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


==[[Leiomyosarcoma overview|Overview]]==
==[[Leiomyosarcoma overview|Overview]]==
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==[[Leiomyosarcoma historical perspective|Historical Perspective]]==
==[[Leiomyosarcoma historical perspective|Historical Perspective]]==
Soft tissue sarcoma derived their name from the Greek term for a fleshy excrescences.As early as Galen(130-200C.E.),it was suggested that they were a cancerous tumor.Early reports of myxoid liposarcoma by Severinius(1580-1637) and retroperitoneal sarcoma by Morgagni (1682-1771) have been recorded.An Edinburg surgeon, Wardrop( 1782-1869),who studied in Vienna,describes the term soft cancer.Charles Bell ( 1772-1842) ,in his book Surgical Observations, published in 1816, has been credited with the utilization of the term soft tissue sarcoma to differentiate it form carcinoma.In 1804, Abernethy describes the first classification of the sarcoma.In1932 Stout( 1885-1967) published a seminal monograph on the pathology and treatment of sarcoma.Ewing was one of the first to list benign and malignant counterparts of tumors arising in soft tissues.
* 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 pathophysiology|Pathophysiology]]==
==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.
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 causes|Causes]]==
==Causes==
Exact cause of leiomyosarcoma is not clearly evident.
Exact cause of leiomyosarcoma is not clearly evident because of the rarety of disease nature. It may arise denovo or by [[genetic]] instability.


==[[Leiomyosarcoma differential diagnosis|Differentiating Leiomyosarcoma from other Diseases]]: ==
==Differentiating Leiomyosarcoma from other Diseases: ==
Leiomyosarcoma must be differentiated from Peptic ulcer, Stomach cancer, Uterine cancer.
'''Leiomyosarcoma''' must be differentiated from special subtypes of leiomyoma, othter [[soft tissue]] [[sarcoma]], perivascular epitheloid cell neoplasm ([[PEComa]]).


==[[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
[[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]]: ==
==Risk Factors: ==
Clinical data has suggested that the development of leiomyosarcoma is related to [[radiation exposure]]. Other causes are not known for certain.
Clinical data has suggested that the development of leiomyosarcoma is related to [[radiation exposure]]. Other [[risk factors]] include use of [[tamoxifen]], [[immunodeficiency]], viral [[infection]].


==[[Leiomyosarcoma natural history|Natural History, Complications and Prognosis]]==
==Natural History, Complications and Prognosis==
Leiomyosarcoma most commonly metastasizes to the lungs, liver, abdomen, pelvis, and pelvic or paraaortic 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.Overall survival rate ranges from 15% to 25% with a median survival of only 10 months.
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==
==Diagnosis==
The majority of patients with leiomyosarcoma remain asymptomatic for decades. General symptoms associated with cancer may occur including fatigue, fever, weight loss, a general feeling of ill health (malaise), and nausea and vomiting.Patients with Leiomyosarcoma generally appear weak.Physical examination findings depends upon the site of the involvement,size and metastasis.Physical Examination findings may include weight loss, abdominal mass, Distension,Ascites, rectal bleeding and vaginal bleeding.
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]].  
 
There are no specific laboratory findings associated with Leiomyosarcoma. However, we may find elevated levels of CA 125 and LDH( isozymes) in women with uterine sarcoma.However,usefulness of these markers are still being investigated.
 
CT scans are often used to diagnose [[leiomyosarcoma]]. It can confirm the location of the sarcoma and show the organs nearby. These are helpful for determining the stage of the cancer and in determining whether surgery is a good treatment option. CT scans can also be used to guide biopsy and a biopsy sample is usually removed and looked at under a microscope.


[[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.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. Before treatment starts, ask your health care team about possible side effects and how treatment may change your normal activities. 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.It has generally noted that only 40% cases responds to the chemotherapeutic regimens.
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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Patient Information

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

Case Studies

Case #1

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Risk calculators and risk factors for Leiomyosarcoma

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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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