The uterine sarcomas form a group of malignant tumors that arises from the
smooth muscle
Smooth muscle is an involuntary non-striated muscle, so-called because it has no sarcomeres and therefore no striations (''bands'' or ''stripes''). It is divided into two subgroups, single-unit and multiunit smooth muscle. Within single-unit mus ...
or
connective tissue
Connective tissue is one of the four primary types of animal tissue, along with epithelial tissue, muscle tissue, and nervous tissue. It develops from the mesenchyme derived from the mesoderm the middle embryonic germ layer. Connective tiss ...
of the
uterus
The uterus (from Latin ''uterus'', plural ''uteri'') or womb () is the organ in the reproductive system of most female mammals, including humans that accommodates the embryonic and fetal development of one or more embryos until birth. The uter ...
.
Signs and symptoms
Clinically, uterine sarcomas and
leiomyomas (fibroids) both have similar symptoms such as increased uterine size, abdominal pain and
vaginal bleeding
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 reproductiv ...
so it can be difficult to tell them apart.
Unusual or postmenopausal bleeding may be a sign of uterine sarcoma and needs to be investigated. Other signs include pelvic pain, pressure, and unusual discharge. A nonpregnant uterus that enlarges quickly is suspicious. However, none of the signs are specific. Specific screening test have not been developed; a
Pap smear
The Papanicolaou test (abbreviated as Pap test, also known as Pap smear (AE), cervical smear (BE), cervical screening (BE), or smear test (BE)) is a method of cervical screening used to detect potentially precancerous and cancerous processes in t ...
is a screening test for
cervical cancer
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 ...
and not designed to detect uterine sarcoma.
Histology
Tumoral entities include
leiomyosarcoma
Leiomyosarcoma is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. While leiomyosarcomas are not thought to arise from leiomyomas, some leiomyoma variants' classification is evolv ...
s,
endometrial stromal sarcoma
Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma (connective tissue) of the endometrium rather than the glands. There are three grades for endometrial stromal tumors, as follows. It was pre ...
s,
carcinosarcoma
Carcinosarcomas are malignant tumors that consist of a mixture of carcinoma (or epithelial cancer) and sarcoma (or mesenchymal/connective tissue cancer). Carcinosarcomas are rare tumors, and can arise in diverse organs, such as the skin, salivary ...
s and "other"
sarcoma
A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal (connective tissue) origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sarcom ...
s.
* If the lesion originates from the stroma of the
uterine lining it is an
endometrial stromal sarcoma
Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma (connective tissue) of the endometrium rather than the glands. There are three grades for endometrial stromal tumors, as follows. It was pre ...
.
* If the uterine
muscle
Skeletal muscles (commonly referred to as muscles) are organs of the vertebrate muscular system and typically are attached by tendons to bones of a skeleton. The muscle cells of skeletal muscles are much longer than in the other types of muscl ...
cell is the originator the tumor is a
uterine leiomyosarcoma
Leiomyosarcoma is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. While leiomyosarcomas are not thought to arise from leiomyomas, some leiomyoma variants' classification is evolvi ...
.
*
Carcinosarcoma
Carcinosarcomas are malignant tumors that consist of a mixture of carcinoma (or epithelial cancer) and sarcoma (or mesenchymal/connective tissue cancer). Carcinosarcomas are rare tumors, and can arise in diverse organs, such as the skin, salivary ...
s comprise both malignant epithelial and malignant sarcomatous components.
Diagnosis
By using
T2*-weighted imaging,
MRI
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...
is able to differentiate distinguishing features of leiomyomas from uterine sarcomas.
Investigations by the physician include imaging (
ultrasound
Ultrasound is sound waves with frequency, frequencies higher than the upper audible limit of human hearing range, hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hea ...
,
CAT scan,
MRI
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...
) and, if possible, obtaining a tissue diagnosis by
biopsy
A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a diseas ...
,
hysteroscopy
Hysteroscopy is the inspection of the uterine cavity by endoscopy with access through the cervix. It allows for the diagnosis of intrauterine pathology and serves as a method for surgical intervention (operative hysteroscopy).
Hysteroscope
A hyst ...
, or
D&C.
Ultimately the diagnosis is established by the histologic examination of the specimen. Typically malignant lesions have >10
mitosis
In cell biology, mitosis () is a part of the cell cycle in which replicated chromosomes are separated into two new nuclei. Cell division by mitosis gives rise to genetically identical cells in which the total number of chromosomes is mainta ...
per high power field. In contrast, a
uterine leiomyoma
Uterine fibroids, also known as uterine leiomyomas or fibroids, are benign smooth muscle tumors of the uterus. Most women with fibroids have no symptoms while others may have painful or heavy periods. If large enough, they may push on the bl ...
as a benign lesion would have < 5 mitoses per high power field.
Classification
Leiomyosarcoma
Leiomyosarcoma is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. While leiomyosarcomas are not thought to arise from leiomyomas, some leiomyoma variants' classification is evolv ...
s are now staged using the 2009 FIGO staging system
(previously they were staged like endometrial carcinomas) at the time of surgery.
*Stage I: tumor is limited to the uterus
:IA: ≤5 cm in greatest dimension
:IB: >5 cm
*Stage II: tumor extends beyond the uterus, but within the pelvis
:IIA: involves
adnexa of uterus
The uterine appendages (or adnexa of uterus) are the structures most closely related structurally and functionally to the uterus.
Terminology
They can be defined in slightly different ways:
* Some sources define the adnexa as the fallopian tubes ...
:IIB: involves other pelvic tissues
*Stage III: tumor infiltrates abdominal tissues
:IIIA: 1 site
:IIIB: >1 site
:IIIC: regional lymph node metastasis
*Stage IVA: invades bladder or rectum
*Stage IVB: distant metastasis (including intra-abdominal or inguinal lymph nodes; excluding adnexa, pelvic and abdominal tissues)
Endometrial stromal sarcoma
Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma (connective tissue) of the endometrium rather than the glands. There are three grades for endometrial stromal tumors, as follows. It was pre ...
s and
uterine adenosarcoma
Uterine adenosarcoma is an uncommon form of cancer that arises from mesenchymal tissue of the uterus and has a benign glandular component.
Signs and symptoms
The most common presentation is vaginal bleeding. Other presentations include pelvic mas ...
s are classified as above, with the exception of different classifications for Stage I tumors.
*Stage I: the tumor is limited to the uterus
:IA: limited to endometrium/endocervix
:IB: invades <½ myometrium
:IC: invades ≥½ myometrium
Finally,
malignant mixed Müllerian tumors, a type of
carcinosarcoma
Carcinosarcomas are malignant tumors that consist of a mixture of carcinoma (or epithelial cancer) and sarcoma (or mesenchymal/connective tissue cancer). Carcinosarcomas are rare tumors, and can arise in diverse organs, such as the skin, salivary ...
, are staged similarly to endometrial carcinomas.
*Stage I: the tumor is limited to the uterus
:IA: invades <½ myometrium
:IB: invades ≥½ myometrium
*Stage II: invades cervical stroma, but no extension beyond the uterus
*Stage III: local and/or regional spread
:IIIA: invades uterine serosa and/or adnexa
:IIIB: vaginal and/or parametrial involvement
:IIIC: metastases to pelvic and/or
paraaortic lymph node
The periaortic lymph nodes (also known as lumbar) are a group of lymph nodes that lie in front of the lumbar vertebrae near the aorta. These lymph nodes receive drainage from the gastrointestinal tract and the abdominal organs.
The periaortic ly ...
s
::IIIC1: positive pelvic nodes
::IIIC2: positive para-aortic lymph nodes
*Stage IVA: invades bladder and/or bowel mucosa
*Stage IVB: distant metastases (including intra-abdominal metastases and/or inguinal lymph nodes)
Management
Therapy is based on staging and patient condition and utilizes one or more of the following approaches.
Surgery
Surgery ''cheirourgikē'' (composed of χείρ, "hand", and ἔργον, "work"), via la, chirurgiae, meaning "hand work". is a medical specialty that uses operative manual and instrumental techniques on a person to investigate or treat a pat ...
is the mainstay of therapy if feasible involving total abdominal
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 b ...
with bilateral salpingo-oophorectomy. Other approaches include
radiation therapy
Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...
,
chemotherapy
Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...
, and
hormonal therapy
Hormone therapy or hormonal therapy is the use of hormones in medical treatment. Treatment with hormone antagonists may also be referred to as hormonal therapy or antihormone therapy. The most general classes of hormone therapy are hormonal therap ...
.
Prognosis is relatively poor.
Epidemiology
Uterine sarcoma is rare, out of all malignancies of the uterine body only about 4% will be uterine sarcomas. Generally, the cause of the lesion is not known, however, patients with a history of pelvic radiation are at higher risk. Most tumors occur 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 d ...
.
Women who take long-term
tamoxifen
Tamoxifen, sold under the brand name Nolvadex among others, is a selective estrogen receptor modulator used to prevent breast cancer in women and treat breast cancer in women and men. It is also being studied for other types of cancer. It has b ...
are at higher risk.
National Cancer Institute
The National Cancer Institute (NCI) coordinates the United States National Cancer Program and is part of the National Institutes of Health (NIH), which is one of eleven agencies that are part of the U.S. Department of Health and Human Services. ...
information, accessed 03-11-2006
See also
* Uterine fibroid
Uterine fibroids, also known as uterine leiomyomas or fibroids, are benign smooth muscle tumors of the uterus. Most women with fibroids have no symptoms while others may have painful or heavy periods. If large enough, they may push on the bl ...
s
* Leiomyosarcoma
Leiomyosarcoma is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. While leiomyosarcomas are not thought to arise from leiomyomas, some leiomyoma variants' classification is evolv ...
References
External links
*
Leiomyosarcoma of the Uterus: A Review
'
{{DEFAULTSORT:Uterine Sarcoma
Gynaecological cancer
Sarcoma