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{{Leiomyosarcoma}}
{{Leiomyosarcoma}}
* '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''.
{{CMG}} {{AE}}'''{{Nnasiri}


'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''.
==[[Leiomyosarcoma overview|Overview]]==
'''Leiomyosarcoma''' is a type of [[sarcoma]] which is a [[neoplasm]] of [[smooth muscle]]. (When [[benign]], it is called a [[leiomyoma]].) Smooth muscle cells make up the [[involuntary muscles]], which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin. Leiomyosarcoma is a very rare [[cancer]]. It makes up 7% of soft tissue sarcomas.


{{CMG}}
==[[Leiomyosarcoma historical perspective|Historical Perspective]]==
* Soft tissue sarcoma derived their name from the Greek term for a fleshy lump.
* Around 460 B.C, Hippocrates recognized soft tissue tumors in the arm and thigh in older people and recommended the removal by knife as therapy.
* Around 200 C.E., a greek physician named Galen suggested that these lumps were cancerous tumors.
* In 1809, an Edinburg surgeon, Wardrop describes the first illustration of amputation and used the term "soft cancer".
* In 1816, Charles Bell, used the term soft tissue [[sarcoma]] to differentiate it form [[carcinoma]].
* In 1804, Abernethy describes the first classification of the [[sarcoma]].
* In 1932, Stout published a litreature on the pathology and treatment of sarcoma.
* In 1982, Rosenberg evaluated limb-sparing surgery with adjuvant radiotherapy compared with major amputation.


==[[Leiomyosarcoma overview|Overview]]==
==Pathophysiology==
The [[pathogenesis]] of leimyosarcoma is characterized by malignant smooth muscle [[neoplasm]] that can appear in any site in the body but most commonly found in the [[uterus]], [[small intestine]] and retro [[peritoneum]].


==[[Leiomyosarcoma historical perspective|Historical Perspective]]==
==Causes==
Exact cause of leiomyosarcoma is not clearly evident because of the rarety of disease nature. It may arise denovo or by [[genetic]] instability.


==[[Leiomyosarcoma pathophysiology|Pathophysiology]]==
==Differentiating Leiomyosarcoma from other Diseases: ==
==[[Leiomyosarcoma causes|Causes]]==
'''Leiomyosarcoma''' must be differentiated from special subtypes of leiomyoma, othter [[soft tissue]] [[sarcoma]], perivascular epitheloid cell neoplasm ([[PEComa]]).
==[[Leiomyosarcoma differential diagnosis|Differentiating Leiomyosarcoma from other Diseases]]: ==
{| class="wikitable"
|'''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 epidemiology and demographics|Epidemiology and Demographics]]:==
==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.<ref name="pmid15047237">Brooks SE, Zhan M, Cote T, Baquet CR (2004) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=15047237 Surveillance, epidemiology, and end results analysis of 2677 cases of uterine sarcoma 1989-1999.] ''Gynecol Oncol'' 93 (1):204-8. [http://dx.doi.org/10.1016/j.ygyno.2003.12.029 DOI:10.1016/j.ygyno.2003.12.029] PMID: [https://pubmed.gov/15047237 15047237]</ref>
[[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, it is twice more common in African-American women, median age of patients is between 40 to 52 years old.  


==[[Leiomyosarcoma risk factors|Risk Factors]]: <small>Following Risk Fcators found to be associated with the development of the Uterine leiomyosarcoma</small>==
==Risk Factors: ==
* Age: Leiomyosarcoma can be seen at 20 years of age but mean age at diagnosis is 50-60 years old.
Clinical data has suggested that the development of leiomyosarcoma is related to [[radiation exposure]]. Other [[risk factors]] include use of [[tamoxifen]], [[immunodeficiency]], viral [[infection]].
* Race:Black women have a higher incidence of development of the uterine leiomyosarcoma as compared to the white women.<ref name="pmid120503512">Wysowski DK, Honig SF, Beitz J (2002) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=12050351 Uterine sarcoma associated with tamoxifen use.] ''N Engl J Med'' 346 (23):1832-3. [http://dx.doi.org/10.1056/NEJM200206063462319 DOI:10.1056/NEJM200206063462319] PMID: [https://pubmed.gov/12050351 12050351]</ref>
* 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.<ref name="pmid11336777">Mourits MJ, De Vries EG, Willemse PH, Ten Hoor KA, Hollema H, Van der Zee AG (2001) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11336777 Tamoxifen treatment and gynecologic side effects: a review.] ''Obstet Gynecol'' 97 (5 Pt 2):855-66. PMID: [https://pubmed.gov/11336777 11336777]</ref>
* History of Pelvic radiations:Pelvic irradiation has been associated with the increased development of the leiomyosarcoma.<ref name="pmid15168177">Fang Z, Matsumoto S, Ae K, Kawaguchi N, Yoshikawa H, Ueda T et al. (2004) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=15168177 Postradiation soft tissue sarcoma: a multiinstitutional analysis of 14 cases in Japan.] ''J Orthop Sci'' 9 (3):242-6. [http://dx.doi.org/10.1007/s00776-004-0768-5 DOI:10.1007/s00776-004-0768-5] PMID: [https://pubmed.gov/15168177 15168177]</ref>
* 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.<ref name="pmid19351917">Yu CL, Tucker MA, Abramson DH, Furukawa K, Seddon JM, Stovall M et al. (2009) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=19351917 Cause-specific mortality in long-term survivors of retinoblastoma.] ''J Natl Cancer Inst'' 101 (8):581-91. [http://dx.doi.org/10.1093/jnci/djp046 DOI:10.1093/jnci/djp046] PMID: [https://pubmed.gov/19351917 19351917]</ref>


==[[Leiomyosarcoma natural history|Natural History, Complications and Prognosis]]==
==Natural History, Complications and Prognosis==
* The majority of patients with leiomyosarcoma remain asymptomatic for decades.
Leiomyosarcoma most commonly metastasizes to the lungs, [[abdomen]], [[pelvis]], and [[lymph nodes]]. [[Bone]] and [[brain]] [[metastases]] are less common sites of involvement. Depending on the extent of the [[tumor]] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor. Stage of tumor is the most important prognostic factor.
* 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:'''
{| class="wikitable"
|'''Stage'''
|'''Percentage'''
|-
|stage 1
|60
|-
|stage 2
|35
|-
|stage 3
|28
|-
|stage 4
|15
|}


==Diagnosis==
==Diagnosis==
The majority of patients with leiomyosarcoma remain asymptomatic for decades. General [[symptoms]] associated with cancer may occur including [[fatigue]], [[fever]], weight loss, and [[nausea]] and [[vomiting]]. Irregular [[vaginal bleeding]] is most common symptom in patients with leiomyosarcoma, other symptoms include feeling pressure in [[pelvic]] or [[abdomen]], painless growing [[mass]] in [[abdominal cavity]], [[vaginal discharge]].
[[Leiomyosarcoma history and symptoms|History and Symptoms]] | [[Leiomyosarcoma physical examination|Physical Examination]] | [[Leiomyosarcoma laboratory tests|Laboratory Findings]] | [[Leiomyosarcoma chest x ray|Chest X Ray]] | [[Leiomyosarcoma CT|CT]] | [[Leiomyosarcoma MRI|MRI]] | [[Leiomyosarcoma ultrasound|Ultrasound]] | [[Leiomyosarcoma other imaging findings|Other Imaging Findings]] | [[Leiomyosarcoma other diagnostic studies|Other Diagnostic Studies]]
[[Leiomyosarcoma history and symptoms|History and Symptoms]] | [[Leiomyosarcoma physical examination|Physical Examination]] | [[Leiomyosarcoma laboratory tests|Laboratory Findings]] | [[Leiomyosarcoma chest x ray|Chest X Ray]] | [[Leiomyosarcoma CT|CT]] | [[Leiomyosarcoma MRI|MRI]] | [[Leiomyosarcoma ultrasound|Ultrasound]] | [[Leiomyosarcoma other imaging findings|Other Imaging Findings]] | [[Leiomyosarcoma other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
==Treatment==
Leiomyosarcoma treatment consist of multidisciplinary approach and is best carried out at the specialized hospital setting.
Leiomyosarcoma treatment consist of multidisciplinary approach and is best carried out at the specialized hospital setting. Patients with leiomyosarcoma have many treatment options. The selection depends on the stage of the [[tumor]]. The options are [[surgery]], [[radiation therapy]], [[chemotherapy]], or a combination of these methods. Because cancer treatments often damage healthy cells and tissues, side effects are common. Side effects may not be the same for each person, and they may change from one treatment session to the next. Non surgical treatment options have shown only limited benefit in the treatment of the leiomyosarcoma and are generally considered to be less responsive of [[chemotherapy]] and [[radiotherapy]].  


[[Leiomyosarcoma medical therapy|Medical Therapy]] | [[Leiomyosarcoma surgery|Surgery]] | [[Leiomyosarcoma secondary prevention|Secondary Prevention]] | [[Leiomyosarcoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Leiomyosarcoma future or investigational therapies|Future or Investigational Therapies]]
[[Leiomyosarcoma medical therapy|Medical Therapy]] | [[Leiomyosarcoma surgery|Surgery]] | [[Leiomyosarcoma secondary prevention|Secondary Prevention]] | [[Leiomyosarcoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Leiomyosarcoma future or investigational therapies|Future or Investigational Therapies]]

Latest revision as of 11:02, 18 July 2020

Leiomyosarcoma Microchapters

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Overview

Historical Perspective

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Pathophysiology

Causes

Differentiating Leiomyosarcoma from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

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History and Symptoms

Physical Examination

Laboratory Findings

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CT

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: '{{Nnasiri}

Overview

Leiomyosarcoma is a type of sarcoma which is a neoplasm of smooth muscle. (When benign, it is called a leiomyoma.) Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin. Leiomyosarcoma is a very rare cancer. It makes up 7% of soft tissue sarcomas.

Historical Perspective

  • Soft tissue sarcoma derived their name from the Greek term for a fleshy lump.
  • Around 460 B.C, Hippocrates recognized soft tissue tumors in the arm and thigh in older people and recommended the removal by knife as therapy.
  • Around 200 C.E., a greek physician named Galen suggested that these lumps were cancerous tumors.
  • In 1809, an Edinburg surgeon, Wardrop describes the first illustration of amputation and used the term "soft cancer".
  • In 1816, Charles Bell, used the term soft tissue sarcoma to differentiate it form carcinoma.
  • In 1804, Abernethy describes the first classification of the sarcoma.
  • In 1932, Stout published a litreature on the pathology and treatment of sarcoma.
  • In 1982, Rosenberg evaluated limb-sparing surgery with adjuvant radiotherapy compared with major amputation.

Pathophysiology

The pathogenesis of leimyosarcoma is characterized by malignant smooth muscle neoplasm that can appear in any site in the body but most commonly found in the uterus, small intestine and retro peritoneum.

Causes

Exact cause of leiomyosarcoma is not clearly evident because of the rarety of disease nature. It may arise denovo or by genetic instability.

Differentiating Leiomyosarcoma from other Diseases:

Leiomyosarcoma must be differentiated from special subtypes of leiomyoma, othter soft tissue sarcoma, perivascular epitheloid cell neoplasm (PEComa).

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, it is twice more common in African-American women, median age of patients is between 40 to 52 years old.

Risk Factors:

Clinical data has suggested that the development of leiomyosarcoma is related to radiation exposure. Other risk factors include use of tamoxifen, immunodeficiency, viral infection.

Natural History, Complications and Prognosis

Leiomyosarcoma most commonly metastasizes to the lungs, abdomen, pelvis, and lymph nodes. Bone and brain metastases are less common sites of involvement. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor. Stage of tumor is the most important prognostic factor.

Diagnosis

The majority of patients with leiomyosarcoma remain asymptomatic for decades. General symptoms associated with cancer may occur including fatigue, fever, weight loss, and nausea and vomiting. Irregular vaginal bleeding is most common symptom in patients with leiomyosarcoma, other symptoms include feeling pressure in pelvic or abdomen, painless growing mass in abdominal cavity, vaginal discharge.

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. Patients with leiomyosarcoma have many treatment options. The selection depends on the stage of the tumor. The options are surgery, radiation therapy, chemotherapy, or a combination of these methods. Because cancer treatments often damage healthy cells and tissues, side effects are common. Side effects may not be the same for each person, and they may change from one treatment session to the next. Non surgical treatment options have shown only limited benefit in the treatment of the leiomyosarcoma and are generally considered to be less responsive of chemotherapy and radiotherapy.

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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