Leiomyosarcoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: '{{Nnasiri}
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 lump.
- Around 460 B.C, Hippocrates recognized soft tissue tumors in the arm and thigh in older people and recommended the removal by knife as therapy.
- Around 200 C.E., a greek physician named Galen suggested that these lumps were cancerous tumors.
- In 1809, an Edinburg surgeon, Wardrop describes the first illustration of amputation and used the term "soft cancer".
- In 1816, Charles Bell, used the term soft tissue sarcoma to differentiate it form carcinoma.
- In 1804, Abernethy describes the first classification of the sarcoma.
- In 1932, Stout published a litreature on the pathology and treatment of sarcoma.
- In 1982, Rosenberg evaluated limb-sparing surgery with adjuvant radiotherapy compared with major amputation.
Pathophysiology
The pathogenesis of leimyosarcoma is characterized by malignant smooth 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 because of the rarety of disease nature. It may arise denovo or by genetic instability.
Differentiating Leiomyosarcoma from other Diseases:
Leiomyosarcoma must be differentiated from special subtypes of leiomyoma, othter soft tissue sarcoma, perivascular epitheloid cell neoplasm (PEComa).
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, it is twice more common in African-American women, median age of patients is between 40 to 52 years old.
Risk Factors:
Clinical data has suggested that the development of leiomyosarcoma is related to radiation exposure. Other risk factors include use of tamoxifen, immunodeficiency, viral infection.
Natural History, Complications and Prognosis
Leiomyosarcoma most commonly metastasizes to the lungs, abdomen, pelvis, and 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. Stage of tumor is the most important prognostic factor.
Diagnosis
The majority of patients with leiomyosarcoma remain asymptomatic for decades. General symptoms associated with cancer may occur including fatigue, fever, weight loss, and nausea and vomiting. Irregular vaginal bleeding is most common symptom in patients with leiomyosarcoma, other symptoms include feeling pressure in pelvic or abdomen, painless growing mass in abdominal cavity, vaginal discharge.
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. 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.
Medical Therapy | Surgery | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies