Hemangiopericytoma: Difference between revisions

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{{CMG}} {{AE}} {{MV}}
{{CMG}} {{AE}} {{MV}}
   
   
{{SK}} HPC;  
{{SK}} HPC; Solitary fibrous tumor
   
   
==Overview==
==Overview==
 
'''Hemangiopericytoma''' (HPC) is a type of soft tissue vascular [[sarcoma]] that originates in the pericytes in the walls of capillaries. Typically,  hemangiopericytomas have large vessels especially located at its periphery, and commonly involve the [[lower limbs]] (35% of cases), especially the thigh, pelvis, and [[retroperitoneum]] (25%). Hemangiopericytoma was first described by Arthur Purdy Stout and Margaret Ransone Murray, two American pathologists, in 1942. mThe NF2 gene mutation has been associated with the development of hemangiopericytoma.
'''Hemangiopericytoma''' (HPC) is a type of soft tissue vascular sarcoma that originates in the pericytes in the walls of capillaries.  
If left untreated, the majority of patients with hemangiopericytoma may progress to develop bone invasion.  On MRI is the imaging modality of choice for hemangiopericytoma. On MRI, characteristic findings of hemangiopericytoma, include: solitary fibrous tumor, demonstrates a deep soft-tissue mass, and heterogenous high signal intensity on coronal STIR imaging.  Surgery is the mainstay of therapy for hemangiopericytoma. Arterial preoperative embolisation is highly recommended to avoid bleeding among patients with hemangiopericytoma.<ref name="pmid22566753">{{cite journal |vauthors=Penel N, Amela EY, Decanter G, Robin YM, Marec-Berard P |title=Solitary fibrous tumors and so-called hemangiopericytoma |journal=Sarcoma |volume=2012 |issue= |pages=690251 |date=2012 |pmid=22566753 |pmc=3337510 |doi=10.1155/2012/690251 |url=}}</ref>


==Historical Perspective==
==Historical Perspective==
*Hemangiopericytoma was first described by Arthur Purdy Stout and Margaret Ransone Murray, two American pathologists, in 1942.<ref name="radiopedia> Hemangiopericytoma. Radiopedia. http://radiopaedia.org/articles/haemangiopericytoma-1 Accessed on April 19, 2016</ref>
*Hemangiopericytoma was first described by Arthur Purdy Stout and Margaret Ransone Murray, two American pathologists, in 1942.


==Classification==
==Classification==
*Hemangiopericytoma may be classified according to [classification method] into [number] subtypes/groups:
*Hemangiopericytoma may be classified into 4 groups:
:*[group1]
 
:*[group2]
:*Hemangiopericytoma of the spleen
:*[group3]
:*Dural hemangiopericytomas
*Other variants of hemangiopericytoma include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
:*Myopericytoma
:*Infantile myofibromatosis
:*Sinonasal hemangiopericytomas
*Other associated conditions, include: Doege-Potter syndrome
 
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of hemangiopericytoma is characterized by [feature1], [feature2], and [feature3].
*Hemangiopericytomas arise from [[pericytes]], which are normally involved in the formation of connective tissue around small vessels walls and are also associated in flow regulation.<ref name="pmid1203874">{{cite journal |vauthors=McMaster MJ, Soule EH, Ivins JC |title=Hemangiopericytoma. A clinicopathologic study and long-term followup of 60 patients |journal=Cancer |volume=36 |issue=6 |pages=2232–44 |date=December 1975 |pmid=1203874 |doi=10.1002/cncr.2820360942 |url=}}</ref>
*The [gene name] gene/Mutation in [gene name] has been associated with the development of hemangiopericytoma, involving the [molecular pathway] pathway.
*The NF2 gene mutation has been associated with the development of hemangiopericytoma.  
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of hemangiopericytoma.
*On gross pathology, characteristic findings of hemangiopericytoma, include:
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of hemangiopericytoma.
 
:*Large mass
:*Poorly demarcated margins
*On microscopic histopathological analysis, characteristic findings of hemangiopericytoma, include:
 
:*Hypervascular lesion
:*Abundant thin-walled branching small vessels of variable size.
:*Spindle or ovoid shaped cells in nests or sheets
:*Cells may "onion-skin" around thin blood vessels
:*Hemangiopericytoma-like area (staghorn vessels)
:*Keloid-like collagen bundles (key feature)
 
==Causes==
==Causes==
* Hemangiopericytoma may be caused by either [cause1], [cause2], or [cause3].
* Hemangiopericytoma is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
* There are no established causes for hemangiopericytoma.
* There are no established causes for hemangiopericytoma.
 
==Differentiating Hemangiopericytoma From Other Diseases==
==Differentiating Hemangiopericytoma From Other Diseases==
*Hemangiopericytoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
*Hemangiopericytoma must be differentiated from other diseases that cause a slow growing painless mass, such as:
:*[Differential dx1]
 
:*[Differential dx2]
:*[[Liposarcoma]]  
:*[Differential dx3]
:*Sinovial sarcoma
:*[[Angiosarcoma]]


==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of hemangiopericytoma is approximately [number or range] per 100,000 individuals worldwide.
*Hemangiopericytoma represents only about 1-2% of all soft-tissue tumors.
* In [year], the incidence of hemangiopericytoma was estimated to be [number or range] cases per 100,000 individuals in [location].
   
   
===Age===
===Age===
*Patients of all age groups may develop hemangiopericytoma.
*The median age at diagnosis is 45 years.
*Hemangiopericytoma is more commonly observed among patients aged between 45 to 50 years old.  
*Hemangiopericytoma is more commonly observed among patients aged [age range] years old.
*Hemangiopericytoma is less commonly observed among children.  
*Hemangiopericytoma is more commonly observed among [elderly patients/young patients/children].
   
   
===Gender===
===Gender===
*Hemangiopericytoma affects men and women equally.
*Hemangiopericytoma affects men and women equally.
*[Gender 1] are more commonly affected with hemangiopericytoma than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
   
   
===Race===
===Race===
*There is no racial predilection for hemangiopericytoma.
*There is no racial predilection for hemangiopericytoma.
 
*Hemangiopericytoma usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop hemangiopericytoma.
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of hemangiopericytoma are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
*There are no associated risk factors in the development of hemangiopericytoma.
 
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with hemangiopericytoma remain asymptomatic for [duration/years].  
*The majority of patients with hemangiopericytoma are asymptomatic.  
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*Early clinical features include painless mass, or slow enlargement.
*If left untreated, [#%] of patients with hemangiopericytoma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, the majority of patients with hemangiopericytoma may progress to develop bone invasion.  
*Common complications of hemangiopericytoma include [complication 1], [complication 2], and [complication 3].
*Common complications of hemangiopericytoma, may include:
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with hemangiopericytoma is approximately [#%].
:*May profusely bleed during resection.
*The mean survival rate of patients with hemangiopericytoma is approximately 13 years.
 
:*1-year survival rate is 95%<ref name="pmid20367074">{{cite journal |vauthors=Rutkowski MJ, Sughrue ME, Kane AJ, Aranda D, Mills SA, Barani IJ, Parsa AT |title=Predictors of mortality following treatment of intracranial hemangiopericytoma |journal=J. Neurosurg. |volume=113 |issue=2 |pages=333–9 |year=2010 |pmid=20367074 |doi=10.3171/2010.3.JNS091882 |url=}}</ref>
:*5-year survival rate is 82%<ref name="pmid20367074">{{cite journal |vauthors=Rutkowski MJ, Sughrue ME, Kane AJ, Aranda D, Mills SA, Barani IJ, Parsa AT |title=Predictors of mortality following treatment of intracranial hemangiopericytoma |journal=J. Neurosurg. |volume=113 |issue=2 |pages=333–9 |year=2010 |pmid=20367074 |doi=10.3171/2010.3.JNS091882 |url=}}</ref>
:*10-year survival rate is 60%<ref name="pmid20367074">{{cite journal |vauthors=Rutkowski MJ, Sughrue ME, Kane AJ, Aranda D, Mills SA, Barani IJ, Parsa AT |title=Predictors of mortality following treatment of intracranial hemangiopericytoma |journal=J. Neurosurg. |volume=113 |issue=2 |pages=333–9 |year=2010 |pmid=20367074 |doi=10.3171/2010.3.JNS091882 |url=}}</ref>
:*20-year survival rate is 23%<ref name="pmid20367074">{{cite journal |vauthors=Rutkowski MJ, Sughrue ME, Kane AJ, Aranda D, Mills SA, Barani IJ, Parsa AT |title=Predictors of mortality following treatment of intracranial hemangiopericytoma |journal=J. Neurosurg. |volume=113 |issue=2 |pages=333–9 |year=2010 |pmid=20367074 |doi=10.3171/2010.3.JNS091882 |url=}}</ref>
 
== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of hemangiopericytoma is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== Symptoms ===
=== Symptoms ===
*Hemangiopericytoma is usually asymptomatic.
*Hemangiopericytoma is usually asymptomatic.
*Symptoms of hemangiopericytoma may include the following:
*There are no hallmark symptoms of hemangiopericytoma.
:*[symptom 1]
 
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]
=== Physical Examination ===
=== Physical Examination ===
*Patients with hemangiopericytoma usually appear [general appearance].
*Patients with hemangiopericytoma usually are well-appearing.
*Physical examination may be remarkable for:
*Physical examination may be remarkable for:
:*[finding 1]
:*Palpable mass
:*[finding 2]
:*No tenderness
:*[finding 3]
:*Located in the [[extremities]] (femur, proximal tibial, axilla or pelvis)
:*[finding 4]
 
:*[finding 5]
:*[finding 6]
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with hemangiopericytoma.
*There are no specific laboratory findings associated with hemangiopericytoma.


*A  [positive/negative] [test name] is diagnostic of hemangiopericytoma.
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of hemangiopericytoma.
*Other laboratory findings consistent with the diagnosis of hemangiopericytoma include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
===Imaging Findings===
*There are no [imaging study] findings associated with hemangiopericytoma.
*MRI is the imaging modality of choice for hemangiopericytoma.
*On MRI, characteristic findings of hemangiopericytoma, include:
*[Imaging study 1] is the imaging modality of choice for hemangiopericytoma.
 
*On [imaging study 1], hemangiopericytoma is characterized by [finding 1], [finding 2], and [finding 3].
:*Solitary fibrous tumor
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
:*Demonstrates a deep soft-tissue mass
:*Heterogenous high signal intensity on coronal [[Magnetic resonance imaging|STIR]] imaging
=== Other Diagnostic Studies ===
:*Brightly enhancing soft tissue mass
*Hemangiopericytoma may also be diagnosed using [diagnostic study name].
:*Often hyperintense on T2WI, with prominent flow void (most marked in haemangiopericytomas)
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
*Other imaging findings for hemangiopericytoma, may include angiography.
*On angiography, findings of hemangiopericytoma, include:
:*Dense
:*Well-circumscribed areas of enhancement with early draining veins and shunting.
:*Hemangiopericytoma are described as having a pedicle formed by the arteries supplying the tumor from which vessels branch to encircle the tumor
 
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy<ref name="pmid26605260">{{cite journal |vauthors=Kim YJ, Park JH, Kim YI, Jeun SS |title=Treatment Strategy of Intracranial Hemangiopericytoma |journal=Brain Tumor Res Treat |volume=3 |issue=2 |pages=68–74 |date=October 2015 |pmid=26605260 |pmc=4656898 |doi=10.14791/btrt.2015.3.2.68 |url=}}</ref>===
*There is no treatment for hemangiopericytoma; the mainstay of therapy is supportive care.
*Common medical therapy for hemangiopericytoma may include is adjuvant radiation therapy.
*The mainstay of therapy for hemangiopericytoma 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 ===
*Surgery is the mainstay of therapy for hemangiopericytoma.
*Surgery is the mainstay of therapy for hemangiopericytoma.<ref name="pmid30101003">{{cite journal |vauthors=Ciliberti MP, D'Agostino R, Gabrieli L, Nikolaou A, Sardaro A |title=The radiation therapy options of intracranial hemangiopericytoma: An overview and update on a rare vascular mesenchymal tumor |journal=Oncol Rev |volume=12 |issue=2 |pages=354 |date=July 2018 |pmid=30101003 |pmc=6068394 |doi=10.4081/oncol.2018.354 |url=}}</ref>
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of hemangiopericytoma.
*Arterial preoperative [[embolisation]] is highly recommended among patients with hemangiopericytoma.<ref name="hemangio"> Solitary Fibrous Tumors and So-Called Hemangiopericytoma. http://www.hindawi.com/journals/sarcoma/2012/690251/cta/ Accessed on April 19, 2016</ref>
*[Surgical procedure] can only be performed for patients with [disease stage] hemangiopericytoma.
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for hemangiopericytoma.
*There are no primary preventive measures available for hemangiopericytoma.<ref name="hemangio"> Solitary Fibrous Tumors and So-Called Hemangiopericytoma. http://www.hindawi.com/journals/sarcoma/2012/690251/cta/ Accessed on April 19, 2016</ref>
*Effective measures for the primary prevention of hemangiopericytoma include [measure1], [measure2], and [measure3].
 
*Once diagnosed and successfully treated, patients with hemangiopericytoma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
 


==References==
==References==
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[[Category: Oncology]]
[[Category: Oncology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Immunology]]
[[Category:Vascular medicine]]

Latest revision as of 12:28, 28 August 2019

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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: HPC; Solitary fibrous tumor

Overview

Hemangiopericytoma (HPC) is a type of soft tissue vascular sarcoma that originates in the pericytes in the walls of capillaries. Typically, hemangiopericytomas have large vessels especially located at its periphery, and commonly involve the lower limbs (35% of cases), especially the thigh, pelvis, and retroperitoneum (25%). Hemangiopericytoma was first described by Arthur Purdy Stout and Margaret Ransone Murray, two American pathologists, in 1942. mThe NF2 gene mutation has been associated with the development of hemangiopericytoma. If left untreated, the majority of patients with hemangiopericytoma may progress to develop bone invasion. On MRI is the imaging modality of choice for hemangiopericytoma. On MRI, characteristic findings of hemangiopericytoma, include: solitary fibrous tumor, demonstrates a deep soft-tissue mass, and heterogenous high signal intensity on coronal STIR imaging. Surgery is the mainstay of therapy for hemangiopericytoma. Arterial preoperative embolisation is highly recommended to avoid bleeding among patients with hemangiopericytoma.[1]

Historical Perspective

  • Hemangiopericytoma was first described by Arthur Purdy Stout and Margaret Ransone Murray, two American pathologists, in 1942.

Classification

  • Hemangiopericytoma may be classified into 4 groups:
  • Hemangiopericytoma of the spleen
  • Dural hemangiopericytomas
  • Myopericytoma
  • Infantile myofibromatosis
  • Sinonasal hemangiopericytomas
  • Other associated conditions, include: Doege-Potter syndrome

Pathophysiology

  • Hemangiopericytomas arise from pericytes, which are normally involved in the formation of connective tissue around small vessels walls and are also associated in flow regulation.[2]
  • The NF2 gene mutation has been associated with the development of hemangiopericytoma.
  • On gross pathology, characteristic findings of hemangiopericytoma, include:
  • Large mass
  • Poorly demarcated margins
  • On microscopic histopathological analysis, characteristic findings of hemangiopericytoma, include:
  • Hypervascular lesion
  • Abundant thin-walled branching small vessels of variable size.
  • Spindle or ovoid shaped cells in nests or sheets
  • Cells may "onion-skin" around thin blood vessels
  • Hemangiopericytoma-like area (staghorn vessels)
  • Keloid-like collagen bundles (key feature)

Causes

  • There are no established causes for hemangiopericytoma.

Differentiating Hemangiopericytoma From Other Diseases

  • Hemangiopericytoma must be differentiated from other diseases that cause a slow growing painless mass, such as:

Epidemiology and Demographics

  • Hemangiopericytoma represents only about 1-2% of all soft-tissue tumors.

Age

  • The median age at diagnosis is 45 years.
  • Hemangiopericytoma is more commonly observed among patients aged between 45 to 50 years old.
  • Hemangiopericytoma is less commonly observed among children.

Gender

  • Hemangiopericytoma affects men and women equally.

Race

  • There is no racial predilection for hemangiopericytoma.

Risk Factors

  • There are no associated risk factors in the development of hemangiopericytoma.

Natural History, Complications and Prognosis

  • The majority of patients with hemangiopericytoma are asymptomatic.
  • Early clinical features include painless mass, or slow enlargement.
  • If left untreated, the majority of patients with hemangiopericytoma may progress to develop bone invasion.
  • Common complications of hemangiopericytoma, may include:
  • May profusely bleed during resection.
  • The mean survival rate of patients with hemangiopericytoma is approximately 13 years.
  • 1-year survival rate is 95%[3]
  • 5-year survival rate is 82%[3]
  • 10-year survival rate is 60%[3]
  • 20-year survival rate is 23%[3]

Diagnosis

Symptoms

  • Hemangiopericytoma is usually asymptomatic.
  • There are no hallmark symptoms of hemangiopericytoma.

Physical Examination

  • Patients with hemangiopericytoma usually are well-appearing.
  • Physical examination may be remarkable for:
  • Palpable mass
  • No tenderness
  • Located in the extremities (femur, proximal tibial, axilla or pelvis)

Laboratory Findings

  • There are no specific laboratory findings associated with hemangiopericytoma.

Imaging Findings

  • MRI is the imaging modality of choice for hemangiopericytoma.
  • On MRI, characteristic findings of hemangiopericytoma, include:
  • Solitary fibrous tumor
  • Demonstrates a deep soft-tissue mass
  • Heterogenous high signal intensity on coronal STIR imaging
  • Brightly enhancing soft tissue mass
  • Often hyperintense on T2WI, with prominent flow void (most marked in haemangiopericytomas)
  • Other imaging findings for hemangiopericytoma, may include angiography.
  • On angiography, findings of hemangiopericytoma, include:
  • Dense
  • Well-circumscribed areas of enhancement with early draining veins and shunting.
  • Hemangiopericytoma are described as having a pedicle formed by the arteries supplying the tumor from which vessels branch to encircle the tumor

Treatment

Medical Therapy[4]

  • Common medical therapy for hemangiopericytoma may include is adjuvant radiation therapy.

Surgery

  • Surgery is the mainstay of therapy for hemangiopericytoma.[5]
  • Arterial preoperative embolisation is highly recommended among patients with hemangiopericytoma.[6]

Prevention

  • There are no primary preventive measures available for hemangiopericytoma.[6]

References

  1. ↑ Penel N, Amela EY, Decanter G, Robin YM, Marec-Berard P (2012). "Solitary fibrous tumors and so-called hemangiopericytoma". Sarcoma. 2012: 690251. doi:10.1155/2012/690251. PMC 3337510. PMID 22566753.
  2. ↑ McMaster MJ, Soule EH, Ivins JC (December 1975). "Hemangiopericytoma. A clinicopathologic study and long-term followup of 60 patients". Cancer. 36 (6): 2232–44. doi:10.1002/cncr.2820360942. PMID 1203874.
  3. ↑ 3.0 3.1 3.2 3.3 Rutkowski MJ, Sughrue ME, Kane AJ, Aranda D, Mills SA, Barani IJ, Parsa AT (2010). "Predictors of mortality following treatment of intracranial hemangiopericytoma". J. Neurosurg. 113 (2): 333–9. doi:10.3171/2010.3.JNS091882. PMID 20367074.
  4. ↑ Kim YJ, Park JH, Kim YI, Jeun SS (October 2015). "Treatment Strategy of Intracranial Hemangiopericytoma". Brain Tumor Res Treat. 3 (2): 68–74. doi:10.14791/btrt.2015.3.2.68. PMC 4656898. PMID 26605260.
  5. ↑ Ciliberti MP, D'Agostino R, Gabrieli L, Nikolaou A, Sardaro A (July 2018). "The radiation therapy options of intracranial hemangiopericytoma: An overview and update on a rare vascular mesenchymal tumor". Oncol Rev. 12 (2): 354. doi:10.4081/oncol.2018.354. PMC 6068394. PMID 30101003.
  6. ↑ 6.0 6.1 Solitary Fibrous Tumors and So-Called Hemangiopericytoma. http://www.hindawi.com/journals/sarcoma/2012/690251/cta/ Accessed on April 19, 2016
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