Leiomyosarcoma

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

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.

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
Peptic Ulcer Disease	Episodic Epigastric pain,Fever,Melena,Hematemesis	Hypotension if perforation,Abdominal Guarding,rigidity,Normal bowel sounds	Ascites Fluid analysis for LDH,glucose and protein, xray chest if perforated, Upper GI Endoscopy, Serum Gastrin level,Secretin, Stimulation test, Biopsy
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 TruTest

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 risk of development of leiomyosarcoma.
• History of Pelvic radiations:Pelvic irradiation has been associated with the increased development of the leiomyosarcoma.
• Certain Hereditary condition like Gardner syndrome, Li-Fraumeni syndrome,Werner syndrome and Neurofibromatosis are associated with the development of leiomyosarcoma.

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 multi-disciplinary 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

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