Leiomyosarcoma

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

Overview

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]

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.

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

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]

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.

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

Survival Rate of Leiomyosarcoma by staging:

Leiomyosarcoma treatment consist of multidisciplinary approach and is best carried out at the specialized hospital setting.