Leiomyosarcoma

(medicine) A malignant tumor composed of anaplastic smooth muscle cells.

Key Terms: Biopsy, Chemotherapy, Oncologist, Pathologist, Radiation therapy.

Definition

Leiomyosarcoma is cancer that consists of smooth muscle cells and small cell sarcoma tumor. The cancer begins in smooth muscle cells that grow uncontrollably and form tumors.

Description

Leiomyosarcomas can start in any organ that contains smooth muscle, but can be found in the walls of the stomach, large and small intestines, esophagus, uterus, or deep within the abdomen (retroperitoneal). But for perspective, smooth muscle cancers are quite rare: Less than 1% of all cancers are leiomyosarcomas. Very rarely, leiomyosarcomas begin in blood vessels or in the skin.

Most leiomyosarcomas are in the stomach. The second most common site is the small bowel, followed by the colon, rectum, and esophagus.

Demographics

Leiomyosarcomas do occur in the breast and uterus, but they are very rare. Uterine sarcomas comprise less than 1% of gynecological malignancies and 2% to 5% of all uterine malignancies. Of these numbers, leiomyosarcomas are found in only 0.1% of women of childbearing age who have tumors of the uterus. Less than 2% of tumors in women over age 60 who are undergoing hysterectomy are leiomyosarcomas.

Causes and Symptoms

The exact causes of leiomyosarcoma are not known, but there are genetic and environmental risk factors associated with it. Certain inherited conditions that run in families may increase the risk of developing leiomyosarcoma. High-dose radiation exposure, such as radiotherapy used to treat other types of cancer, has also been linked to leiomyosarcoma. It is possible that exposure to certain chemical herbicides may increase the risk of developing sarcomas, but this association has not been proven.

Since leiomyosarcoma can occur in any location, the symptoms are different and depend on the site of the tumor. When leiomyosarcoma begins in an organ in the abdomen, such as the stomach or small bowel, the physician may be able to feel a large lump or mass when he examines the abdomen. When leiomyosarcoma affects a blood vessel, it may block the flow of blood to the body part supplied by the artery. Commonly occurring symptoms include:

• painless lump or mass
• painful swelling
• abdominal pain
• weight loss
• nausea and vomiting

Diagnosis

Some patients who have leiomyosarcomas may be visiting the doctor because they have discovered a lump or mass or swelling on a body part. Others have symptoms related to the internal organ that is affected by the leiomyosarcoma. For example, a tumor in the stomach may cause nausea, feelings of fullness, internal bleeding, and weight loss. The patient's doctor will take a detailed medical history to find out about the symptoms. The history is followed by a complete physical examination with special attention to the suspicious symptom or body part.

Depending on the location of the tumor, the doctor may order imaging studies such as x ray, computed tomography (CT) scan, and magnetic resonance imaging (MRI) to help determine the size, shape, and exact location of the tumor. A biopsy of the tumor is necessary to make the definitive diagnosis of leiomyosarcoma. The tissue sample is examined by a pathologist (specialist in the study of diseased tissue).

Types of Biopsy

The type of biopsy done depends on the location of the tumor. For some small tumors, the doctor may perform an excisional biopsy, removing the entire tumor and a margin of surrounding normal tissue. Most often, the doctor will perform an incisional biopsy, a procedure that involves cutting out only a piece of the tumor that is used to determine its type and grade.

Treatment Team

Patients with leiomyosarcoma are usually cared for by a multidisciplinary team of health professionals. The patient's family or primary care doctor may refer the patient to other specialists, such as surgeons and oncologists (specialists in cancer medicine), radiologic technicians, nurses, and laboratory technicians. Depending on the tumor location and treatment plan, patients may benefit from rehabilitation therapy with physical therapists and nutritional counseling from dieticians.

Clinical Staging, Treatments, and Prognosis

Staging

The purpose of staging a tumor is to determine how far it has advanced. This is important because treatment varies depending on the stage. Stage is determined by the size of the tumor, whether the tumor has spread to nearby lymph nodes, whether the tumor has spread elsewhere in the body, and what the cells look like under the microscope.

Examining the tissue sample under the microscope, using special chemical stains, the pathologist is able to classify tumors as high grade or low grade. High-grade tumors have the more rapidly growing cells and so are considered more serious.

Tumors are staged using numbers I through IV. The higher the number, the more the tumor has advanced. Stage IV leiomyosarcomas have involved either lymph nodes or have spread to distant parts of the body.

Treatment

Treatment for leiomyosarcoma varies depending on the location of the tumor, its size and grade, and the extent of its spread. Treatment planning also takes into account the patient's age, medical history, and general health.

Leiomyosarcomas on the arms and legs may be treated by amputation (removal of the affected limb) or by limb-sparing surgery to remove the tumor. These tumors may also be treated with radiation therapy, chemotherapy, or a combination of both.

Generally, tumors inside the abdomen are surgically removed. The site, size, and extent of the tumor determine the type of surgery performed. Leiomyosarcomas of organs in the abdomen may also be treated with radiation and chemotherapy.

Side Effects

The surgical treatment of leiomyosarcoma carries risks related to the surgical site, such as loss of function resulting from amputation or from nerve and/or muscle loss. There also are risks associated with any surgical procedure, such as reactions to general anesthesia or infection after surgery.

The side effects of radiation therapy depend on the site being radiated. Radiation therapy can produce side effects such as fatigue, skin rashes, nausea, and diarrhea. Most of the side effects lessen or disappear completely after the radiation therapy has been completed.

The side effects of chemotherapy vary depending on the medication, or combination of anticancer drugs, used. Nausea, vomiting, anemia, lower resistance to infection, and hair loss (alopecia) are common side effects. Medication may be given to reduce the unpleasant side effects of chemotherapy.

Alternative and Complementary Therapies

Many patients explore alternative and complementary therapies to help to reduce the stress associated with illness, improve immune function, and feel better. While there is no evidence that these therapies specifically combat disease, activities such as biofeedback, relaxation, therapeutic touch, massage therapy, and guided imagery have been reported to enhance well-being.

Prognosis

The outlook for patients with leiomyosarcoma varies. It depends on the location and size of the tumor and its type and extent of spread. Some patients, such as those who have had small tumors located in or near the skin surgically removed, have excellent prognoses. Their 5-year survival is greater than 90%. Among patients with leiomyosarcomas in organs in the abdomen, survival is best when the tumor has been completely removed. In general, high-grade tumors that have spread widely throughout the body are not associated with favorable survival rates.

Coping With Cancer Treatment

Fatigue is one of the most common complaints during cancer treatment and recovery. Many patients benefit from learning energy-conserving approaches to accomplish their daily activities. They should be encouraged to rest when tired and take breaks from strenuous activities. Planning activities around times of day when energy is highest is often helpful. Mild exercise, small, frequent nutritious snacks, and limiting physical and emotional stress also help to combat fatigue.

Depression, emotional distress, and anxiety associated with the disease and its treatment may respond to counseling from a mental health professional. Many cancer patients and their families find participation in mutual aid and group support programs helps to relieve feelings of isolation and loneliness. By sharing problems with others who have lived through similar difficulties, patients and families can exchange ideas and coping strategies.

Clinical Trials

Several clinical studies were underway as of 2001. For example, doctors at Memorial Sloan-Kettering Cancer Center were using specific chemotherapeutic drugs to treat patients with leiomyosarcoma that cannot be removed by surgery or has recurred. These drugs, gemcitabine, docetaxel, and filgrastim (G-CSF), work by stopping tumor cells from dividing, so they cannot grow. To learn more about this clinical trial and the availability of others, patients and families may wish to contact Memorial Sloan-Kettering Cancer Center at (212) 639-6555, or visit the National Cancer Institute (NCI) website at .

Prevention

Since the causes of leiomyosarcoma are not known, there are no recommendations about how to prevent its development. It is linked to radiation exposure; however, much of this excess radiation exposure is the result of therapy to treat other forms of cancer. Among families with an inherited tendency to develop soft tissue sarcomas, careful monitoring may help to ensure early diagnosis and treatment of the disease.

Questions to Ask the Doctor

• What stage is the leiomyosarcoma?
• What are the recommended treatments?
• What are the side effects of the recommended treatment?
• Is treatment expected to cure the disease or only to prolong life?

Special Concerns

Leiomyosarcoma, like other cancer diagnoses, may produce a range of emotional responses. Education, counseling, and participation in support group programs may help to reduce feelings of fear, anxiety and hopelessness. For many patients suffering from spiritual distress, visits with clergy members and participation in organized prayer may offer comfort.

Resources

Books

Pelletier, Kenneth R. The Best of Alternative Medicine. New York: Simon & Schuster, 2000.

Periodicals

Ishida, J., et al. "Primary Leiomyosarcoma of the Greater Omentum." Journal Of Clinical Gastroenterology 28, no. 2 (March 1999): 167–170.

Organizations

American Cancer Society. 1599 Clifton Road, N.E., Atlanta, GA 30329. (800) 227–2345.

Cancer Research Institute. 681 Fifth Avenue, New York, NY 10022. (800) 992–2623.

National Cancer Institute (National Institutes of Health). 9000 Rockville Pike, Bethesda, MD 20892. (800) 422–6237.

Other

National Cancer Institute Clinical Cancer Trials..

—Barbara Wexler, M.P.H.

A malignant neoplasm of muscle that contains spindle cells of unstriated muscle.

A sarcoma containing cells of smooth muscle.

Leiomyosarcoma
Classification and external resources
Leiomyosarcoma of the adrenal vein. Sagittal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.
ICD-10	C49..M48
ICD-O:	M8890/3
DiseasesDB	34362
eMedicine	med/1180
MeSH	D007890

Leiomyosarcoma (Gr. "smooth muscle connective tissue tumor"), aka LMS, is rare type of cancer that is a malignant neoplasm of smooth muscle. (When a such a neoplasm is benign, it is a leiomyoma.) Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body, including the uterus, stomach and intestines, the walls of all blood vessels, and the skin. It is therefore possible for leiomyosarcomas to appear at any site in the body (including the breasts); they are most commonly found in the stomach, small intestine and retroperitoneum.^[1]

Leiomyosarcoma is a relatively rare form of cancer, comprising between 5-10% of soft tissue sarcomas, which are in themselves relatively rare.^[2] Leiomyosarcomas can be very unpredictable. They can remain dormant for long periods of time and recur after years. It is a resistant cancer, meaning generally not very responsive to chemotherapy or radiation. The best outcomes occur, as with all cancers, when it can be removed surgically with wide margins early, while small and still in situ.^[3]

Uterine leiomyosarcomas come from the smooth muscle in the muscle layer of the uterus. Cutaneous leiomyosarcomas derive from the pilo-erector muscles in the skin. Gastrointestinal leiomyosarcomas might come from smooth muscle in the GI tract or, alternatively, also from a blood vessel. At most other primary sites, retroperitoneal extremity (in the abdomen, behind the intestines), truncal, abdominal organs, et al, leiomyosarcomas appear to grow from the muscle layer of a blood vessel^{[clarification needed]}, thus a leiomyosarcoma can have a primary site of origin anywhere in the body where there is a blood vessel.^[4]

Contents 1 Treatment 2 Notable patients 3 See also 4 References 5 External links

Treatment

Surgery, with as wide a margin of removal as possible, has generally been the most effective and preferred way to attack LMS. If surgical margins are narrow or not clear of tumor, however, or in some situations where tumor cells were left behind, chemotherapy or radiation has been shown to give a clear survival benefit.^[5] While LMS tends to be resistant to radiation and chemotherapy, each case is different and results can vary widely. Regardless, anyone diagnosed with LMS should see a sarcoma oncologist (not just a general oncologist) as soon as possible.

Complementary methods (such as over-the-counter supplements) should always be discussed with one's doctor(s); they can be helpful, but some supplements can actually hinder treatments like chemotherapy.

Notable patients

Notable people who have had leiomyosarcoma include:

• Katie Price (Jordan)^[6]
• Canadian public health physician Sheela Basrur
• American actress Diana Sands
• Canadian comedian Irwin Barker, who was featured in a documentary, "That's my Time", which chronicled his battle with leiomyosarcoma. In Barker's own words. “Cancer has my body but not my spirit, and I'll continue to make jokes, not so much about cancer, but in spite of it.”^{[citation needed]}

See also

• Uterine sarcoma

References

1. ^ Piovanello P, Viola V, Costa G, et al. (2007). "Locally advanced leiomyosarcoma of the spleen. A case report and review of the literature". World J Surg Oncol 5 (1): 135. doi:10.1186/1477-7819-5-135. PMID 18045454. http://www.wjso.com/content/5/1/135. 
2. ^ Weaver MJ & Abraham JA (2007). "Leiomyosarcoma of the Bone and Soft Tissue: A Review". ESUN 4 (2). http://sarcomahelp.org/learning_center/leiomyosarcoma.html. 
3. ^ Basic info
4. ^ Basic info., ibid.
5. ^ [1]
6. ^ "Jordan treated for cancer". BBC Online. 2002-08-11. http://news.bbc.co.uk/1/hi/uk/2185943.stm. Retrieved 2009-10-30. 

External links

• Comprehensive reference site for information on leiomyosarcoma
• National Leiomyosarcoma Foundation
• Leiomyosarcoma Direct Research Foundation
• CancerBackup
• Liddy Shriver Sarcoma Initiative
• Sloan Kettering website
• #2434 (Pathology images)
• Gastrointestinal Stromal Tumors at eMedicine

v • d • e Connective/soft tissue tumors and sarcomas (ICD-O 8800-9059) (C45-C49/D17-D21, 171/214-215) Not otherwise specified (8800-8809) Soft tissue sarcoma · Desmoplastic small round cell tumor Connective tissue neoplasm Fibromatous (8810-8839) Fibroma/fibrosarcoma: Dermatofibrosarcoma · Dermatofibrosarcoma protuberans · Desmoplastic fibroma Histiocytoma/histiocytic sarcoma: Benign fibrous histiocytoma · Malignant fibrous histiocytoma Myxomatous (8840-8849) Myxoma/myxosarcoma · Angiomyxoma · Ossifying fibromyxoid tumour Fibroepithelial (9000-9039) Brenner tumour · Fibroadenoma · Phyllodes tumor Synovial-like (9040-9049) Synovial sarcoma · Clear-cell sarcoma Lipomatous (8850-8889) Lipoma/liposarcoma (Myelolipoma, Myxoid liposarcoma) · PEComa (Angiomyolipoma) Myomatous (8890-8929) general: Myoma/myosarcoma smooth muscle: Leiomyoma/leiomyosarcoma skeletal muscle: Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma (Sarcoma botryoides) · Alveolar rhabdomyosarcoma Complex Mixed And Stromal (8930-8999) Adenomyoma · Pleomorphic adenoma · Mixed Müllerian tumor · Mesoblastic nephroma · Wilms' tumor · Rhabdoid tumour · Clear-cell sarcoma of the kidney · Hepatoblastoma · Pancreatoblastoma · Carcinosarcoma Mesothelial (9050-9059) Mesothelioma · Adenomatoid tumor muscle, DF+DRCT navs: anat/hist/physio, acquired myopathy/congenital myopathy/neoplasia, symptoms+signs/eponymous, proc muscle, DF+DRCT navs: anat/hist/physio, acquired myopathy/congenital myopathy/neoplasia, symptoms+signs/eponymous, proc

v • d • e Tumors: urogenital neoplasia · genital neoplasia (C51-C63/D25-29, 179-187/218-222) Female Ovaries Glandular and epithelial Clear cell adenocarcinoma · Endometrioid tumor CMS: Krukenberg tumor · Serous cystadenoma/Mucinous cystadenoma · Cystadenocarcinoma/Papillary serous cystadenocarcinoma Sex cord-gonadal stromal Leydig cell tumour · Sertoli cell tumour · Sertoli-Leydig cell tumour · Thecoma · Granulosa cell tumour · Luteoma Connective tissue Fibroma (Meigs syndrome) · Surface epithelial-stromal tumor (Brenner tumour) Germ cell Dysgerminoma · Embryonal carcinoma · Gonadoblastoma · Teratoma · Choriocarcinoma Fallopian tube Adenomatoid tumor Uterus Uterine sarcoma · Leiomyosarcoma Endometrium (Endometrioid tumor), (Uterine papillary serous carcinoma), (Clear cell carcinoma) Adenomyoma Cervix (SCC, Cervical intraepithelial neoplasia) Vagina SCC · Botryoid rhabdomyosarcoma · Adenocarcinoma/Clear cell adenocarcinoma Vulva Papillary hidradenoma · Extramammary Paget's disease Placenta Choriocarcinoma Male Testicles Sex cord-gonadal stromal Sertoli-Leydig cell tumour (Sertoli cell tumor, Leydig cell tumor) Germ cell G Seminoma (Spermatocytic seminoma) · Intratubular germ cell neoplasia NG Embryonal carcinoma · Endodermal sinus tumor · Gonadoblastoma · Teratoma · Choriocarcinoma · Embryoma Prostate Adenocarcinoma · Prostatic intraepithelial neoplasia (HGPIN) · Small cell carcinoma · Transitional cell carcinoma Penis Carcinoma (Extramammary Paget's disease) · Bowen's disease · Bowenoid papulosis · Erythroplasia of Queyrat reproductive system navs: anat female,male/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

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