Malignant transformation of vaginal adenosis to clear cell carcinoma without prenatal diethylstilbestrol exposure: a case report and literature review

Abstract

Background We report an extremely rare case of vaginal clear cell carcinoma, which originated from the malignant transformation of vaginal adenosis without prenatal diethylstilbestrol (DES) exposure. Case presentation In this case, the patient was a Chinese woman with a history of two decades of intermittent vaginal pain, sexual intercourse pain and vaginal contact bleeding. On September 1, 2011, when the patient was 39 years old, a vaginal biopsy revealed vaginal adenosis. After intermittent drug and laser treatment, her symptoms did not improve. Four years later, on March 4, 2015, another vaginal biopsy for abnormal vaginal cytology revealed atypical vaginal adenosis. After treatment with sirolimus, her symptoms and abnormal vaginal cytology results persisted, and she underwent laparoscopic hysterectomy with bilateral salpingo-oophorectomy and excision of the vaginal lesions. One year after the hysterectomy, on August 15, 2017, the vaginal cytology results suggested atypical glandular cells, and a biopsy revealed vaginal clear cell carcinoma originating from the atypical vaginal adenosis. A wide local resection of the vaginal lesions was performed, followed by concurrent chemoradiotherapy. Regular follow-up over 16 months showed no evidence of the recurrence of vaginal adenosis or cancer. Conclusions Based on the evolution of a series of pathological evidence, we report the fourth case in the world of vaginal clear cell carcinoma originating from vaginal adenosis without prenatal DES exposure. Wide local excision with radiotherapy provided at least 16 months of disease-free survival.

Primary Vaginal Adenocarcinoma of Intestinal-Type: A Case Report of a Rare Tumor With Review of Histology, Differential Diagnosis, and Literature

“…Subtypes of vaginal adenocarcinomas include clear cell (most common), endometroid, serous, and mucinous [3]. Clear cell variant can occur in the setting of background vaginal adenosis or in women with a history of in-utero exposure to diethylstilbestrol (DES) [5]. Mucinous adenocarcinomas can be further subdivided into endocervical and intestinal subtypes [3].…”

Diagnostic and Therapeutic Approach in a Metastatic Vaginal Adenocarcinoma: A Case Report

“…The majority of all vaginal carcinomas are squamous cell carcinomas. Vaginal adenocarcinomas (VAC) are most often reported after intrauterine exposition to diethylstilbestrol (DES) (2), rarely in the context of vaginal adenosis (3,4) or in the context of chromosomal abnormalities or malformations of the uterus or the vagina (5). The largest study on VACs without DES exposition showed a poor prognosis with significantly worse survival compared to squamous cell carcinomas or DESassociated VACs.…”

Primary clear cell adenocarcinoma of the vagina in a young female with uterus didelphys

“…Primary adenocarcinomas accounts for 6-14% of vaginal tumours which in turn account for 2% of all gynaecological malignancies. 1 Clear cell adenocarcinomas, also known as Mesonephroid carcinomas or Mesonephric carcinomas are rare tumours accounting for 2-9% of adenocarcinoma s of the female genital tract. 2 Ovary, cervix and urinary tract are the most commonly involved areas.…”

“…85-90 % of women with vaginal cancer present with Squamous cell carcinoma. 1 Adenocarcinomas of clear cell type are uncommon, accounting for 2-9 % of vaginal malignancies. 2 Clear cell adenocarcinomas (CCAs) arising in the setting of in-utero exposure to Diethylstilbestrol (DES) is well documented, Herbst et al being the first to report CCAs occurring in women whose mothers were exposed to DES during pregnancy.…”