Clear-cell adenocarcinoma

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Clear-cell adenocarcinoma
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Micrograph of an ovarian clear-cell adenocarcinoma. H&E stain
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Clear-cell adenocarcinoma is a rare and aggressive form of cancer that typically arises in the female reproductive organs, particularly the ovaries and the endometrium as well as the kidneys and is characterized by the presence of clear, [1] glycogen-rich cells. [2] Specific criteria must be met for a tumor to be classified as clear cell adenocarcinoma. According to the WHO, these criteria include polygonal or hobnail or cells with clear or eosinophilic/oxyphilic cytoplasm and nuclear atypia, with different architectural patterns of growth, such as papillary, tubulocystic, or solid. [1]

Contents

Types

Cervical

Cervical adenocarcinoma is less likely to be caused by high-risk HPV strains than cervical squamous cell carcinoma is: around 10-15% of cervical adenocarcinomas are non-HPV-related. Cervical clear cell carcinoma (CCC) is typically HPV-negative, though many are p16 positive. [3] Traditionally, clear cell carcinoma of the lower genital tract in females was related to diethylstilbestrol (DES) exposure in utero. More recently, these cases develop sporadically or are related to vaginal adenosis. Risk factors are similar to other types of cervical cancer, including immunosuppression, smoking, long-term oral contraceptive use, increased number of sexual partners, early onset of sexual activity, hormone replacement therapy, and obesity. [3]

As with other types of cervical cancer, cervical CCC is diagnosed with a cervical biopsy. Further treatment is managed similarly to other types of cervical cancer, such as performing an excision procedure in the form of a cold knife conization or a loop electrosurgical excision procedure (LEEP). Further surgical management depends upon the depth of tumor invasion. Invasive tumors may require chemotherapy and radiation treatment. Cervical CCC appears to have a similar prognosis to other types of cervical cancer. [4]

Ovarian

Ovarian clear cell carcinoma is a subtype of epithelial ovarian carcinoma. These tumors are typically unilateral. They are associated with endometriosis, in which endometrial tissue grows outside the uterine cavity. Benign endometriotic cysts, also known as adenofibromas, are frequently the source of these tumor cells. [5]

Treatment of ovarian clear cell carcinoma typically begins with surgery to remove the ovaries and other affected organs. Chemotherapeutic treatment of gynecological cancers typically includes a platinum-based therapy and a taxane. Clear cell ovarian tumors have a higher rate of resistance to these medications. Other treatment routes, including immunologic agents against certain biomarkers, are being explored. [6]

Uterine

Uterine clear cell carcinoma is a rare, aggressive form of endometrial cancer. This is an example of a Type II endometrial carcinoma, meaning that it is unrelated to endometrial hyperplasia. Uterine clear cell tumors arise from an endometrium that has become thin and dry. [7] Patients usually present with abnormal uterine bleeding.

Treatment of uterine clear cell carcinoma is primarily surgical. A tumor debulking procedure is typically performed, consisting of a total hysterectomy and bilateral salpingo-oopherectomy. Depending on the level of tumor invasion, an omentectomy and other malignancy removal may be performed at the same time. Cytoreductive surgery, which involves removing all visible cancer cells during the operation, is the current standard of care. Minimally invasive surgical techniques are preferred over open surgeries, as patients tend to have better short-term outcomes after the surgery. [8]

Patients may be treated with chemotherapy or radiation following tumor debulking. Platinum-based chemotherapy is the mainstay for most gynecologic cancers, including endometrial cancers. Depending on the genomic subtype of the tumor, various other chemotherapeutic or immunologic therapies may be employed. [8]

Vaginal

Clear cell adenocarcinoma of the vagina is a rare cancer that is related to DES exposure in utero. DES was a drug previously given to some women with high-risk pregnancies to prevent miscarriage. Women who were exposed to DES in utero should be evaluated yearly with a pap smear and pelvic exam due to risk of developing clear cell adenocarcinoma. [9] [10]

Vaginal clear cell carcinoma frequently presents with abnormal uterine bleeding. As with other gynecological cancers, treatment varies with the stage of the cancer. Lower grade tumors can be treated with surgical resection, while higher grade tumors may need chemotherapy and radiation. [11]

See also

Related Research Articles

<span class="mw-page-title-main">Endometrium</span> Inner mucous membrane of the mammalian uterus

The endometrium is the inner epithelial layer, along with its mucous membrane, of the mammalian uterus. It has a basal layer and a functional layer: the basal layer contains stem cells which regenerate the functional layer. The functional layer thickens and then is shed during menstruation in humans and some other mammals, including other apes, Old World monkeys, some species of bat, the elephant shrew and the Cairo spiny mouse. In most other mammals, the endometrium is reabsorbed in the estrous cycle. During pregnancy, the glands and blood vessels in the endometrium further increase in size and number. Vascular spaces fuse and become interconnected, forming the placenta, which supplies oxygen and nutrition to the embryo and fetus. The speculated presence of an endometrial microbiota has been argued against.

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

Cervical cancer is a cancer arising from the cervix or in the any layer of the wall of 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">Uterine cancer</span> Medical condition

Uterine cancer, also known as womb cancer, includes two types of cancer that develop from the tissues of the uterus. Endometrial cancer forms from the lining of the uterus, and uterine sarcoma forms from the muscles or support tissue of the uterus. Endometrial cancer accounts for approximately 90% of all uterine cancers in the United States. Symptoms of endometrial cancer include changes in vaginal bleeding or pain in the pelvis. Symptoms of uterine sarcoma include unusual vaginal bleeding or a mass in the vagina.

<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">Ovarian cancer</span> Cancer originating in or on the ovary

Ovarian cancer is a cancerous tumor of an ovary. It may originate from the ovary itself or more commonly from communicating nearby structures such as fallopian tubes or the inner lining of the abdomen. The ovary is made up of three different cell types including epithelial cells, germ cells, and stromal cells. When these cells become abnormal, they have the ability to divide and form tumors. These cells can also invade or spread to other parts of the body. When this process begins, there may be no or only vague symptoms. Symptoms become more noticeable as the cancer progresses. These symptoms may include bloating, vaginal bleeding, pelvic pain, abdominal swelling, constipation, and loss of appetite, among others. Common areas to which the cancer may spread include the lining of the abdomen, lymph nodes, lungs, and liver.

<span class="mw-page-title-main">Serous tumour</span> Medical condition

A serous tumour is a neoplasm that typically has papillary to solid formations of tumor cells with crowded nuclei, and which typically arises on the modified Müllerian-derived serous membranes that surround the ovaries in females. Such ovarian tumors are part of the surface epithelial-stromal tumour group of ovarian tumors. They are common neoplasms with a strong tendency to occur bilaterally, and they account for approximately a quarter of all ovarian tumors.

<span class="mw-page-title-main">Surface epithelial-stromal tumor</span> Medical condition

Surface epithelial-stromal tumors are a class of ovarian neoplasms that may be benign or malignant. Neoplasms in this group are thought to be derived from the ovarian surface epithelium or from ectopic endometrial or fallopian tube (tubal) tissue. Tumors of this type are also called ovarian adenocarcinoma. This group of tumors accounts for 90% to 95% of all cases of ovarian cancer; however is mainly only found in postmenopausal women with the exception of the United States where 7% of cases occur in women under the age of 40. Serum CA-125 is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment. 75% of women with epithelial ovarian cancer are found within the advanced-stages; however younger patients are more likely to have better prognoses than older patients.

<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.

<span class="mw-page-title-main">Primary peritoneal carcinoma</span> Medical condition

Primary peritoneal cancer or carcinoma is also known as serous surface papillary carcinoma, primary peritoneal carcinoma, extra-ovarian serous carcinoma, primary serous papillary carcinoma, and psammomacarcinoma. It was historically classified under "carcinoma of unknown primary" (CUP). Primary peritoneal cancer is a cancer of the cells lining the peritoneum, or abdominal cavity. It usually affects women and is diagnosed after the age of 60; it very rarely affects men.

<span class="mw-page-title-main">Mixed Müllerian tumor</span> Medical condition

A malignant mixed Müllerian tumor, also known as malignant mixed mesodermal tumor (MMMT) is a cancer found in the uterus, the ovaries, the fallopian tubes and other parts of the body that contains both carcinomatous and sarcomatous components. It is divided into two types, homologous and a heterologous type. MMMT account for between two and five percent of all tumors derived from the body of the uterus, and are found predominantly in postmenopausal women with an average age of 66 years. Risk factors are similar to those of adenocarcinomas and include obesity, exogenous estrogen therapies, and nulliparity. Less well-understood but potential risk factors include tamoxifen therapy and pelvic irradiation.

<span class="mw-page-title-main">Vulvar cancer</span> Cancer involving the vulva

Vulvar cancer is a cancer of the vulva, the outer portion of the female genitals. It most commonly affects the labia majora. Less often, the labia minora, clitoris, or Bartholin's glands are affected. Symptoms include a lump, itchiness, changes in the skin, or bleeding from the vulva.

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.

Gynecologic oncology is a specialized field of medicine that focuses on cancers of the female reproductive system, including ovarian cancer, uterine cancer, vaginal cancer, cervical cancer, and vulvar cancer. As specialists, they have extensive training in the diagnosis and treatment of these cancers.

Gynecologic cancer is a type of cancer that affects the female reproductive system, including ovarian cancer, uterine cancer, vaginal cancer, cervical cancer, and vulvar cancer.

<span class="mw-page-title-main">Uterine serous carcinoma</span> Type of cancer of the uterus

Uterine serous carcinoma is a malignant form of serous tumor that originates in the uterus. It is an uncommon form of endometrial cancer that typically arises in postmenopausal women. It is typically diagnosed on endometrial biopsy, prompted by post-menopausal bleeding.

Uterine clear-cell carcinoma (CC) is a rare form of endometrial cancer with distinct morphological features on pathology; it is aggressive and has high recurrence rate. Like uterine papillary serous carcinoma CC does not develop from endometrial hyperplasia and is not hormone sensitive, rather it arises from an atrophic endometrium. Such lesions belong to the type II endometrial cancers.

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">Villoglandular adenocarcinoma of the cervix</span> Medical condition

Villoglandular adenocarcinoma of the cervix is a rare type of cervical cancer that, in relation to other cervical cancers, is typically found in younger women and has a better prognosis.

<span class="mw-page-title-main">Gynecologic cancer disparities in the United States</span>

Gynecologic cancer disparities in the United States refer to differences in incidence, prevalence, and mortality from gynecologic cancers between population groups. The five main types of gynecologic cancer include cervical cancer, ovarian cancer, endometrial cancer, vaginal cancer, and vulvar cancer. For patients with these and other gynecologic malignancies within the United States, disparities across the care continuum by socioeconomic status and racial/ethnic background have been previously identified and studied. The causes behind these disparities are multifaceted and a complex interplay of systemic differences in health as well as individual patient factors such as cultural, educational, and economic barriers.

<span class="mw-page-title-main">Ovarian squamous cell carcinoma</span> Medical condition

Ovarian squamous cell carcinoma (oSCC) or squamous ovarian carcinoma (SOC) is a rare tumor that accounts for 1% of ovarian cancers. Included in the World Health Organization's classification of ovarian cancer, it mainly affects women above 45 years of age. Survival depends on how advanced the disease is and how different or similar the individual cancer cells are.

References

  1. 1 2 "NCI Dictionary of Cancer Terms". National Cancer Institute. 2 February 2011. Retrieved 10 July 2019.
  2. ↑ Guo Y, Shrestha A, Maskey N, Dong X, Zheng Z, Yang F, et al. (2022). "Recent Trends in the Incidence of Clear Cell Adenocarcinoma and Survival Outcomes: A SEER Analysis". Frontiers in Endocrinology. 13: 762589. doi: 10.3389/fendo.2022.762589 . PMC   8907425 . PMID   35282450.
  3. 1 2 Stolnicu S, Hoang L, Soslow RA (November 2019). "Recent advances in invasive adenocarcinoma of the cervix". Virchows Archiv. 475 (5): 537–549. doi:10.1007/s00428-019-02601-0. PMC   6864265 . PMID   31209635.
  4. ↑ Park KJ (January 2020). "Cervical adenocarcinoma: integration of HPV status, pattern of invasion, morphology and molecular markers into classification". Histopathology. 76 (1): 112–127. doi:10.1111/his.13995. PMID   31846527.
  5. ↑ Sugiyama T, Kamura T, Kigawa J, Terakawa N, Kikuchi Y, Kita T, et al. (June 2000). "Clinical characteristics of clear cell carcinoma of the ovary: a distinct histologic type with poor prognosis and resistance to platinum-based chemotherapy". Cancer. 88 (11): 2584–2589. doi:10.1002/1097-0142(20000601)88:11<2584::aid-cncr22>3.3.co;2-x. PMID   10861437.
  6. ↑ Chan JK, Teoh D, Hu JM, Shin JY, Osann K, Kapp DS (June 2008). "Do clear cell ovarian carcinomas have poorer prognosis compared to other epithelial cell types? A study of 1411 clear cell ovarian cancers". Gynecologic Oncology. 109 (3): 370–376. doi:10.1016/j.ygyno.2008.02.006. PMID   18395777.
  7. ↑ Gründker C, Günthert AR, Emons G (2008). "Hormonal heterogeneity of endometrial cancer". In Berstein LM, Santen RJ (eds.). Innovative Endocrinology of Cancer. Advances in Experimental Medicine and Biology. Vol. 630. New York, NY: Springer New York. pp. 166–188. doi:10.1007/978-0-387-78818-0_11. ISBN   978-0-387-78817-3. PMID   18637491.
  8. 1 2 Bogani G, Ray-Coquard I, Concin N, Ngoi NY, Morice P, Enomoto T, et al. (March 2022). "Clear cell carcinoma of the endometrium". Gynecologic Oncology. 164 (3): 658–666. doi:10.1016/j.ygyno.2022.01.012. PMID   35063279.
  9. ↑ Moyer VA (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.
  10. ↑ Tournaire M, Devouche E, Lafaye N, Levadou A (23 December 2020). "Screening for cancers of the cervix and vagina for women exposed to diethylstilbestrol (DES) in utero". Journal of Gynecology Obstetrics and Human Reproduction. 50 (7): 102042. doi:10.1016/j.jogoh.2020.102042. ISSN   2468-7847.
  11. ↑ "Clear cell carcinoma-vagina". www.pathologyoutlines.com. Retrieved 2024-07-01.
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