Leiomyosarcoma
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Leiomyosarcoma On the Web |
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American Roentgen Ray Society Images of Leiomyosarcoma |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Leiomyosarcoma is a type of sarcoma which is a neoplasm of smooth muscle. (When benign, it is called a leiomyoma.) Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin. Leiomyosarcoma is a very rare cancer. It makes up 7% of soft tissue sarcomas.
Historical Perspective
Soft tissue sarcoma derived their name from the Greek term for a fleshy excrescences.As early as Galen(130-200C.E.),it was suggested that they were a cancerous tumor.Early reports of myxoid liposarcoma by Severinius(1580-1637) and retroperitoneal sarcoma by Morgagni (1682-1771) have been recorded.An Edinburg surgeon, Wardrop( 1782-1869),who studied in Vienna,describes the term soft cancer.Charles Bell ( 1772-1842) ,in his book Surgical Observations, published in 1816, has been credited with the utilization of the term soft tissue sarcoma to differentiate it form carcinoma.In 1804, Abernethy describes the first classification of the sarcoma.In1932 Stout( 1885-1967) published a seminal monograph on the pathology and treatment of sarcoma.Ewing was one of the first to list benign and malignant counterparts of tumors arising in soft tissues.
Pathophysiology
The pathogenesis of leimyosarcoma is characterized by malignant smoth muscle neoplasm that can appear in any site in the body but most commonly found in the uterus,small intestine and retro peritoneum.
Causes
Exact cause of leiomyosarcoma is not clearly evident.
Differentiating Leiomyosarcoma from other Diseases:
Leiomyosarcoma must be differentiated from Peptic ulcer, Stomach cancer, Uterine cancer.
Epidemiology and Demographics:
Leiomyosarcoma is one the most common types of soft tissue sarcoma, about 1 person in 100,000 gets diagnosed with LMS each year.Uterine sarcoma are extremely rare, with the incidence of 3 to 7 per 100,000 US population however rate of sarcoma appears to be rising as compared to early years
Risk Factors:
Clinical data has suggested that the development of leiomyosarcoma is related to radiation exposure. Other causes are not known for certain.
Natural History, Complications and Prognosis
Leiomyosarcoma most commonly metastasizes to the lungs, liver, abdomen, pelvis, and pelvic or paraaortic lymph nodes.Bone and brain metastases are less common sites of involvement.Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor.Overall survival rate ranges from 15% to 25% with a median survival of only 10 months.
Diagnosis
The majority of patients with leiomyosarcoma remain asymptomatic for decades. General symptoms associated with cancer may occur including fatigue, fever, weight loss, a general feeling of ill health (malaise), and nausea and vomiting.Patients with Leiomyosarcoma generally appear weak.Physical examination findings depends upon the site of the involvement,size and metastasis.Physical Examination findings may include weight loss, abdominal mass, Distension,Ascites, rectal bleeding and vaginal bleeding.
There are no specific laboratory findings associated with Leiomyosarcoma. However, we may find elevated levels of CA 125 and LDH( isozymes) in women with uterine sarcoma.However,usefulness of these markers are still being investigated.Plain x-ray of chest may be done to see if the sarcoma has spread to the lungs.CT scans are often used to diagnose leiomyosarcoma. It can confirm the location of the sarcoma and show the organs nearby. These are helpful for determining the stage of the cancer and in determining whether surgery is a good treatment option. CT scans can also be used to guide biopsy and a biopsy sample is usually removed and looked at under a microscope.MRI is an imaging method using signal detection from spinning protons exposed to a magnetic field and offers superior soft tissue contrast to and better evaluation of tumor size, spread, and neurovascular involvement than CT.It also has a valuable role in follow-up for the evaluation for tumor recurrence.Leiomyosarcoma is generally a diagnostic dilemma requires the use of both radiology and pathology for their accurate diagnosis.Ultrasonography is relatively cheap,non invasive and easily available technology in early detection of soft tissue mass.
PET and PET/CT and Bone scan may prove particularly useful in evaluating patients who have undergone surgery in looking for local disease recurrence, or in the search for metastatic lesions.
History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Leiomyosarcoma treatment consist of multidisciplinary approach and is best carried out at the specialized hospital setting.Patients with leiomyosarcoma have many treatment options. The selection depends on the stage of the tumor. The options are surgery, radiation therapy, chemotherapy, or a combination of these methods. Before treatment starts, ask your health care team about possible side effects and how treatment may change your normal activities. Because cancer treatments often damage healthy cells and tissues, side effects are common. Side effects may not be the same for each person, and they may change from one treatment session to the next.Non surgical treatment options have shown only limited benefit in the treatment of the Leiomyosarcoma and are generally considered to be less responsive of chemotherapy and radiotherapy.It has generally noted that only 40% cases responds to the chemotherapeutic regimens.
Medical Therapy | Surgery | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies