Uterine sarcoma

Last updated

The uterine sarcomas form a group of malignant tumors that arises from the smooth muscle or connective tissue of the uterus.

Contents

Signs and symptoms

Clinically, uterine sarcomas and leiomyomas (fibroids) both have similar symptoms such as increased uterine size, abdominal pain and vaginal bleeding so it can be difficult to tell them apart. [1] 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 is a screening test for cervical cancer and not designed to detect uterine sarcoma.

Histology

Tumoral entities include leiomyosarcomas, endometrial stromal sarcomas, carcinosarcomas and "other" sarcomas. [2]

Diagnosis

By using T2*-weighted imaging, MRI is able to differentiate distinguishing features of leiomyomas from uterine sarcomas. [1] Investigations by the physician include imaging (ultrasound, CAT scan, MRI) and, if possible, obtaining a tissue diagnosis by biopsy, hysteroscopy, or D&C. Ultimately the diagnosis is established by the histologic examination of the specimen. Typically malignant lesions have >10 mitosis per high power field. In contrast, a uterine leiomyoma as a benign lesion would have < 5 mitoses per high power field.

Classification

Leiomyosarcomas are now staged using the 2009 FIGO staging system [3] (previously they were staged like endometrial carcinomas) at the time of surgery.

IA: ≤5 cm in greatest dimension
IB: >5 cm
IIA: involves adnexa of uterus
IIB: involves other pelvic tissues
IIIA: 1 site
IIIB: >1 site
IIIC: regional lymph node metastasis

Endometrial stromal sarcomas and uterine adenosarcomas are classified as above, with the exception of different classifications for Stage I tumors.

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. [4]

IA: invades <½ myometrium
IB: invades ≥½ myometrium
IIIA: invades uterine serosa and/or adnexa
IIIB: vaginal and/or parametrial involvement
IIIC: metastases to pelvic and/or paraaortic lymph nodes
IIIC1: positive pelvic nodes
IIIC2: positive para-aortic lymph nodes

Management

Therapy is based on staging and patient condition and utilizes one or more of the following approaches. Surgery is the mainstay of therapy if feasible involving total abdominal hysterectomy with bilateral salpingo-oophorectomy. Other approaches include radiation therapy, chemotherapy, and hormonal therapy.

Prognosis is relatively poor. [5]

Epidemiology

Uterine sarcoma is rare, out of all malignancies of the uterine body only about 4% will be uterine sarcomas. [6] 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. Women who take long-term tamoxifen are at higher risk. [7]

See also

Related Research Articles

<span class="mw-page-title-main">Sarcoma</span> Medical condition

A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sarcomas can arise in any of these types of tissues. As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates. Sarcomas are primary connective tissue tumors, meaning that they arise in connective tissues. This is in contrast to secondary connective tissue tumors, which occur when a cancer from elsewhere in the body spreads to the connective tissue. Sarcomas are one of five different types of cancer, classified by the cell type from which they originate. The word sarcoma is derived from the Greek σάρκωμα sarkōma 'fleshy excrescence or substance', itself from σάρξsarx meaning 'flesh'.

<span class="mw-page-title-main">Uterine cancer</span> Medical condition

Uterine cancer, also known as womb cancer, includes two types of cancer that develop 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. Endometrial cancer accounts for approximately 90% of all uterine cancers in the United States. Symptoms of endometrial cancer include changes in vaginal bleeding or pain in the pelvis. Symptoms of uterine sarcoma include unusual vaginal bleeding or a mass in the vagina.

<span class="mw-page-title-main">Endometrial cancer</span> Uterine cancer that is located in tissues lining the uterus

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.

<span class="mw-page-title-main">Gastrointestinal stromal tumor</span> Human disease (cancer)

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. GISTs arise in the smooth muscle pacemaker interstitial cell of Cajal, or similar cells. They are defined as tumors whose behavior is driven by mutations in the KIT gene (85%), PDGFRA gene (10%), or BRAF kinase (rare). 95% of GISTs stain positively for KIT (CD117). Most (66%) occur in the stomach and gastric GISTs have a lower malignant potential than tumors found elsewhere in the GI tract.

This is a list of terms related to oncology. The original source for this list was the US National Cancer Institute's public domain Dictionary of Cancer Terms.

<span class="mw-page-title-main">Leiomyoma</span> Medical condition

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 to increased erythropoietin production as part of a paraneoplastic syndrome.

<span class="mw-page-title-main">Serous tumour</span> Medical condition

A serous tumour is a neoplasm that typically has papillary to solid formations of tumor cells with crowded nuclei, and which typically arises on the modified Mullerian-derived serous membranes that surround the ovaries in females. Such ovarian tumors are part of the surface epithelial-stromal tumour group of ovarian tumors. They are common neoplasms with a strong tendency to occur bilaterally, and they account for approximately a quarter of all ovarian tumors.

<span class="mw-page-title-main">Adenomyosis</span> Extension of endometrial tissue into the myometrium

Adenomyosis is a medical condition characterized by the growth of cells that proliferate on the inside of the uterus (endometrium) atypically located among the cells of the uterine wall (myometrium), as a result, thickening of the uterus occurs. As well as being misplaced in patients with this condition, endometrial tissue is completely functional. The tissue thickens, sheds and bleeds during every menstrual cycle.

<span class="mw-page-title-main">Uterine fibroid</span> Medical condition with benign tumors of uterus

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 bladder, causing a frequent need to urinate. They may also cause pain during penetrative sex or lower back pain. A woman can have one uterine fibroid or many. Occasionally, fibroids may make it difficult to become pregnant, although this is uncommon.

<span class="mw-page-title-main">Leiomyosarcoma</span> Medical condition

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 evolving.

Adenomyoma is a tumor (-oma) including components derived from glands (adeno-) and muscle (-my-). It is a type of complex and mixed tumor, and several variants have been described in the medical literature. Uterine adenomyoma, the localized form of uterine adenomyosis, is a tumor composed of endometrial gland tissue and smooth muscle in the myometrium. Adenomyomas containing endometrial glands are also found outside of the uterus, most commonly on the uterine adnexa but can also develop at distant sites outside of the pelvis. Gallbladder adenomyoma, the localized form of adenomyomatosis, is a polypoid tumor in the gallbladder composed of hyperplastic mucosal epithelium and muscularis propria.

<span class="mw-page-title-main">Mixed Müllerian tumor</span> Medical condition

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.

<span class="mw-page-title-main">Uterine serous carcinoma</span> Medical condition

Uterine serous carcinoma is a malignant form of serous tumor that originates in the uterus. It 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.

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.

Genital leiomyomas are leiomyomas that originate in the dartos muscles, or smooth muscles, of the genitalia, areola, and nipple. They are a subtype of cutaneous leiomyomas that affect smooth muscle found in the scrotum, labia, or nipple. They are benign tumors, but may cause pain and discomfort to patients. Genital leiomyoma can be symptomatic or asymptomatic and is dependent on the type of leiomyoma. In most cases, pain in the affected area or region is most common. For vaginal leiomyoma, vaginal bleeding and pain may occur. Uterine leiomyoma may exhibit pain in the area as well as painful bowel movement and/or sexual intercourse. Nipple pain, enlargement, and tenderness can be a symptom of nipple-areolar leiomyomas. Genital leiomyomas can be caused by multiple factors, one can be genetic mutations that affect hormones such as estrogen and progesterone. Moreover, risk factors to the development of genital leiomyomas include age, race, and gender. Ultrasound and imaging procedures are used to diagnose genital leiomyomas, while surgically removing the tumor is the most common treatment of these diseases. Case studies for nipple areolar, scrotal, and uterine leiomyoma were used, since there were not enough secondary resources to provide more evidence.

<span class="mw-page-title-main">Endometrial stromal sarcoma</span> Medical condition

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.

Cervical cancer staging is the assessment of cervical cancer to determine the extent of the disease. This is important for determining disease prognosis and treatment. Cancer staging generally runs from stage 0, which is pre-cancerous or non-invasive, to stage IV, in which the cancer has spread throughout a significant part of the body.

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.

References

  1. 1 2 Smith J, Zawaideh JP, Sahin H, Freeman S, Bolton H, Addley HC (September 2021). "Differentiating uterine sarcoma from leiomyoma: BET1T2ER Check!". Br J Radiol. 94 (1125): 20201332. doi:10.1259/bjr.20201332. PMC   9327746 . PMID   33684303. S2CID   232159216.
  2. Zagouri F, Dimopoulos AM, Fotiou S, Kouloulias V, Papadimitriou CA (2009). "Treatment of early uterine sarcomas: disentangling adjuvant modalities". World J Surg Oncol. 7: 38. doi:10.1186/1477-7819-7-38. PMC   2674046 . PMID   19356236.
  3. Yim GW, Nam EJ, Kim SW, Kim YT (May 2014). "FIGO staging for uterine sarcomas: can the revised 2008 staging system predict survival outcome better?". Yonsei Med J. 55 (3): 563–9. doi:10.3349/ymj.2014.55.3.563. PMC   3990069 . PMID   24719120.
  4. "Endometrial Cancer Treatment". 1980-01-01.
  5. Gadducci A, Cosio S, Romanini A, Genazzani AR (February 2008). "The management of patients with uterine sarcoma: a debated clinical challenge". Crit. Rev. Oncol. Hematol. 65 (2): 129–42. doi:10.1016/j.critrevonc.2007.06.011. PMID   17706430.
  6. American Cancer Society information, accessed 03-11-2006
  7. National Cancer Institute information, accessed 03-11-2006