Uterine clear-cell carcinoma | |
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Specialty | Gynecology, oncology |
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. [1]
The lesion is found in patients who present typically with abnormal or postmenopausal bleeding or discharge. Such bleeding is followed by further evaluation leading to a tissue diagnosis, usually done by a dilatation and curettage (D&C). A work-up to follow would look for metastasis using imaging technology including sonography and MRI. The median age at diagnosis in a large study was 66 years. [2] Histologically the lesion may coexist with classical endometrial cancer.[ citation needed ]
Prognosis of the CC is affected by age, stage, and histology as well as treatment
The primary treatment is surgical. FIGO-cancer staging is done at the time of surgery which consists of peritoneal cytology, total hysterectomy, bilateral salpingo-oophorectomy, pelvic/para-aortic lymphadenectomy, and omentectomy. The tumor is aggressive and spreads quickly into the myometrium and the lymphatic system. Thus even in presumed early stages, lymphadenectomy and omentectomy should be included in the surgical approach. If the tumor has spread surgery is cytoreductive followed by radiation therapy and/or chemotherapy. [2] [3]
The five years survival was reported to be 68%. [2]
Uterine clear-cell carcinoma is staged like other forms of endometrial carcinoma at time of surgery using the International Federation of Gynecology and Obstetrics (FIGO) cancer staging system 2009. [4]
IA Tumor confined to the uterus, no or < ½ myometrial invasion
IB Tumor confined to the uterus, > ½ myometrial invasion
II Cervical stromal invasion, but not beyond uterus
IIIA Tumor invades serosa or adnexa
IIIB Vaginal and/or parametrial involvement
IIIC1 Pelvic node involvement
IIIC2 Para-aortic involvement
IVA Tumor invasion bladder and/or bowel mucosa
IVB Distant metastases including abdominal metastases and/or inguinal lymph nodes
The uterus or womb is a major female hormone-responsive secondary sex organ of the reproductive system in humans and most other mammals. In the human, the lower end of the uterus, the cervix, opens into the vagina, while the upper end, the fundus, is connected to the fallopian tubes. It is within the uterus that the fetus develops during gestation. In the human embryo, the uterus develops from the paramesonephric ducts which fuse into the single organ known as a simplex uterus. The uterus has different forms in many other animals and in some it exists as two separate uteri known as a duplex uterus.
Gynecologic hemorrhage represents excessive bleeding of the female reproductive system. Such bleeding could be visible or external, namely bleeding from the vagina, or it could be internal into the pelvic cavity or form a hematoma. Normal menstruation is not considered a gynecologic hemorrhage, as it is not excessive. Hemorrhage associated with a pregnant state or during delivery is an obstetrical hemorrhage.
Uterine cancer, also known as womb cancer, are two types of cancer that develops 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. Symptoms of endometrial cancer include unusual vaginal bleeding or pain in the pelvis. Symptoms of uterine sarcoma include unusual vaginal bleeding or a mass in the vagina.
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.
Ovarian cancer is a cancer that forms in or on an ovary. It results in abnormal cells that have the ability to 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, pelvic pain, abdominal swelling, 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.
Serous tumours are part of the surface epithelial-stromal tumour group of ovarian neoplasms, which derive from Mullerian epithelium. They are common neoplasms with a strong tendency to occur bilaterally, and they account for approximately a quarter of all ovarian tumors.
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. 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.
Adenomyosis is a gynecologic medical condition characterized by the abnormal presence of endometrial tissue within the myometrium. When endometrial tissue is present abnormally entirely outside the uterus, it is considered to be a similar but distinct medical condition, endometriosis. The two conditions are found together in many cases, but often occur separately. Before being recognized as a distinct condition, adenomyosis was called endometriosis interna. The less-commonly-used term "adenomyometritis" is a more specific name for the condition, specifying involvement of the uterus.
Vaginal bleeding is any bleeding through the vagina, including bleeding from the vaginal wall itself, as well as bleeding from another location of the female reproductive system, often the uterus. 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.
The uterine sarcomas form a group of malignant tumors that arises from the smooth muscle or connective tissue of the uterus.
Endometrial intraepithelial neoplasia (EIN) is a premalignant lesion of the uterine lining that predisposes to endometrioid endometrial adenocarcinoma. It is composed of a collection of abnormal endometrial cells, arising from the glands that line the uterus, which have a tendency over time to progress to the most common form of uterine cancer—endometrial adenocarcinoma, endometrioid type.
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.
Vulvar cancer is a cancer of the vulva, the outer portion of the female genitals. It most commonly affects the outer vaginal lips. Less often, the inner vaginal lips, clitoris, or vaginal glands. Symptoms include a lump, itchiness, changes in the skin, or bleeding from the vulva.
Hematometra is a medical condition involving collection or retention of blood in the uterus. It is most commonly caused by an imperforate hymen or a transverse vaginal septum.
Primary fallopian tube cancer (PFTC), often just tubal cancer, is a malignant neoplasm that originates from the fallopian tube.
Uterine serous carcinoma (USC), 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.
Neuroendocrine carcinoma of the cervix is best defined separately:
Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma of the endometrium rather than the glands. There are three grades for endometrial stromal tumors, as follows. It was previously known as endolymphatic stromal myosis because of diffuse infiltration of myometrial tissue or the invasion of lymphatic channels.
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.
Adenosarcoma is a rare malignant tumor that occurs in women of all age groups, but most commonly post-menopause. Adenosarcoma arises from mesenchymal tissue and has a mixture of the tumoral components of an adenoma, a tumor of epithelial origin, and a sarcoma, a tumor originating from connective tissue. The adenoma, or epithelial component of the tumor, is benign, while the sarcomatous stroma is malignant. The most common site of adenosarcoma formation is the uterus, but it can also occur in the cervix and ovaries. It more rarely arises in the vagina and fallopian tubes as well as primary pelvic or peritoneal sites, such as the omentum, especially in those with a history of endometriosis. The rare cases of adenosarcoma outside the female genital tract usually occur in the liver, bladder, kidney, as well as the intestine and are typically associated with endometriosis.
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