Synovial sarcoma: Difference between revisions

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Revision as of 19:54, 19 May 2016

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Malignant synovioma

Overview

Synovial sarcoma (also known as malignant synovioma) is a very rare form of soft tissue sarcoma, which usually occurs near to the joints in upper and lower extremities.

Historical Perspective

Synovial sarcoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].

Classification

Synovial sarcoma may be classified according to histopathological findings into 4 subtypes:

Biphasic
Monophasic fibrous (most common)
Monophasic epithelial
Poorly differentiated

Pathophysiology

The pathogenesis of synovial sarcoma is characterized by the dysregulation of gene expression of SYT-SSX gene.
The most common location for these synovial sarcoma, include:

Knee
Adjacent to large joints
Popliteal fossa

The SYT-SSX fusion gene (chromosome 18) has been associated with the development of synovial sarcoma.
On gross pathology, characteristic findings of synovial sarcoma, include:

Solid often lobulated
Grey-yellow
Pushing border to ill-defined border

On microscopic histopathological analysis, characteristic findings of synovial sarcoma, include:

Non-specific appearances
Well or poorly defined heterogeneous masses
Frequent areas of hemorrhage
Necrosis

Causes

There are no established causes for synovial sarcoma.

Differentiating Synovial Sarcoma from Other Diseases

Synovial sarcoma must be differentiated from other diseases that cause joint pain, mass growth, and limited range of motion, such as:

Malignant fibrous histiocytoma (MFH)-fibrosarcoma
Liposarcoma
Osteosarcoma
Chondrosarcoma

Epidemiology and Demographics

The prevalence of synovial sarcoma remains unknown.
Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma

Age

Synovial sarcoma is more commonly observed among patients aged 15-40 years old.
Synovial sarcoma is more commonly observed among adolescents and young adults.

Gender

Males are more commonly affected with synovial sarcoma than females
The male to female ratio is approximately 1.2 to 1.

Race

There is no racial predilection for synovial sarcoma.

Risk Factors

There are no known risk factors associated with the development of synovial sarcoma.

Natural History, Complications and Prognosis

The majority of patients with synovial sarcoma remain asymptomatic for years.
Early clinical feature includes a soft palpable mass.
If left untreated, patients with synovial sarcoma may progress to develop metastases
Common complications of synovial sarcoma, include:

Cannonball metastases

Prognosis is generally poor, and the median survival rate of patients with synovial sarcoma is approximately 35%

Diagnosis

Diagnostic Criteria

The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18).

Symptoms

Synovial sarcoma is usually asymptomatic.
Symptoms of synovial sarcoma may include the following:

[symptom 1]
[symptom 2]
[symptom 3]
[symptom 4]
[symptom 5]
[symptom 6]

Physical Examination

Patients with synovial sarcoma usually appear [general appearance].
Physical examination may be remarkable for:

[finding 1]
[finding 2]
[finding 3]
[finding 4]
[finding 5]
[finding 6]

Laboratory Findings

There are no specific laboratory findings associated with synovial sarcoma.

Imaging Findings

MRI is the imaging modality of choice for synovial sarcoma.
On ultrasound, characteristic findings of synovial sarcoma, include:

Non-specific
Heterogeneous
Hypoechoic mass

On CT, characteristic findings of synovial sarcoma, include:

Non-specific
Soft tissue mass
Heterogeneous density and enhancement
Calcifications

On MRI, characteristic findings of synovial sarcoma, include:

T1: iso- (slightly hyper-) intense to muscle/ heterogeneous
T2: mostly hyperintense, markedly heterogeneous appearance of synovial cell sarcomas on fluid sensitive sequences result in so called "triple sign" which is due to areas of necrosis and cystic degeneration with very high signal, relatively high signal soft tissue components, and areas of low signal intensity due to dystrophic calcifications and fibrotic bands, due to high tendency of lesions to bleed, there might be areas of fluid-fluid levels known as "bowl of grapes" are seen in up to 10-25% of cases
T1 C + (Gd): enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or peripheral (20%)

Treatment

Medical Therapy

The mainstay of therapy for synovial sarcoma, includes:

Surgery

Surgery is the mainstay of therapy for synovial sarcoma.
Surgical resection in conjunction with chemotherapy or radiation is the most common approach to the treatment of synovial sarcoma.

Prevention

There are no primary preventive measures available for synovial sarcoma.

References

@@ Line 3: / Line 3: @@
 {{CMG}} {{AE}} {{MV}}
-{{SK}} Synonym 1; Synonym 2; Synonym 3
+{{SK}} Malignant synovioma
 ==Overview==
+'''Synovial sarcoma''' (also known as '''malignant synovioma''') is a very rare form of soft tissue sarcoma, which usually occurs near to the joints in upper and lower extremities.
 ==Historical Perspective==
 *Synovial sarcoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
-*In [year], [gene] mutations were first identified in the pathogenesis of synovial sarcoma.
-*In [year], the first [discovery] was developed by [scientist] to treat/diagnose synovial sarcoma.
 ==Classification==
-*Synovial sarcoma may be classified according to [classification method] into [number] subtypes/groups:
+*Synovial sarcoma may be classified according to histopathological findings into 4 subtypes:
-:*[group1]
+:*Biphasic
-:*[group2]
+:*Monophasic fibrous (most common)
-:*[group3]
+:*Monophasic epithelial
-*Other variants of synovial sarcoma include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
+:*Poorly differentiated
 ==Pathophysiology==
-*The pathogenesis of synovial sarcoma is characterized by [feature1], [feature2], and [feature3].
+*The pathogenesis of synovial sarcoma is characterized by the dysregulation of gene expression of SYT-SSX gene.
-*The [gene name] gene/Mutation in [gene name] has been associated with the development of synovial sarcoma, involving the [molecular pathway] pathway.
+*The most common location for these synovial sarcoma, include:
-*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of synovial sarcoma.
+:*Knee
-*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of synovial sarcoma.
+:*Adjacent to large joints
+:*Popliteal fossa
+*The SYT-SSX fusion gene (chromosome 18) has been associated with the development of synovial sarcoma.
+*On gross pathology, characteristic findings of synovial sarcoma, include:
+:*Solid often lobulated
+:*Grey-yellow
+:*Pushing border to ill-defined border
+*On microscopic histopathological analysis, characteristic findings of synovial sarcoma, include:
+:*Non-specific appearances
+:*Well or poorly defined heterogeneous masses
+:*Frequent areas of hemorrhage
+:*Necrosis
 ==Causes==
-* Synovial sarcoma may be caused by either [cause1], [cause2], or [cause3].
-* Synovial sarcoma is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
 * There are no established causes for synovial sarcoma.
-==Differentiating synovial sarcoma from other Diseases==
+==Differentiating Synovial Sarcoma from Other Diseases==
-*Synovial sarcoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
+*Synovial sarcoma must be differentiated from other diseases that cause joint pain, mass growth, and limited range of motion, such as:
-:*[Differential dx1]
+:*Malignant fibrous histiocytoma (MFH)-fibrosarcoma
-:*[Differential dx2]
+:*Liposarcoma
-:*[Differential dx3]
+:*Osteosarcoma
+:*Chondrosarcoma
 ==Epidemiology and Demographics==
-* The prevalence of synovial sarcoma is approximately [number or range] per 100,000 individuals worldwide.
+* The prevalence of synovial sarcoma remains unknown.
-* In [year], the incidence of synovial sarcoma was estimated to be [number or range] cases per 100,000 individuals in [location].
+* Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma
 ===Age===
-*Patients of all age groups may develop synovial sarcoma.
+*Synovial sarcoma is more commonly observed among patients aged 15-40 years old.
+*Synovial sarcoma is more commonly observed among adolescents and young adults.
-*Synovial sarcoma is more commonly observed among patients aged [age range] years old.
-*Synovial sarcoma is more commonly observed among [elderly patients/young patients/children].
 ===Gender===
-*Synovial sarcoma affects men and women equally.
+* Males are more commonly affected with synovial sarcoma than females
+* The male to female ratio is approximately 1.2 to 1.
-*[Gender 1] are more commonly affected with synovial sarcoma than [gender 2].
-* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
 ===Race===
 *There is no racial predilection for synovial sarcoma.
-*Synovial sarcoma usually affects individuals of the [race 1] race.
-*[Race 2] individuals are less likely to develop synovial sarcoma.
 ==Risk Factors==
-*Common risk factors in the development of synovial sarcoma are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
+*There are no known risk factors associated with the development of synovial sarcoma.
 == Natural History, Complications and Prognosis==
-*The majority of patients with synovial sarcoma remain asymptomatic for [duration/years].
+*The majority of patients with synovial sarcoma remain asymptomatic for years.
-*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
+*Early clinical feature includes a soft palpable mass.
-*If left untreated, [#%] of patients with synovial sarcoma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
+*If left untreated, patients with synovial sarcoma may progress to develop metastases
-*Common complications of synovial sarcoma include [complication 1], [complication 2], and [complication 3].
+*Common complications of synovial sarcoma, include:
-*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with synovial sarcoma is approximately [#%].
+:*Cannonball metastases
+:*
+:*
+*Prognosis is generally poor, and the median survival rate of patients with synovial sarcoma is approximately 35%
 == Diagnosis ==
 ===Diagnostic Criteria===
-*The diagnosis of synovial sarcoma is made when at least [number] of the following [number] diagnostic criteria are met:
+* The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18).
-:*[criterion 1]
-:*[criterion 2]
-:*[criterion 3]
-:*[criterion 4]
 === Symptoms ===
 *Synovial sarcoma is usually asymptomatic.
@@ Line 99: / Line 99: @@
 === Laboratory Findings ===
 *There are no specific laboratory findings associated with synovial sarcoma.
-*A  [positive/negative] [test name] is diagnostic of synovial sarcoma.
-*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of synovial sarcoma.
-*Other laboratory findings consistent with the diagnosis of synovial sarcoma include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
 ===Imaging Findings===
-*There are no [imaging study] findings associated with synovial sarcoma.
+*MRI is the imaging modality of choice for synovial sarcoma.
+*On ultrasound, characteristic findings of synovial sarcoma, include:
-*[Imaging study 1] is the imaging modality of choice for synovial sarcoma.
+:*Non-specific
-*On [imaging study 1], synovial sarcoma is characterized by [finding 1], [finding 2], and [finding 3].
+:*Heterogeneous
-*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
+:*Hypoechoic mass
+*On CT, characteristic findings of synovial sarcoma, include:
-=== Other Diagnostic Studies ===
+:*Non-specific
-*Synovial sarcoma may also be diagnosed using [diagnostic study name].
+:*Soft tissue mass
-*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
+:*Heterogeneous density and enhancement
+:*Calcifications
+*On MRI, characteristic findings of synovial sarcoma, include:
+:*T1: iso- (slightly hyper-) intense to muscle/ heterogeneous
+:*T2: mostly hyperintense, markedly heterogeneous appearance of synovial cell sarcomas on fluid sensitive sequences result in so called "triple sign" which is due to areas of necrosis and cystic degeneration with very high signal, relatively high signal soft tissue components, and areas of low signal intensity due to dystrophic calcifications and fibrotic bands, due to high tendency of lesions to bleed, there might be areas of fluid-fluid levels known as "bowl of grapes" are seen in up to 10-25% of cases
+:*T1 C + (Gd): enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or peripheral (20%)
 == Treatment ==
 === Medical Therapy ===
-*There is no treatment for synovial sarcoma; the mainstay of therapy is supportive care.
+*The mainstay of therapy for synovial sarcoma, includes:
+:*
-*The mainstay of therapy for synovial sarcoma is [medical therapy 1] and [medical therapy 2].
+:*
-*[Medical therapy 1] acts by [mechanism of action1].
+:*
-*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
 === Surgery ===
 *Surgery is the mainstay of therapy for synovial sarcoma.
-*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of synovial sarcoma.
+*Surgical resection in conjunction with chemotherapy or radiation is the most common approach to the treatment of synovial sarcoma.
-*[Surgical procedure] can only be performed for patients with [disease stage] synovial sarcoma.
 === Prevention ===
 *There are no primary preventive measures available for synovial sarcoma.
-*Effective measures for the primary prevention of synovial sarcoma include [measure1], [measure2], and [measure3].
-*Once diagnosed and successfully treated, patients with synovial sarcoma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
 ==References==
 {{Reflist|2}}
 [[Category: Oncology]]