Clear-cell adenocarcinoma of the vagina

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Clear-cell adenocarcinoma of the vagina (and/or cervix)
Specialty Oncology/gynecology

Clear-cell adenocarcinoma of the vagina (and/or cervix) is a rare adenocarcinoma often linked to prenatal exposure to diethylstilbestrol (DES), a drug which was prescribed in high-risk pregnancy.

Contents

Presentation

After age 30 it was thought that women exposed prenatally, "DES daughters", were no longer at risk for the disease, but as they age into their 40s and 50, cases continue to be reported. [1]

According to the Centers for Disease Control and Prevention (CDC), DES daughters should have a pap/pelvic exam every year because of their lifelong risk for clear-cell adenocarcinoma. [2] [3]

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 in 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. [4]

Treatment

Low grade cancer is treated by surgical resection. High grade will require neoadjuvant chemotherapy and resection. Long-term surveillance will be required. [4]

History

The synthetic estrogen 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, or a pregnancy with bleeding, threatened miscarriage or premature labor.

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 vitamins.[ citation needed ]

In the late 1960s through 1971 a cluster of young women, from their teens into their twenties, was mysteriously diagnosed with clear-cell adenocarcinoma (CCA), a cancer not generally found in women until after menopause. 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. [5]

See also

Related Research Articles

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The cervix or cervix uteri is the lower part of the uterus (womb) in the human female reproductive system. The cervix is usually 2 to 3 cm long and roughly cylindrical in shape, which changes during pregnancy. The narrow, central cervical canal runs along its entire length, connecting the uterine cavity and the lumen of the vagina. The opening into the uterus is called the internal os, and the opening into the vagina is called the external os. The lower part of the cervix, known as the vaginal portion of the cervix, bulges into the top of the vagina. The cervix has been documented anatomically since at least the time of Hippocrates, over 2,000 years ago.

<span class="mw-page-title-main">Cervical cancer</span> Cancer arising from the cervix

Cervical cancer is a cancer arising from the cervix. It is due to the abnormal growth of cells that have the ability to invade or spread to other parts of the body. Early on, typically no symptoms are seen. Later symptoms may include abnormal vaginal bleeding, pelvic pain or pain during sexual intercourse. While bleeding after sex may not be serious, it may also indicate the presence of cervical cancer.

<span class="mw-page-title-main">Diethylstilbestrol</span> Chemical compound

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 history of recurrent miscarriage, hormone therapy for menopausal symptoms and estrogen deficiency, treatment of prostate cancer and breast cancer, and other uses. By 2007, it was only used in the treatment of prostate cancer and breast cancer. In 2011, Hoover and colleagues reported on adverse health outcomes linked to DES including infertility, miscarriage, ectopic pregnancy, preeclampsia, preterm birth, stillbirth, infant death, menopause prior to age 45, breast cancer, cervical cancer, and vaginal cancer. While most commonly taken by mouth, DES was available for use by other routes as well, for instance, vaginal, topical, and by injection.

<span class="mw-page-title-main">Hysterectomy</span> Surgical removal of the uterus

Hysterectomy is the partial or total surgical removal of the uterus. It may also involve removal of the cervix, ovaries (oophorectomy), fallopian tubes (salpingectomy), and other surrounding structures. Partial hysterectomies allow for hormone regulation while total hysterectomies do not.

<span class="mw-page-title-main">Endometrial cancer</span> Uterine cancer that is located in tissues lining the uterus

Endometrial cancer is a cancer that arises from the endometrium. It is the result of the abnormal growth of cells that have the ability to invade or spread to other parts of the body. The first sign is most often vaginal bleeding not associated with a menstrual period. Other symptoms include pain with urination, pain during sexual intercourse, or pelvic pain. Endometrial cancer occurs most commonly after menopause.

<span class="mw-page-title-main">Vaginal bleeding</span> Medical condition

Vaginal bleeding is any expulsion of blood from the vagina. This bleeding may originate from the uterus, vaginal wall, or cervix. Generally, it is either part of a normal menstrual cycle or is caused by hormonal or other problems of the reproductive system, such as abnormal uterine bleeding.

<span class="mw-page-title-main">Endometrial polyp</span> Medical condition

An endometrial polyp or uterine polyp is a mass in the inner lining of the uterus. They may have a large flat base (sessile) or be attached to the uterus by an elongated pedicle (pedunculated). Pedunculated polyps are more common than sessile ones. They range in size from a few millimeters to several centimeters. If pedunculated, they can protrude through the cervix into the vagina. Small blood vessels may be present, particularly in large polyps.

<span class="mw-page-title-main">Cervical intraepithelial neoplasia</span> Medical condition

Cervical intraepithelial neoplasia (CIN), also known as cervical dysplasia, is the abnormal growth of cells on the surface of the cervix that could potentially lead to cervical cancer. More specifically, CIN refers to the potentially precancerous transformation of cells of the cervix.

Vaginal cancer is an extraordinarily rare form of cancer that develops in the tissue of the vagina. Primary vaginal cancer originates from the vaginal tissue – most frequently squamous cell carcinoma, but primary vaginal adenocarcinoma, sarcoma, and melanoma have also been reported – while secondary vaginal cancer involves the metastasis of a cancer that originated in a different part of the body. Secondary vaginal cancer is more common. Signs of vaginal cancer may include abnormal vaginal bleeding, dysuria, tenesmus, or pelvic pain, though as many as 20% of women diagnosed with vaginal cancer are asymptomatic at the time of diagnosis. Vaginal cancer occurs more frequently in women over age 50, and the mean age of diagnosis of vaginal cancer is 60 years. It often can be cured if found and treated in early stages. Surgery alone or surgery combined with pelvic radiation is typically used to treat vaginal cancer.

<span class="mw-page-title-main">Pelvic examination</span> Physical medical examination

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, discharge, urinary incontinence, or trauma. It can also be used to assess a woman's anatomy in preparation for procedures. The exam can be done awake in the clinic and emergency department, or under anesthesia in the operating room. The most commonly performed components of the exam are 1) the external exam, to evaluate the external genitalia 2) the internal exam with palpation to examine the uterus, ovaries, and fallopian tubes, and 3) the internal exam using the speculum to visualize the vaginal walls and cervix. During the pelvic exam, sample of cells and fluids may be collected to screen for sexually transmitted infections or cancer.

<span class="mw-page-title-main">Cervical polyp</span> Medical condition

A cervical polyp is a common benign polyp or tumour on the surface of the cervical canal. They can cause irregular menstrual bleeding but often show no symptoms. Treatment consists of simple removal of the polyp and prognosis is generally good. About 1% of cervical polyps will show neoplastic change which may lead to cancer. They are most common in post-menarche, pre-menopausal women who have been pregnant.

A vaginal disease is a pathological condition that affects part or all of the vagina.

Neuroendocrine carcinoma of the cervix is best defined separately:Neuroendocrine: Of, relating to, or involving the interaction between the nervous system and the hormones of the endocrine glands.Carcinoma: An invasive malignant tumor derived from epithelial tissue that tends to metastasize to other areas of the body.

<span class="mw-page-title-main">Postcoital bleeding</span> Medical condition

Postcoital bleeding (PCB) is non-menstrual vaginal bleeding that occurs during or after sexual intercourse. Though some causes are with associated pain, it is typically painless and frequently associated with intermenstrual bleeding.

Müllerian duct anomalies are those structural anomalies caused by errors in müllerian duct development during embryonic morphogenesis. Factors that precipitate include genetics, and maternal exposure to teratogens.

Vaginal adenosis is a benign abnormality in the vagina, commonly thought to be caused by intrauterine and neonatal exposure of diethylstilbestrol and other progestogens and nonsteroidal estrogens, however it has also been observed in otherwise healthy women and has been considered at times idiopathic or congenital. Postpubertal lesions have also been observed to grow de novo. It has a rather common incidence, of about 10% of adult women.

<span class="mw-page-title-main">Dimestrol</span> Chemical compound

Dimestrol, also known as dianisylhexene, 4,4'-dimethoxy-α,α'-diethylstilbene, diethylstilbestrol dimethyl ether, and dimethoxydiethylstilbestrol, is a synthetic nonsteroidal estrogen of the stilbestrol group which is related to diethylstilbestrol. It has been used clinically as a hormonal therapy in cases of delayed female puberty, hypogonadism, menopausal, and postmenopausal symptoms. It is known to induce the development of female secondary sexual characteristics in the case of female delayed puberty or hypogonadism. The drug has also been used as a growth promoter in livestock.

<span class="mw-page-title-main">Vaginal epithelium</span>

The vaginal epithelium is the inner lining of the vagina consisting of multiple layers of (squamous) cells. The basal membrane provides the support for the first layer of the epithelium-the basal layer. The intermediate layers lie upon the basal layer, and the superficial layer is the outermost layer of the epithelium. Anatomists have described the epithelium as consisting of as many as 40 distinct layers. The mucus found on the epithelium is secreted by the cervix and uterus. The rugae of the epithelium create an involuted surface and result in a large surface area that covers 360 cm2. This large surface area allows the trans-epithelial absorption of some medications via the vaginal route.

Vaginal melanoma is a rare malignancy that originates from melanocytes in the vaginal epithelium. It is also known as a melanocytic tumor or as a malignant melanoma. It is aggressive and infrequently cured. The median overall survival is 16 months. Vaginal melanoma accounts 5.5% of all vaginal cancers and only 1% of all melanomas diagnosed in women. Vaginal melanomas are frequently diagnosed in advanced stages of the disease. The prognosis is poor and the most important risk factor is the presence of lymph node metastases.

Diethylstilbestrol (DES), a synthetic nonsteroidal estrogen which was previously used clinically to support pregnancy, has been linked to a variety of long-term adverse effects in women who were treated with it during pregnancy and in their offspring.

References

  1. Smith, Emily K.; White, Mary C.; Weir, Hannah K.; Peipins, Lucy A.; Thompson, Trevor D. (1 January 2012). "Higher incidence of clear cell adenocarcinoma of the cervix and vagina among women born between 1947 and 1971 in the United States". Cancer Causes & Control. SpringerLink. 23 (1): 207–211. doi:10.1007/s10552-011-9855-z. PMC   3230753 . PMID   22015647.
  2. "Annual Exam for DES Daughters" (PDF). Centers for Disease Control and Prevention. Archived from the original (PDF) on 4 October 2013. Retrieved 18 June 2013.
  3. Moyer, Virginia A.; U.S. Preventive Services Task Force (19 June 2012). "Screening for Cervical Cancer: U.S. Preventive Services Task Force Recommendation Statement". Annals of Internal Medicine. 156 (12): 880–91, W312. doi: 10.7326/0003-4819-156-12-201206190-00424 . PMID   22711081.
  4. 1 2 Gera S. Clear cell adenocarcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vaginaclearcelladeno.html. Accessed May 25th, 2019
  5. Hatch EE, Palmer JR, Titus-Ernstoff L, et al. (August 1998). "Cancer risk in women exposed to diethylstilbestrol in utero". JAMA. 280 (7): 630–4. doi: 10.1001/jama.280.7.630 . PMID   9718055.


PD-icon.svg This article incorporates public domain material from Dictionary of Cancer Terms. U.S. National Cancer Institute.