Clear-cell adenocarcinoma (CCA) of the

vagina In mammals, the vagina is the elastic, muscular part of the female genital tract. In humans, it extends from the vestibule to the cervix. The outer vaginal opening is normally partly covered by a thin layer of mucosal tissue called the hymen ...

or cervix is a rare adenocarcinoma often linked to prenatal exposure to

diethylstilbestrol Diethylstilbestrol (DES), also known as stilbestrol or stilboestrol, is a nonsteroidal estrogen medication, which is presently rarely used. In the past, it was widely used for a variety of indications, including pregnancy support for those with a ...

(DES), a drug which was prescribed in high-risk pregnancy.

Presentation

After age 30 it was thought that women exposed prenatally, "DES daughters", were no longer were at risk for the disease, but as they age into their 40s and 50, cases continue to be reported. According to the

Centers for Disease Control and Prevention The Centers for Disease Control and Prevention (CDC) is the national public health agency of the United States. It is a United States federal agency, under the Department of Health and Human Services, and is headquartered in Atlanta, Georgi ...

(CDC), DES daughters should have a pap/

pelvic exam A pelvic examination is the physical examination of the external and internal female pelvic organs. It is frequently used in gynecology for the evaluation of symptoms affecting the female reproductive and urinary tract, such as pain, bleeding, d ...

every year because of their lifelong risk for clear-cell adenocarcinoma.

Diagnosis

Clear-cell adenocarcinoma of the vagina is a rare cancer, occurring in up to 10% of primary vaginal malignancies. It is all but confirmed if maternal use of DES is established. Even though it was once thought to no longer occur past the age of 30, it is still seen into the 40s and 50s. Some of the main signs and symptoms for clear-cell adenocarcinoma of the vagina are spotting between menstrual cycles, bleeding post-menopause, abnormal bleeding, and malignant pericardial effusion or cardiac tamponade.Gera S. Clear cell adenocarcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vaginaclearcelladeno.html. Accessed May 25th, 2019

Treatment

Low grade cancer is treated by surgical resection. High grade will require neoadjuvant chemotherapy and resection. Long-term surveillance will be required.

History

The synthetic

estrogen Estrogen or oestrogen is a category of sex hormone responsible for the development and regulation of the female reproductive system and secondary sex characteristics. There are three major endogenous estrogens that have estrogenic hormonal ac ...

DES was given to millions of pregnant women in the United States and other countries. Use in the US was primarily from 1938 to 1971 but not limited to those years. Internationally, DES use continued until the early 1980s. DES was given if a woman had a previous miscarriage,

diabetes Diabetes, also known as diabetes mellitus, is a group of metabolic disorders characterized by a high blood sugar level ( hyperglycemia) over a prolonged period of time. Symptoms often include frequent urination, increased thirst and increased ...

, or a pregnancy with bleeding, threatened miscarriage or

premature labor Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is between ...

. Up until the mid to late 1950s some women were given DES shots. After that, DES was primarily prescribed in pill form. DES also was included in some

prenatal vitamin Prenatal vitamins, also known as prenatal supplements, are vitamin and mineral supplements intended to be taken before and during pregnancy and during postnatal lactation. Although not intended to replace a healthy diet, prenatal vitamins provid ...

s. In the late 1960s through 1971 a cluster of young women, from their teens into their twenties, was mysteriously diagnosed with CCA, a cancer not generally found in women until after

menopause Menopause, also known as the climacteric, is the time in women's lives when menstrual periods stop permanently, and they are no longer able to bear children. Menopause usually occurs between the age of 47 and 54. Medical professionals often ...

. Doctors at Massachusetts General Hospital eventually linked DES exposure before birth to the development of CCA in these young women. They determined the risk for developing CCA among DES daughters is estimated at 1 in a 1,000.

References

External links