Leiomyosarcoma
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Leiomyosarcoma Microchapters |
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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
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.[1]
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.[2]
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.[3]
- 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.[4]
- History of Pelvic radiations:Pelvic irradiation has been associated with the increased development of the leiomyosarcoma.[5]
- 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.[6]
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
- ↑ Bell SW, Kempson RL, Hendrickson MR (1994) Problematic uterine smooth muscle neoplasms. A clinicopathologic study of 213 cases. Am J Surg Pathol 18 (6):535-58. PMID: 8179071
- ↑ 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