The uterine sarcomas form a group of malignant tumors that arises from the

smooth muscle Smooth muscle is one of the three major types of vertebrate muscle tissue, the others being skeletal and cardiac muscle. It can also be found in invertebrates and is controlled by the autonomic nervous system. It is non- striated, so-called bec ...

connective tissue Connective tissue is one of the four primary types of animal tissue, a group of cells that are similar in structure, along with epithelial tissue, muscle tissue, and nervous tissue. It develops mostly from the mesenchyme, derived from the mesod ...

of the

uterus The uterus (from Latin ''uterus'', : uteri or uteruses) or womb () is the hollow organ, organ in the reproductive system of most female mammals, including humans, that accommodates the embryonic development, embryonic and prenatal development, f ...

. They can be difficult to detect, as symptoms are common to other uterine conditions and no specific screening test has been developed. This presents an issue for treatment, as the cancer spreads quickly. Uterine sarcoma is rare and mostly occurs in post-menopausal women. The most common type, leiomyosarcoma, is twice as common in Black women as it is in white women. Uterine sarcoma condition is most commonly treated by

radical hysterectomy Hysterectomy is the surgical removal of the uterus and cervix. Supracervical hysterectomy refers to removal of the uterus while the cervix is spared. These procedures may also involve removal of the ovaries (oophorectomy), fallopian tubes (salpin ...

. If cancer has spread beyond the uterus, radiation, chemotherapy, and hormonal therapy may be used. If detected in its early stages, survival rates for uterine sarcoma are 66% after 5 years. If cancer has spread beyond the uterus, the survival rate declines to approximately 13-34%.

Signs and symptoms

Clinically, uterine sarcomas and

leiomyomas A leiomyoma, also known as a fibroid, is a benign smooth muscle tumor that very rarely becomes cancer (0.1%). They can occur in any organ, but the most common forms occur in the uterus, small bowel, and the esophagus. Polycythemia may occur due t ...

(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 ...

is a screening test for

cervical cancer Cervical cancer is a cancer arising from the cervix or in any layer of the wall of the cervix. It is due to the abnormal growth of cells that can invade or spread to other parts of the body. Early on, typically no symptoms are seen. Later sympt ...

and not designed to detect uterine sarcoma.

Histology

Tumoral entities include

leiomyosarcoma A leiomyosarcoma (LMS) is a rare malignant (cancerous) smooth muscle tumor. The word is . The stomach, bladder, uterus, blood vessels, and Gastrointestinal tract, intestines are examples of hollow organs made up of smooth muscles where LMS can be ...

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 prev ...

carcinosarcoma Carcinosarcomas are malignant tumors that consist of a Mixed tumor, 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 ski ...

s and "other"

sarcoma A sarcoma is a rare type of cancer that arises from cells of mesenchymal origin. Originating from mesenchymal cells means that sarcomas are cancers of connective tissues such as bone, cartilage, muscle, fat, or vascular tissues. Sarcom ...

s. * If the lesion originates from the stroma of the

uterine lining 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 ...

it is an

* If the uterine

muscle Muscle is a soft tissue, one of the four basic types of animal tissue. There are three types of muscle tissue in vertebrates: skeletal muscle, cardiac muscle, and smooth muscle. Muscle tissue gives skeletal muscles the ability to muscle contra ...

cell is the originator the tumor is a uterine leiomyosarcoma. *

Carcinosarcoma Carcinosarcomas are malignant tumors that consist of a Mixed tumor, 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 ski ...

s comprise both malignant epithelial and malignant sarcomatous components.

Diagnosis

By using

T2*-weighted imaging ''T''2*-weighted imaging is an MRI sequence to quantify observable or effective ''T''2 (T2* or "T2-star"). In this sequence, hemorrhages and hemosiderin deposits become hypointense. Physics ''T''2*-weighted imaging is built from the basic physi ...

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...

is able to differentiate distinguishing features of leiomyomas from uterine sarcomas. Investigations by the physician include imaging (

ultrasound Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...

CAT scan A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers ...

) and, if possible, obtaining a tissue diagnosis by

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, an interventional radiologist, or an interventional cardiology, interventional cardiologist. The process involves the extraction of sampling (medicine), sample ...

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 Mitosis () is a part of the cell cycle in eukaryote, eukaryotic cells in which replicated chromosomes are separated into two new Cell nucleus, nuclei. Cell division by mitosis is an equational division which gives rise to genetically identic ...

per high power field. In contrast, a

uterine leiomyoma Uterine fibroids, also known as uterine leiomyomas, fibromyoma or fibroids, are benign smooth muscle tumors of the uterus, part of the female reproductive system. Most people with fibroids have no symptoms while others may have painful or h ...

as a benign lesion would have < 5 mitoses per high power field.

Classification

Leiomyosarcoma A leiomyosarcoma (LMS) is a rare malignant (cancerous) smooth muscle tumor. The word is . The stomach, bladder, uterus, blood vessels, and Gastrointestinal tract, intestines are examples of hollow organs made up of smooth muscles where LMS can be ...

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 tube ...

: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 prev ...

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 ma ...

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

, 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 lym ...

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 is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery s ...

is the mainstay of therapy if feasible involving total abdominal

hysterectomy Hysterectomy is the surgical removal of the uterus and cervix. Supracervical hysterectomy refers to removal of the uterus while the cervix is spared. These procedures may also involve removal of the ovaries (oophorectomy), fallopian tubes ( salpi ...

with bilateral salpingo-oophorectomy. Other approaches include

radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...

chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

, 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 oncologic hor ...

. 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 when Menstruation, menstrual periods permanently stop, marking the end of the Human reproduction, reproductive stage for the female human. It typically occurs between the ages of 45 and 5 ...

. 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 men. It is also being studied for other types of cancer. It has been used for Albright syndrome ...

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

References

External links

*
Leiomyosarcoma of the Uterus: A Review
' {{DEFAULTSORT:Uterine Sarcoma Gynaecological cancer Sarcoma