stage III. Due to poor prognostic factors (locally advanced stage, retroperitoneal metastasis and tumor greater than 5 cm), he received coadjuvant treatment based on chemotherapy. Systemic: cisplatin: 60 mg x m2 body surface, days 1 and 2; epirubicin: 75 mg x m2 body surface, day 1; and cyclophosphamide: 750 mg x m2 body surface, six cycles in total. Currently, after two years of treatment, the patient is asymptomatic, with no findings of Tumor activity demonstrated by computed tomography of the thorax, abdomen and pelvis.
The presented case is of great importance and implies a great challenge for the obstetrician-gynecologist, since timely diagnosis is difficult in the case of a cancer that was considered primary at the beginning of the study and after surgery it was confirmed as the primary of the vagina. The objective is to offer a young and nubile patient an optimal treatment.
Vaginal carcinoma is an infrequent gynecological neoplasm, accounting for only 1% of neoplasms of the female genital tract.1-4 In the United States only 2,100 cases have been diagnosed and 600 patients died from this tumor in the year 2000. Clear cell carcinoma of the vagina has been related to maternal exposure to diethylstilbestrol, with a high degree of histological and manifestation in young women; its incidence peak is at 19 years of age.5-7 This was observed in a healthy woman exposed in utero to diethylstilbestrol, as the only risk factor.
The annual incidence of cases associated with this hormone decreased in 1979, compared to 1973, and has continued in this line; However, in 1987 there were more than 500 cases of adenocarcinomas of clear cells of the vagina and cervix since this control began in 1971. The annual rate of new cases dropped from 25-30 in the early 1970s to 10 to 15 in the 1980s.8
In 1998, the National Health System in Mexico reported the hospital death of 13 people with malignant tumors of the vulva and vagina, without reporting their age; Of these, eight belonged to the rightful population and five to the open population.9 In 1999, the National Health System reported 136 hospital discharges of cases with malignant tumors of the vulva and vagina; 7, 2, 43, 25 and 59 cases in patients from 1 to 4, 5 to 14, 15 to 44, 45 to 64 and 65 or more years of age, respectively. There were no specific reports of the causes.
It is estimated that the incidence of Clear cell denocarcinoma in women exposed in utero to diethylstilbestrol is 1 in 1,000; It has been observed that the risk is higher in those that the exposure occurred in the first 18 weeks in utero. 11 Other factors, such as the dose and duration of treatment with said hormone, could not be related to the frequency and location of clear cell adenocarcinoma.
In the presented case it was of sum importance to know the clinical history of the patient, who was exposed in utero to diethylstilbestrol in the year of 1984 before the threat of abortion of her mother. Before the era of diethylstilbestrol, vaginal adenocarcinoma was rare; it was believed to be caused by Gartner's vaginal adenosis, endometriosis and remnant ducts. However, from the description of Herbst and Scully of clear cell adenocarcinoma in 197012 and the subsequent relationship with prenatal exposure to said hormone, the diagnosis in stages early stages has been increased by biopsies, the Pap test result and colposcopy.