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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]]==
'''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.


==[[Leiomyosarcoma historical perspective|Historical Perspective]]==
==[[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 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]].
* 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.
'''Uterine Leiomyosarcoma:'''


==[[Leiomyosarcoma causes|Causes]]==
==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 differential diagnosis|Differentiating Leiomyosarcoma from other Diseases]]==
==Differentiating Leiomyosarcoma from other Diseases: ==
'''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]]: <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.
* Long term use of the tamoxifen: Prolonged use of the tamoxifen esp more than five years has been associated with the increased use of development of leiomyosarcoma
* History of Pelvic radiations:Pelvic irradiation has been associated with the increased development of the leiomyosarcoma.
* Certain Hereditary condition


==[[Leiomyosarcoma natural history|Natural History, Complications and Prognosis]]==
==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==
==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. 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]]


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[[Category:Medicine]]
[[Category:Medicine]]
[[Category:Gynecology]]
[[Category:Gynecology]]
<references />

Latest revision as of 11:02, 18 July 2020

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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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