Leiomyosarcoma
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Leiomyosarcoma Microchapters |
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Diagnosis |
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Case Studies |
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Leiomyosarcoma On the Web |
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American Roentgen Ray Society Images of Leiomyosarcoma |
For patient information click here.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Historical Perspective
Pathophysiology
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 aberration is yet to be elucidated.
- Over expression 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 tumor 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:
| Disease | Clinical Features | Physical Examination | Diagnosis |
| Gastric cancer | Abdominal pain, Weight loss, Heartburn, Belching,Nausea,Vomiting,Weight loss,Loss of Appetite,Dysphagia,Hemetemesis,Melena | Pallor,Jaundice,Acanthosis Nigrans,Leasr-Trelat signs,Left supraclavicular Lypmph adenopathy,Abdominal mass and distension,Splenomegaly,Ascites,Hepattomegaly. | Esophagogastroduodenoscopy |
| Endometrial Cancer | Abnormal Vaginal bleeding,Post coital bleeding,Fatigue,weight loss,Pelvic pain and dysparunia | Tachycardia,Tachypnea,Pallor,Jaundice,Vaginal bleeding, Enlarged uterus,Pedal Edema,Hepatomegaly,Lymphadenopathy,Decreased breath sounds(depending upon the staging of the cancer) | CBC,LFT,CA-125,CA,PFT,xray chest,CT,MRI,Ultrasound, Endometrial Curettage,D and C,Endometrial Biopsy and Tru test. |
| Peptic Ulcer Disease | Episodic epigastric pain,fever,melena,hematemesis | Hypotension if perforation,Abdominal guarding and rigidity,normal bowel sounds | Ascites fluid analysis for LDH,glucose,and protein,chest xray if perforation,Upper GI Endoscopy,Serum gastrin level,Secretin stimulation test,Biopsy |
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.[1]
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.[2]
- 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.[3]
- History of Pelvic radiations:Pelvic irradiation has been associated with the increased development of the leiomyosarcoma.[4]
- 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.[5]
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
Related Chapters
- ↑ Brooks SE, Zhan M, Cote T, Baquet CR (2004) Surveillance, epidemiology, and end results analysis of 2677 cases of uterine sarcoma 1989-1999. Gynecol Oncol 93 (1):204-8. DOI:10.1016/j.ygyno.2003.12.029 PMID: 15047237
- ↑ 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
- ↑ 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
- ↑ 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
- ↑ 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