Clear Cell Adenocarcinoma of the Vagina: Case Report and Review of the Literature

Abstract

Carcinomas of the vagina are uncommon 1,2 and are usually squamous cell in type. Over the past few decades, however, the incidence of vaginal adenocarcinomas has increased, predominantly of the clear cell type and mainly affecting younger subjects. 1,[3][4][5] In 1970 Herbst and Scully 6 described clear cell adenocarcinoma of the vagina in six girls and young adults and subsequently confirmed its association with exposure to diethylstilbestrol (DES) in utero. Since then, numerous reports of the condition have appeared in the medical literature.5 By 1980 over 400 cases have been reported from various parts of the world, 5,7,8 the majority being in the United States. The first case in the United Kingdom was that reported by Monaghan and Sirisena in 1978. 9 By 1984 only four cases had been reported in the UK. 8 The variation in incidence of the disease in different parts of the world is a reflection of the extent to which mothers were treated with DES during pregnancy for threatened or habitual abortion in the different countries.We present the first report of clear cell adenocarcinoma of the vagina in a 23-year-old Arab woman. Exposure to DES in utero could not be confirmed in this case, but the presence of the condition raises many issues which are discussed in this paper. Case ReportA 23-year-old Yemeni woman had a 2-month history of swelling of left lower limb, associated with vague suprapubic pain, dysuria, and hematuria. She also had bilateral inguinal swelling. She gave no history of relevant previous illness or drug ingestion. She was para 3, all dead (one neonatal death and two stillbirths).Examination revealed a hard supraclavicular lymph node on the right side with smaller ones in the posterior triangle. The abdomen showed suprapubic tenderness and an enlarged, smooth, nontender liver. Enlarged inguinal nodes were noted on both sides, and the entire left lower limb was swollen and tender. Rectal examination revealed hard masses filling the vagina anteriorly and to the left, confirmed by vaginal examination. Gynecologic examination under anesthesia revealed normal vulva and a firm, irregular, friable mass, filling the upper vagina and destroying the cervix, with associated malodorous, blood-tinged discharge. The uterus was bulky and immobile. The fornices were thickened, suggesting parametrial involvement. Biopsy specimens were taken from the vaginal mass and the supraclavicular lymph node. Cystoscopic examination showed slightly congested bladder mucosa with no obvious tumor infiltration of the wall.Other investigations revealed microcytic, hypochromic anemia with hemoglobin of 82 g/L and erythrocyte sedimentation rate of 80 mm/h. Results of biochemical investigations were within normal limits. Chest x-ray appearance was normal, but abdominal roentgenogram showed spina bifida at S1 and right-sided psoas mass containing streaks of calcification. Renal shadows were normal, and x-ray examination of the left leg showed only soft tissue swelling. Results of microbiological investigations were noncontributory....