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 mu ...

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. Th ...

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 d ...

(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 reproducti ...

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 evol ...

s, endometrial stromal sarcomas, carcinosarcomas 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 sarcoma ...

s. * If the lesion originates from the stroma of the uterine lining it is an endometrial stromal sarcoma. * 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 m ...

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

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

ultrasound Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies ...

, CAT scan,

) 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 disea ...

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 h ...

, 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 maintai ...

per high power field. In contrast, a uterine leiomyoma 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 evol ...

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 :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 sarcomas and uterine adenosarcomas 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, 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 ''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 be ...

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. Rad ...

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. Chemother ...

, and hormonal therapy. 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 ...

. 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

References

External links

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