Leiomyosarcoma

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Leiomyosarcoma from other Diseases

Epidemiology and Demographics

Risk Factors

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

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Leiomyosarcoma from other Diseases:

Epidemiology and Demographics:

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.[1]
  • Long term use of the tamoxifen: Prolonged use of the tamoxifen esp more than five years has been associated with the increased risk of development of leiomyosarcoma.[2]
  • History of Pelvic radiations:Pelvic irradiation has been associated with the increased development of the leiomyosarcoma.[3]
  • Certain Hereditary condition like Gardner syndrome, Li-Fraumeni syndrome,Werner syndrome and Neurofibromatosis are associated with the development of leiomyosarcoma.
  • Long term survivors of the Retinoblastoma are at higher risk of developing variety of soft tissue sarcoma.[4]

Natural History, Complications and Prognosis

  • The majority of patients with leiomyosarcoma remain asymptomatic for decades.
  • Most leiomyosarcomas are diagnosed incidentally at an advanced stage.
  • They are invariably aggressive and associated with the poor prognosis.

Prognosis: The prognosis of leiomyosarcoma is poor and it depends on varying factors:

  • Tumor size
  • Tumor location
  • Tumor type/Grade
  • DNA content
  • Hormonal receptor status
  • cellular division and mitotic rate
  • local and distant extension

Survival Rate of Leiomyosarcoma by staging:

Stage Percentage
stage 1 60
stage 2 35
stage 3 28
stage 4 15

Diagnosis

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.

Medical Therapy | Surgery | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

Uterine sarcoma


Template:WikiDoc Sources

  1. Wysowski DK, Honig SF, Beitz J (2002) Uterine sarcoma associated with tamoxifen use. N Engl J Med 346 (23):1832-3. DOI:10.1056/NEJM200206063462319 PMID: 12050351
  2. Mourits MJ, De Vries EG, Willemse PH, Ten Hoor KA, Hollema H, Van der Zee AG (2001) Tamoxifen treatment and gynecologic side effects: a review. Obstet Gynecol 97 (5 Pt 2):855-66. PMID: 11336777
  3. Fang Z, Matsumoto S, Ae K, Kawaguchi N, Yoshikawa H, Ueda T et al. (2004) Postradiation soft tissue sarcoma: a multiinstitutional analysis of 14 cases in Japan. J Orthop Sci 9 (3):242-6. DOI:10.1007/s00776-004-0768-5 PMID: 15168177
  4. Yu CL, Tucker MA, Abramson DH, Furukawa K, Seddon JM, Stovall M et al. (2009) Cause-specific mortality in long-term survivors of retinoblastoma. J Natl Cancer Inst 101 (8):581-91. DOI:10.1093/jnci/djp046 PMID: 19351917