Leiomyosarcoma
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Leiomyosarcoma On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Historical Perspective
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.
- On gross pathology, leiomyosarcoma appears to be soft, large, yellow or tan solitary masses with fleshy cut surfaces and areas of hemorrhage and necrosis.
- On microscopic histopathological analysis,prominent cellular atypia, abundant mitoses(15-30 per 10 high power field),and areas of coagulative necrosis are characteristic features of the leiomyosarcoma.
Uterine Leiomyosarcoma:
Causes
- Exact cause of leiomyosarcoma is not clearly evident.
- Although not proven but some reasearchers believes that it could be a malignant transformation of the fibroid( leiomyoma).
- It may arise denovo or can be due to the genomic instabilty. However molecular mechanism of tumorgenesis driven by genomic and genetic abervation is yet to be elucidated.
- Overexpression of the protooncogene C-myc is seen in 50% of the cases.
- Retinobalstoma gene discrepancies are also seen in 90% of cases.
- P16 chromosomal deletion causes loss of tumour suppression and so is involved in the development of leiomyosarcoma.
- P53 gene mutation is also frequently seen in uterine leiomyosarcoma
Differentiating Leiomyosarcoma from other Diseases
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: Following Risk Fcators found to be associated with the development of the Uterine leiomyosarcoma
- Age: Leiomyosarcoma can be seen at 20 years of age but mean age at diagnosis is 50-60 years old.
- Race:Black women have a higher incidence of development of the uterine leiomyosarcoma as compared to the white women.
- Long term use of the tamoxifen: Prolonged use of the tamoxifen esp more than five years has been associated with the increased use of development of leiomyosarcoma
- History of Pelvic radiations:Pelvic irradiation has been associated with the increased development of the leiomyosarcoma.
- Certain Hereditary condition
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies