Uterine clear-cell carcinoma (CC) is a rare form of
endometrial cancer
Endometrial cancer is a cancer that arises from the endometrium (the lining of the uterus or womb). 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 ...
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.
Diagnosis
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.
Histologically the lesion may coexist with classical endometrial cancer.
Treatment
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
Hysterectomy is the surgical removal of the uterus. It may also involve removal of the cervix, ovaries (oophorectomy), Fallopian tubes (salpingectomy), and other surrounding structures.
Usually performed by a gynecologist, a hysterectomy may be ...
, bilateral
salpingo-oophorectomy, pelvic/para-aortic
lymphadenectomy
Lymphadenectomy or lymph node dissection is the surgical removal of one or more groups of lymph nodes. It is almost always performed as part of the surgical management of cancer. In a regional lymph node dissection, some of the lymph nodes in the ...
, and
omentectomy. The tumor is aggressive and spreads quickly into the
myometrium
The myometrium is the middle layer of the uterine wall, consisting mainly of uterine smooth muscle cells (also called uterine myocytes) but also of supporting stromal and vascular tissue. Its main function is to induce uterine contractions.
Stru ...
and the
lymphatic system
The lymphatic system, or lymphoid system, is an organ system in vertebrates that is part of the immune system, and complementary to the circulatory system. It consists of a large network of lymphatic vessels, lymph nodes, lymphatic or lymphoid ...
. 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.
[
The five years survival was reported to be 68%.][
]
Staging
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.[International Journal of Gynecology and Obstetrics 105 (2009) 103–104 Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium]
IA Tumor confined to the uterus, no or < myometrial invasion
IB Tumor confined to the uterus, > 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
References
External links
{{DEFAULTSORT:Uterine Clear Cell Carcinoma
Gynaecological cancer