Liposarcoma overview: Difference between revisions

Jump to navigation Jump to search
No edit summary
(Mahshid)
 
(23 intermediate revisions by 5 users not shown)
Line 5: Line 5:
==Overview==
==Overview==


'''Liposarcoma''' is a [[malignant]] [[tumor]] that arises in [[Adipose tissue|fat cells]] in deep soft tissue, such as that inside the thigh or in the [[retroperitoneum]].  
Liposarcoma is a uncommon cancer of connective tissues. Liposarcoma is a [[malignant tumor]] that arises in [[Adipose tissue|fat cells]] in deep soft tissue, such as that inside the thigh or in the [[retroperitoneum]].  


They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor.  
They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor.


== Historical Perspective ==
==Historical Perspective==
Liposarcoma was first described by Dr. Rudolph Virchow, a German pathologist, in 1857. Virchow reported a "myxoma lipomatoides malignum", highlighting the malignant nature of the tumor. Between 1954 and 1979, several authors reported cases of liposarcoma and suggested that liposarcoma should be classified according to histopathological analysis into well-differentiated, myxoid, and dedifferentiated subtypes.


== Classification ==
==Classification==
Liposarcoma may be classified into well-differentiated, de-differentiated, myxoid, round cell, or pleomorphic liposarcoma.


== Pathophysiology ==
==Pathophysiology==


== Causes ==
The pathogenesis of liposarcoma depends on the histological subtype. The role of proto-oncogenes has been implicated in the development of well-differentiated liposarcoma


== Differentiating Liposarcoma from other Diseases ==
==Causes==
Liposarcoma must be differentiated from other diseases that cause a painless, growing [[mass]] such as spindle cell [[lipoma]], [[neurofibroma]], [[dermatofibrosarcoma]] protuberans, and malignant peripheral nerve sheath tumor.
There are no established causes for liposarcoma.


== Epidemiology and Demographics ==
==Differential Diagnosis==
Liposarcoma must be differentiated from other diseases that cause a painless, [[Mass|growing mass]] such as [[Lipoma|spindle cell lipoma]], [[neurofibroma]], [[dermatofibrosarcoma protuberans]], and [[malignant peripheral nerve sheath tumor]].


== Risk Factors ==
==Epidemiology and Demographics==
Liposarcoma is the second most common type of all soft tissue sarcomas in adults. The annual incidence is 0.25 cases per 100,000 individuals. Men are slightly more affected with liposarcoma than women. It is most frequent in middle-aged and older adults.


== Natural History, Complications and Prognosis ==
==Risk Factors==
Liposarcoma may have different clinical presentations depending on the location of the [[mass]], being the lower [[extremities]] the most common area involved.  [[Metastasis]] is the most important aspect to assess in the [[prognosis]] of the disease, but the mass may compress adjacent structures causing different clinical manifestations.  The prognosis will depend on the [[histopathological]] subtype, being the well differentiated liposarcoma the subtype with the best [[prognosis]], and the pleomorphic liposarcoma with the worst [[prognosis]].
Common risk factors in the development of liposarcoma include chemical carcinogens, [[radiation]], [[immunodeficiency]], [[genetic disorders]], and [[Viral infection|viral infections]].


== Diagnosis ==
==Natural History, Complications and Prognosis==
Common complication of liposarcoma include [[metastasis]].  Mass may compress adjacent structures causing different clinical manifestations. [[Metastasis]] is the most important aspect to assess in the [[prognosis]] of liposarcoma. The prognosis will depend on the [[histopathological]] subtype, being the well differentiated liposarcoma the subtype with the best [[prognosis]], and the pleomorphic liposarcoma with the worst [[prognosis]].


=== Staging ===
==Diagnosis==
The staging for liposarcoma is based on the TNM classification, depending on the primary tumor, [[lymph node]] involvement, [[metastasis]] and the [[histological]] grade of the tumor.
===Staging===
The staging of liposarcoma is based on the TNM classification, which includes primary tumor spread, lymph node involvement, and presence of metastasis.
===History and Symptoms===
Liposarcoma usually presents as a  painless [[mass]]. The most common location of a liposarcoma is the lower [[extremities]].  Patients with [[retroperitoneal]] liposarcoma remain [[asymptomatic]] until the mass invades adjacent structures, which may cause [[pain]] or obstructive symptoms.
===Physical Examination===
Physical examination findings of liposarcoma depend on the location of the tumor.  Since most liposarcomas are located in the lower extremities, liposarcoma is commonly associated with the findings of palpable firm nontender mass in one lower extremity.  Physical examination of retroperitoneal liposarcomas is usually unremarkable, but other pertinent findings on physical examination of [[retroperitoneal]] liposarcomas include palpation of an [[abdominal mass]], [[abdominal distension]], and [[tenderness]].
===Laboratory Findings===
There are no specific laboratory tests for the diagnosis of liposarcoma. The pertinent laboratory findings of liposarcoma include [[anemia]] and [[BUN|elevated BUN]] due to GI bleeding and elevated [[creatinine]] among patients with [[obstructive nephropathy]].
===Biopsy===
The definitive diagnosis of liposarcoma is made by biopsy, which also provides histopathological classification of the liposarcoma's subtype.
===CT===
CT imaging is crucial for the diagnosis of liposarcoma. CT scan may detect regions of lipid and non-lipid components that correlate with histological subtypes of liposarcoma. CT imaging may demonstrate the size, location, and depth of a liposarcoma, as well as [[lymph node]] involvement and distant [[metastasis]]. CT findings may be correlated with the [[histopathological]] subtype.
===MRI===
MRI is the optimal imaging test for the diagnosis of liposarcoma.  MRI findings for a well-differentiated liposarcoma include a mass with at least 75% of [[adipose]] content with thin irregular [[septa]].  MRI also evaluates the size, location, and depth of the mass.  MRI findings help to differentiate between the different subtypes of liposarcoma and help to assess distant [[metastasis]].
===Other Imaging Findings===
[[Positron emission tomography]] (PET) scan is useful in cases where the liposarcoma is firm, deep, and has a size greater than 3 cm.<ref name=NCCN>{{cite web | title = NCCN Guidelines for Patients - Soft Tissue Sarcoma | url =http://www.nccn.org/patients/guidelines/sarcoma/index.html#2 }}</ref>  PET scan may also help evaluate the grade and prognosis of the tumor,as well as its shrinkage following chemotherapy.
===Other Diagnostic Studies===
[[Li-Fraumeni syndrome]] is associated with liposarcoma. Mutations in the ''TP53'' gene that encodes the tumor suppressor gene p53 cause Li-Fraumeni syndrome.<ref name=NCCN>{{cite web | title = NCCN Guidelines for Patients - Soft Tissue Sarcoma | url =http://www.nccn.org/patients/guidelines/sarcoma/index.html#2 }}</ref>


=== History and Symptoms ===
==Treatment==
Liposarcomas usually present as a growing [[mass]], not associated with [[pain]] and commonly located in the lower [[extremities]].  [[Retroperitoneal]] liposarcomas remain [[asymptomatic]] until they affect adjacent structures, causing [[pain]] or obstructive symptoms.
===Medical Therapy===
 
Medical therapy for liposarcoma includes [[Chemotherapy]], chemoradiation, [[immunotherapy]] and targeted therapy.
=== Physical Examination ===
===Surgery===
The physical examination findings will depend on the location of the liposarcoma, being the most common location the lower extremities. The main finding is a painless, slow growing, firm [[mass]] in one of the lower [[extremities]].  [[Retroperitoneal]] liposarcomas would have a late presentation and the physical examination findings include abdominal tenderness, [[abdominal distention]] and [[weight loss]].
The predominant therapy for liposarcoma is surgical resection. [[Chemotherapy|Adjunctive chemotherapy]] and [[radiation]] may be required.
 
=== Laboratory Findings ===
 
=== CT ===
CT imaging is one of the most important tests for the diagnosis of liposarcoma. CT should assess the size, location and depth of the liposarcoma, as well as the [[lymph node]] involvement and distant [[metastasis]].  CT findings can be correlated with the [[histopathological]] subtype.
 
=== MRI ===
MRI is the imaging test of choice for the diagnosis of liposarcoma and should assess size, location and depth of the mass.  MRI findings help to differentiate between the different subtypes of liposarcoma and help to assess distant [[metastasis]].
 
=== Other Imaging Findings ===
 
=== Other Diagnostic Studies ===
 
== Treatment ==
 
=== Medical Therapy ===
 
=== Surgery ===
 
=== Cost-Effectiveness of Therapy ===
 
=== Future or Investigational Therapies ===




Line 67: Line 67:
[[Category:Disease]]
[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Surgery]]
[[Category:Surgery]]


{{WikiDoc Help Menu}}
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]

Latest revision as of 02:17, 27 November 2017

Liposarcoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Liposarcoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Liposarcoma overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Liposarcoma overview

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Liposarcoma overview

CDC on Liposarcoma overview

Liposarcoma overview in the news

Blogs on Liposarcoma overview

Directions to Hospitals Treating Liposarcoma

Risk calculators and risk factors for Liposarcoma overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alejandro Lemor, M.D. [2]; Ammu Susheela, M.D. [3]

Overview

Liposarcoma is a uncommon cancer of connective tissues. Liposarcoma is a malignant tumor that arises in fat cells in deep soft tissue, such as that inside the thigh or in the retroperitoneum.

They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor.

Historical Perspective

Liposarcoma was first described by Dr. Rudolph Virchow, a German pathologist, in 1857. Virchow reported a "myxoma lipomatoides malignum", highlighting the malignant nature of the tumor. Between 1954 and 1979, several authors reported cases of liposarcoma and suggested that liposarcoma should be classified according to histopathological analysis into well-differentiated, myxoid, and dedifferentiated subtypes.

Classification

Liposarcoma may be classified into well-differentiated, de-differentiated, myxoid, round cell, or pleomorphic liposarcoma.

Pathophysiology

The pathogenesis of liposarcoma depends on the histological subtype. The role of proto-oncogenes has been implicated in the development of well-differentiated liposarcoma

Causes

There are no established causes for liposarcoma.

Differential Diagnosis

Liposarcoma must be differentiated from other diseases that cause a painless, growing mass such as spindle cell lipoma, neurofibroma, dermatofibrosarcoma protuberans, and malignant peripheral nerve sheath tumor.

Epidemiology and Demographics

Liposarcoma is the second most common type of all soft tissue sarcomas in adults. The annual incidence is 0.25 cases per 100,000 individuals. Men are slightly more affected with liposarcoma than women. It is most frequent in middle-aged and older adults.

Risk Factors

Common risk factors in the development of liposarcoma include chemical carcinogens, radiation, immunodeficiency, genetic disorders, and viral infections.

Natural History, Complications and Prognosis

Common complication of liposarcoma include metastasis. Mass may compress adjacent structures causing different clinical manifestations. Metastasis is the most important aspect to assess in the prognosis of liposarcoma. The prognosis will depend on the histopathological subtype, being the well differentiated liposarcoma the subtype with the best prognosis, and the pleomorphic liposarcoma with the worst prognosis.

Diagnosis

Staging

The staging of liposarcoma is based on the TNM classification, which includes primary tumor spread, lymph node involvement, and presence of metastasis.

History and Symptoms

Liposarcoma usually presents as a painless mass. The most common location of a liposarcoma is the lower extremities. Patients with retroperitoneal liposarcoma remain asymptomatic until the mass invades adjacent structures, which may cause pain or obstructive symptoms.

Physical Examination

Physical examination findings of liposarcoma depend on the location of the tumor. Since most liposarcomas are located in the lower extremities, liposarcoma is commonly associated with the findings of palpable firm nontender mass in one lower extremity. Physical examination of retroperitoneal liposarcomas is usually unremarkable, but other pertinent findings on physical examination of retroperitoneal liposarcomas include palpation of an abdominal mass, abdominal distension, and tenderness.

Laboratory Findings

There are no specific laboratory tests for the diagnosis of liposarcoma. The pertinent laboratory findings of liposarcoma include anemia and elevated BUN due to GI bleeding and elevated creatinine among patients with obstructive nephropathy.

Biopsy

The definitive diagnosis of liposarcoma is made by biopsy, which also provides histopathological classification of the liposarcoma's subtype.

CT

CT imaging is crucial for the diagnosis of liposarcoma. CT scan may detect regions of lipid and non-lipid components that correlate with histological subtypes of liposarcoma. CT imaging may demonstrate the size, location, and depth of a liposarcoma, as well as lymph node involvement and distant metastasis. CT findings may be correlated with the histopathological subtype.

MRI

MRI is the optimal imaging test for the diagnosis of liposarcoma. MRI findings for a well-differentiated liposarcoma include a mass with at least 75% of adipose content with thin irregular septa. MRI also evaluates the size, location, and depth of the mass. MRI findings help to differentiate between the different subtypes of liposarcoma and help to assess distant metastasis.

Other Imaging Findings

Positron emission tomography (PET) scan is useful in cases where the liposarcoma is firm, deep, and has a size greater than 3 cm.[1] PET scan may also help evaluate the grade and prognosis of the tumor,as well as its shrinkage following chemotherapy.

Other Diagnostic Studies

Li-Fraumeni syndrome is associated with liposarcoma. Mutations in the TP53 gene that encodes the tumor suppressor gene p53 cause Li-Fraumeni syndrome.[1]

Treatment

Medical Therapy

Medical therapy for liposarcoma includes Chemotherapy, chemoradiation, immunotherapy and targeted therapy.

Surgery

The predominant therapy for liposarcoma is surgical resection. Adjunctive chemotherapy and radiation may be required.


References

  1. 1.0 1.1 "NCCN Guidelines for Patients - Soft Tissue Sarcoma".


Template:WikiDoc Sources