Jump to content

Epithelioid hemangioendothelioma: Difference between revisions

From Wikipedia, the free encyclopedia
Browse history interactively
← Previous edit
Content deleted Content added
VisualWikitext
Yobot (talk | contribs)
Bots
4,733,870 edits
m Removed invisible unicode characters + other fixes, replaced: → (2) using AWB (12065)
Citation bot (talk | contribs)
Bots
5,404,410 edits
Alter: template type. Add: pmc, pmid, pages, issue, volume, journal, date, title, doi, authors 1-7. Removed proxy/dead URL that duplicated identifier. | Use this bot. Report bugs. | Suggested by Jay8g | Linked from User:Jay8g/sandbox | #UCB_webform_linked 20/252
 
(47 intermediate revisions by 28 users not shown)
Line 1: Line 1:
{{Infobox medical condition
{{Infobox medical condition (new)
|Name = Epithelioid Hemangioendothelioma
| name = Epithelioid Hemangioendothelioma
|ICD10 =
| synonyms =
|ICD9 =
| image = Epithelioid hemangioendothelioma.jpg
|ICDO = {{ICDO|9133|}}
| width = 200px
|Image = Epithelioid hemangioendothelioma.jpg
| caption = [[Micrograph]] of an epithelioid hemangioendothelioma of the liver.
|Image_size = 200px
| pronounce =
| field = oncology
|Caption = [[Micrograph]] of an epithelioid hemangioendothelioma of the liver.
|OMIM =
| symptoms =
| complications =
⚫
|OMIM_mult =
|MedlinePlus =
| onset =
| duration =
⚫
|eMedicineSubj =
| types =
⚫
|eMedicineTopic =
|DiseasesDB = 34264
| causes =
|MeshID = D000230
| risks =
| diagnosis =
|
| differential =
| prevention =
| treatment =
| medication =
| prognosis =
| frequency =
| deaths =
}}
}}
'''Epithelioid hemangioendothelioma''' (eHAE) is a rare tumor, first characterized by [[Sharon Weiss]] and Franz Enzinger that both clinically and histologically is intermediate between [[angiosarcoma]] and [[hemangioma]]. However, a distinct, disease-defining genetic alteration recently described for EHE indicates that it is an entirely separate entity from both angiosarcoma and hemangioma.
'''Epithelioid hemangioendothelioma''' (EHE) is a rare tumor, first characterized by [[Sharon Weiss]] and Franz Enzinger in 1982<ref name="pmid7093931">{{cite journal | vauthors = Weiss SW, Enzinger FM | title = Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma | journal = Cancer | volume = 50 | issue = 5 | pages = 970–81 | date = September 1982 | pmid = 7093931 | doi = 10.1002/1097-0142(19820901)50:5<970::aid-cncr2820500527>3.0.co;2-z | doi-access=free}}</ref> that both clinically and histologically is intermediate between [[angiosarcoma]] and [[hemangioma]]. However, a distinct, disease-defining genetic alteration recently described for EHE indicates that it is an entirely separate entity from both angiosarcoma and hemangioma.


EHE is a soft tissue sarcoma and is generally considered a vascular [[cancer]] insofar as the ‘lesional’ cells have surface markers typical of endothelial cells, (cells lining the interior of blood vessels). EHE was originally described as occurring most commonly in the veins of the extremities (arms and legs) and two organs, the liver and lungs. It has since been described in organs throughout the body. In addition to liver and lungs, bones and skin have been the most frequent organs.
EHE is a soft tissue sarcoma and is generally considered a vascular [[cancer]] insofar as the ‘lesional’ cells have surface markers typical of endothelial cells (cells lining the interior of blood vessels). EHE was originally described as occurring most commonly in the veins of the extremities (arms and legs) and two organs, the liver<ref name="pmid22544493">{{cite journal | vauthors = Mistry AM, Gorden DL, Busler JF, Coogan AC, Kelly BS | title = Diagnostic and therapeutic challenges in hepatic epithelioid hemangioendothelioma | journal = J Gastrointest Cancer | volume = 43 | issue = 4 | pages = 521–5 | date = December 2012 | pmid = 22544493 | doi = 10.1007/s12029-012-9389-y | s2cid = 23391808 }}</ref> and lungs. It has since been described in organs throughout the body. In addition to liver and lungs, bones and skin have been the most frequent organs.


Before the initial description of Weiss, the tumor had been reported under a variety of other names, including [[Histiocytoid hemangioma|histiocytoid]] [[hemangioendothelioma]], intravascular [[Bronchioalveolar carcinoma|bronchoalveolar]] tumor (in the lung), and [[Cholangiocarcinoma|sclerosing cholangiocarcinoma]]. In the lung and liver, common sites of metastatic tumor, it was most likely to be confused with [[carcinoma]] a far more common type of tumor.
Before the initial description of Weiss, the tumor had been reported under a variety of other names, including [[Histiocytoid hemangioma|histiocytoid]] [[hemangioendothelioma]], intravascular [[Bronchioalveolar carcinoma|bronchoalveolar]] tumor (in the lung), and [[Cholangiocarcinoma|sclerosing cholangiocarcinoma]]. In the lung and liver, common sites of metastatic tumor, it was most likely to be confused with [[carcinoma]] a far more common type of tumor.


EHE typically occurs in the 20 - 40 age range although the overall age range involved is much broader and a modest predilection for females over males. It often has an indolent course, and many affected people have survived for decades with multi-organ disease.<sup>[1]:601</sup> The extent and number of organs involved apparently has little effect on longevity.
EHE typically occurs in the 20 – 40 age range although the overall age range involved is much broader and a modest predilection for females over males. It often has an indolent course, and many affected people have survived for decades with multi-organ disease.<sup>[1]:601</sup> The extent and number of organs involved apparently has little effect on longevity.


==Genetics==
==Genetics==
The cytogenetics of EHE gave some of the first clues of an underlying genetic alteration. A balanced, reciprocal translocation t(1;3)(p36.3;q25) in EHE tumor cells was first described by Mendlick et al in 2001 (PMID 11342784). This led to the landmark paper by Tanas at el in 2011(PMID 21885404) describing the specific genes involved in the translocation associated with the most common forms of EHE. This alteration results in the fusion of genes coding for two transcription co-activators (transcriptional regulators): TAZ (transcriptional co-activator with PDZ-binding motif) also known as WWTR1 (WW domain-containing transcription regulator protein 1) and CAMTA1 (calmodulin-binding transcription activator 1). The names in parenthesis are not relevant to casual (or even science) readers but are included to help distinguish them from other genes. For instance, another gene with an entirely different function, [[Tafazzin]], is unrelated to EHE but confusingly, also referred to as TAZ. In any case, the EHE translocation results in an abnormal ‘fusion gene’ that expresses an abnormal mRNA resulting in synthesis of a fusion protein variant of TAZ that is always turned on. This form of TAZ always resides in the nucleus and therefore is constitutively active. It binds and turns on a very important member of the TEAD family of transcription factors and this causes cells to proliferate. It is this production of the TAZ-TEAD transcriptome that causes the affected endothelial cells to grow into tumors. In normal cells, TAZ is considered a major negative transducer of the Hippo pathway, a signaling system that regulates organ size by causing cells to stop growing when they touch each other (contact inhibition). Many upstream inputs regulate the Hippo signal which normally functions to turn off or de-activate TAZ by keeping it in the cytoplasm and out of the nucleus. In EHE cells, the abnormal fusion TAZ is ‘immune’ to this input and just stays in the nucleus, and continuing to stimulate cell growth.
The cytogenetics of EHE gave some of the first clues of an underlying genetic alteration. A balanced, reciprocal translocation t(1;3)(p36.3;q25) in EHE tumor cells was first described by Mendlick et al. in 2001.<ref name="pmid11342784">{{cite journal | vauthors = Mendlick MR, Nelson M, Pickering D, Johansson SL, Seemayer TA, Neff JR, Vergara G, Rosenthal H, Bridge JA |display-authors = 6| title = Translocation t(1;3)(p36.3;q25) is a nonrandom aberration in epithelioid hemangioendothelioma | journal = Am J Surg Pathol | volume = 25 | issue = 5 | pages = 684–7 | date = May 2001 | pmid = 11342784 | doi = 10.1097/00000478-200105000-00019 |s2cid = 26397433}}</ref> This led to the landmark paper by Tanas et al. in 2011<ref name="pmid21885404">{{cite journal | vauthors = Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP|display-authors = 6 | title = Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma | journal = Sci Transl Med | volume = 3 | issue = 98 | pages = 98ra82 | date = August 2011 | pmid = 21885404 | doi = 10.1126/scitranslmed.3002409 |s2cid = 206678129 }}</ref> describing the specific genes involved in the translocation associated with the most common forms of EHE. This alteration results in the fusion of genes coding for two transcription co-activators (transcriptional regulators): TAZ (transcriptional co-activator with PDZ-binding motif) also known as WWTR1 (WW domain-containing transcription regulator protein 1) and CAMTA1 (calmodulin-binding transcription activator 1). The names in parentheses are not relevant to casual (or even science) readers but are included to help distinguish them from other genes. For instance, another gene with an entirely different function, [[Tafazzin]], is unrelated to EHE but confusingly, also referred to as TAZ. In any case, the EHE translocation results in an abnormal ‘fusion gene’ that expresses an abnormal mRNA resulting in synthesis of a fusion protein variant of TAZ that is always turned on. This form of TAZ always resides in the nucleus and therefore is constitutively active. It binds and turns on a very important member of the TEAD family of transcription factors and this causes cells to proliferate. It is this production of the TAZ-TEAD transcriptome that causes the affected endothelial cells to grow into tumors. In normal cells, TAZ is considered a major negative transducer of the Hippo pathway, a signaling system that regulates organ size by causing cells to stop growing when they touch each other (contact inhibition). Many upstream inputs regulate the Hippo signal which normally functions to turn off or de-activate TAZ by keeping it in the cytoplasm and out of the nucleus. In EHE cells, the abnormal fusion TAZ is ‘immune’ to this input and just stays in the nucleus, and continuing to stimulate cell growth.


Note that about 10% of EHE patients harbor a different translocation. This one similarly results in the constitutive activation of YAP, an orthologue of TAZ (i.e., a gene that has sequence and function that are very similar to TAZ). This also results in persistent, unregulated growth of the affected cells and therefore causes EHE-type tumors.
Note that about 10% of EHE patients harbor a different translocation. This one similarly results in the constitutive activation of YAP, an orthologue of TAZ (i.e., a gene that has sequence and function that are very similar to TAZ). This also results in persistent, unregulated growth of the affected cells and therefore causes EHE-type tumors.


==Epidemiology==
==Treatment==
*[[Sirolimus]]: Also known as rapamycin, this oral medication suppresses the immune system and slows the growth of abnormal lymphatic vessels that form the tumor. This can help shrink EHE tumors and improve symptoms, including pain
It is so rare that only 0.01 percent of the cancer population has it and it affects about 1 person in every 1,000,000 worldwide.<ref>http://www.childrenshospital.org/az/Site844/mainpageS844P1.html</ref> Around 20 cases are diagnosed in [[United States|America]] every year - the cause is unknown.<ref>http://www.heardsupport.org/whatisHEARD.html</ref> It is unresponsive to any known strain of [[chemotherapy]], making treatment very difficult.


*[[Tyrosine kinase inhibitors]]: These drugs, designed as targeted therapies for cancers, have shown short-term success with EHE. Examples include sorafenib, sunitinib and pazopanib.
⚫
==Prognosis==
⚫
Although Epithelioid Hemangioendothelioma typically presents as a low grade tumour occasionally eHAE presents as high grade and more aggressive. eHAE presenting in the pleura, for example, is associated with a much more aggressive and hard to treat course.<ref>http://www.ajronline.org/content/175/6/1545.long</ref> There is no standard chemotherapy treatment for eHAE at current but success with drugs such as Interferon, Paclitaxel, MAID, Thalidomide and Doxurubicin have been reported.


*[[Vincristine]]: This chemotherapy drug targets all dividing cells within the body and is therefore used to treat many cancers. It is also used for aggressive benign vascular tumors.
== The EHE Foundation - 501(c)(3) Organization ==
In the United States, [http://fightehe.org/ The EHE Foundation] is the leading non-profit organization dedicated to furthering EHE research. Dr. Brian Rubin, a world-renowned pathologist and medical researcher at the Cleveland Clinic, has been studying the molecular and genetic makeup of EHE since 2011. Dr. Rubin's discovery of the specific gene fusion (WWTR1-CAMTA1) associated with EHE tumors led to the development of cell-based biological EHE models, a scientific breakthrough and quantum leap forward.<ref>{{Cite web|url=http://fightehe.org/ehe-epithelioid-hemangioendothelioma/|title=What is Epithelioid Hemangioendothelioma (EHE)?|website=fightehe.org|access-date=2016-08-05}}</ref>


*[[Interferon]]: The body produces interferon to combat infections or control inflammation. It has been formulated into a medication that targets blood vessel growth.
Though The EHE Foundation supports various programs related to EHE, the vast majority of its funds are directed to Dr. Rubin at the Cleveland Clinic.


*[[Multi-agent chemotherapy]]: EHE tumors that grow rapidly, spread to other tissues or do not respond to other medications may require more aggressive drug therapy. However, this combination of medications is rarely needed in children and young adults with EHE.
Dr. Rubin's laboratory is currently studying more than 40 patient-directed tissue samples and trying to determine a more accurate method of predicting disease progression. The lab is focused on developing therapeutic compounds that target TAZ-CAMTA1 under the belief that if the encoded protein can be inhibited, EHE can be controlled.


*Others like [[surgery]] can be done as treatment for the virus during its most severe.<ref>{{cite web|url=https://www.childrenshospital.org/conditions-and-treatments/conditions/e/epithelioid-hemangioendothelioma/diagnosis-and-treatment|publisher=[[Boston Children's Hospital]]|accessdate=June 17, 2021|title=Epithelioid Hemangioendolthelioma (EHE) - Diagnosis & Treatment}}</ref>
==Communities of people with eHAE==

⚫
There is a Facebook site set up for people with EHE.<ref>[https://www.facebook.com/groups/EHEcancer]</ref>
⚫
==Prognosis==
⚫
There is also a Registry for patients to enter their medical history.<ref>[https://connect.patientcrossroads.org/?org=cravat]</ref>
⚫
Although Epithelioid Hemangioendothelioma typically presents as a low-grade tumor, occasionally, eHAE presents as high grade and more aggressive. eHAE presenting in the pleura, for example, is associated with a much more aggressive and hard to treat course.<ref name="pmid11090371">{{cite journal | vauthors = Crotty EJ, McAdams HP, Erasmus JJ, Sporn TA, Roggli VL | title = Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features | journal = AJR Am J Roentgenol | volume = 175 | issue = 6 | pages = 1545–9 | date = December 2000 | pmid = 11090371 | doi = 10.2214/ajr.175.6.1751545 }}</ref> There is no standard chemotherapy treatment for eHAE at current but success with drugs such as [[Interferon]], [[Paclitaxel]], MAID combination chemotherapy, [[Thalidomide]] and [[Doxorubicin]] have been reported.
CRAVAT [[Center for Research and Analysis of VAscular Tumors]] is a website for the EHE community.<ref>[http://www.cravatfoundation.org]</ref>

⚫
. The EHE Rare Cancer Foundation Australia was established in 2015 by Australians with Epithelioid Hemangioendothelioma (EHE).<ref>{{Cite web|url=http://www.ehefoundation.com.au/|title=EHE Rare Cancer Foundation Australia|last=generator|first=metatags|website=www.ehefoundation.com.au|access-date=2016-07-06}}</ref> The core objective of the EHE Rare Cancer Foundation Australia is to proactively fundraise in order to support research into this rare cancer in the hope that a maintenance program or cure can be found.<ref>{{Cite web|url=http://www.ehefoundation.com.au/|title=EHE Rare Cancer Foundation Australia|last=generator|first=metatags|website=www.ehefoundation.com.au|access-date=2016-07-06}}</ref>
==Epidemiology==
It is so rare that only 0.01 percent of the cancer population has it and it affects about 1 person in every 1,000,000 worldwide.<ref>{{cite web|archive-url=https://web.archive.org/web/20170818005852/http://www.childrenshospital.org/conditions-and-treatments/conditions/epithelioid-hemangioendothelioma|url=http://www.childrenshospital.org/conditions-and-treatments/conditions/epithelioid-hemangioendothelioma|archive-date=August 18, 2017|publisher=Boston Children's Hospital | title = Epithelioid Hemangioendothelioma in children}}</ref> Around 90 cases are diagnosed in the [[United States]] every year.<ref>{{Cite journal |doi=10.1002/cam4.6181 |title=Incidence, demographics, and survival of malignant hemangioendothelioma in the United States |date=2023 |last1=Paulson |first1=Kelly G. |last2=Ravi |first2=Vinod |last3=Rubin |first3=Brian P. |last4=Park |first4=Min |last5=Loggers |first5=Elizabeth T. |last6=Cranmer |first6=Lee D. |last7=Wagner |first7=Michael J. |journal=Cancer Medicine |volume=12 |issue=14 |pages=15101–15106 |pmid=37260142 |pmc=10417180 }}</ref> It is unresponsive to any known strain of [[chemotherapy]], making treatment very difficult.

==Society==
⚫
There is a Facebook site set up for people with EHE.<ref>{{Cite web | url=https://www.facebook.com/groups/EHEcancer | title=Epithelioid Hemangioendothelioma (EHE) Cancer| website=[[Facebook]]}}</ref>
⚫
There is also a Registry for patients to enter their medical history.<ref>{{Cite web | url=https://connect.patientcrossroads.org/?org=cravat | title=Home}}</ref>
⚫
CRAVAT [[Center for Research and Analysis of VAscular Tumors]] is a website for the EHE community.<ref>{{Cite web | url=http://www.cravatfoundation.org | title=Epithelioid Hemangioendothelioma Cancer Foundation}}</ref> The EHE Rare Cancer Foundation Australia was established in 2015 by Australians with Epithelioid Hemangioendothelioma (EHE).<ref>{{Cite web|url=http://www.ehefoundation.com.au/|title=EHE Rare Cancer Foundation Australia|last=generator|first=metatags|website=www.ehefoundation.com.au|access-date=2016-07-06}}</ref> The core objective of the EHE Rare Cancer Foundation Australia is to proactively fundraise in order to support research into this rare cancer in the hope that a maintenance program or cure can be found.<ref>{{Cite web|url=http://www.ehefoundation.com.au/|title=EHE Rare Cancer Foundation Australia|last=generator|first=metatags|website=www.ehefoundation.com.au|access-date=2016-07-06}}</ref>

⚫
In 2003 photographer and actress [[Kris Carr]] was diagnosed with a stable and low grade version of eHAE. Carr has become a success as a 'Wellness Warrior' advocating a vegan lifestyle as a way to avoid and stabilize disease.<ref>{{Cite web|url=https://www.scientificamerican.com/article/living-with-cancer-kris-carr/|title=Living with Cancer: The Kris Carr's Story|vauthors = Stein L|website= Scientific American|date=July 16, 2008}}</ref>


⚫
In 2003 photographer and actress [[Kris Carr]] was diagnosed with a stable and low grade version of eHAE. Carr has become a success as a 'Wellness Warrior' advocating a vegan lifestyle as a way to avoid and stabilize disease.<ref>[http://www.sciam.com/article.cfm?id=living-with-cancer-kris-carr Living with Cancer: The Kris Carr's Story]</ref>


== See also ==
== See also ==
Line 55: Line 68:


==References==
==References==
{{Research help|Med}}
{{reflist}}
{{reflist}}


== External links ==
{{Medical resources
| ICD10 = D18.0
| ICD9 =
| ICDO = {{ICDO|9133|}}
| OMIM =
⚫
| OMIM_mult =
| MedlinePlus =
⚫
| eMedicineSubj =
⚫
| eMedicineTopic =
| DiseasesDB = 34264
| MeshID = D000230
| Orphanet = 157791
}}
{{Vascular tumors}}
{{Vascular tumors}}


Latest revision as of 20:12, 22 December 2023

Epithelioid Hemangioendothelioma
Micrograph of an epithelioid hemangioendothelioma of the liver.
SpecialtyOncology

Epithelioid hemangioendothelioma (EHE) is a rare tumor, first characterized by Sharon Weiss and Franz Enzinger in 1982[1] that both clinically and histologically is intermediate between angiosarcoma and hemangioma. However, a distinct, disease-defining genetic alteration recently described for EHE indicates that it is an entirely separate entity from both angiosarcoma and hemangioma.

EHE is a soft tissue sarcoma and is generally considered a vascular cancer insofar as the ‘lesional’ cells have surface markers typical of endothelial cells (cells lining the interior of blood vessels). EHE was originally described as occurring most commonly in the veins of the extremities (arms and legs) and two organs, the liver[2] and lungs. It has since been described in organs throughout the body. In addition to liver and lungs, bones and skin have been the most frequent organs.

Before the initial description of Weiss, the tumor had been reported under a variety of other names, including histiocytoid hemangioendothelioma, intravascular bronchoalveolar tumor (in the lung), and sclerosing cholangiocarcinoma. In the lung and liver, common sites of metastatic tumor, it was most likely to be confused with carcinoma a far more common type of tumor.

EHE typically occurs in the 20 – 40 age range although the overall age range involved is much broader and a modest predilection for females over males. It often has an indolent course, and many affected people have survived for decades with multi-organ disease.[1]:601 The extent and number of organs involved apparently has little effect on longevity.

Genetics

[edit]

The cytogenetics of EHE gave some of the first clues of an underlying genetic alteration. A balanced, reciprocal translocation t(1;3)(p36.3;q25) in EHE tumor cells was first described by Mendlick et al. in 2001.[3] This led to the landmark paper by Tanas et al. in 2011[4] describing the specific genes involved in the translocation associated with the most common forms of EHE. This alteration results in the fusion of genes coding for two transcription co-activators (transcriptional regulators): TAZ (transcriptional co-activator with PDZ-binding motif) also known as WWTR1 (WW domain-containing transcription regulator protein 1) and CAMTA1 (calmodulin-binding transcription activator 1). The names in parentheses are not relevant to casual (or even science) readers but are included to help distinguish them from other genes. For instance, another gene with an entirely different function, Tafazzin, is unrelated to EHE but confusingly, also referred to as TAZ. In any case, the EHE translocation results in an abnormal ‘fusion gene’ that expresses an abnormal mRNA resulting in synthesis of a fusion protein variant of TAZ that is always turned on. This form of TAZ always resides in the nucleus and therefore is constitutively active. It binds and turns on a very important member of the TEAD family of transcription factors and this causes cells to proliferate. It is this production of the TAZ-TEAD transcriptome that causes the affected endothelial cells to grow into tumors. In normal cells, TAZ is considered a major negative transducer of the Hippo pathway, a signaling system that regulates organ size by causing cells to stop growing when they touch each other (contact inhibition). Many upstream inputs regulate the Hippo signal which normally functions to turn off or de-activate TAZ by keeping it in the cytoplasm and out of the nucleus. In EHE cells, the abnormal fusion TAZ is ‘immune’ to this input and just stays in the nucleus, and continuing to stimulate cell growth.

Note that about 10% of EHE patients harbor a different translocation. This one similarly results in the constitutive activation of YAP, an orthologue of TAZ (i.e., a gene that has sequence and function that are very similar to TAZ). This also results in persistent, unregulated growth of the affected cells and therefore causes EHE-type tumors.

Treatment

[edit]
  • Sirolimus: Also known as rapamycin, this oral medication suppresses the immune system and slows the growth of abnormal lymphatic vessels that form the tumor. This can help shrink EHE tumors and improve symptoms, including pain
  • Tyrosine kinase inhibitors: These drugs, designed as targeted therapies for cancers, have shown short-term success with EHE. Examples include sorafenib, sunitinib and pazopanib.
  • Vincristine: This chemotherapy drug targets all dividing cells within the body and is therefore used to treat many cancers. It is also used for aggressive benign vascular tumors.
  • Interferon: The body produces interferon to combat infections or control inflammation. It has been formulated into a medication that targets blood vessel growth.
  • Multi-agent chemotherapy: EHE tumors that grow rapidly, spread to other tissues or do not respond to other medications may require more aggressive drug therapy. However, this combination of medications is rarely needed in children and young adults with EHE.
  • Others like surgery can be done as treatment for the virus during its most severe.[5]

Prognosis

[edit]

Although Epithelioid Hemangioendothelioma typically presents as a low-grade tumor, occasionally, eHAE presents as high grade and more aggressive. eHAE presenting in the pleura, for example, is associated with a much more aggressive and hard to treat course.[6] There is no standard chemotherapy treatment for eHAE at current but success with drugs such as Interferon, Paclitaxel, MAID combination chemotherapy, Thalidomide and Doxorubicin have been reported.

Epidemiology

[edit]

It is so rare that only 0.01 percent of the cancer population has it and it affects about 1 person in every 1,000,000 worldwide.[7] Around 90 cases are diagnosed in the United States every year.[8] It is unresponsive to any known strain of chemotherapy, making treatment very difficult.

Society

[edit]

There is a Facebook site set up for people with EHE.[9] There is also a Registry for patients to enter their medical history.[10] CRAVAT Center for Research and Analysis of VAscular Tumors is a website for the EHE community.[11] The EHE Rare Cancer Foundation Australia was established in 2015 by Australians with Epithelioid Hemangioendothelioma (EHE).[12] The core objective of the EHE Rare Cancer Foundation Australia is to proactively fundraise in order to support research into this rare cancer in the hope that a maintenance program or cure can be found.[13]

In 2003 photographer and actress Kris Carr was diagnosed with a stable and low grade version of eHAE. Carr has become a success as a 'Wellness Warrior' advocating a vegan lifestyle as a way to avoid and stabilize disease.[14]


See also

[edit]

References

[edit]
  1. ^ Weiss SW, Enzinger FM (September 1982). "Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma". Cancer. 50 (5): 970–81. doi:10.1002/1097-0142(19820901)50:5<970::aid-cncr2820500527>3.0.co;2-z. PMID 7093931.
  2. ^ Mistry AM, Gorden DL, Busler JF, Coogan AC, Kelly BS (December 2012). "Diagnostic and therapeutic challenges in hepatic epithelioid hemangioendothelioma". J Gastrointest Cancer. 43 (4): 521–5. doi:10.1007/s12029-012-9389-y. PMID 22544493. S2CID 23391808.
  3. ^ Mendlick MR, Nelson M, Pickering D, Johansson SL, Seemayer TA, Neff JR, et al. (May 2001). "Translocation t(1;3)(p36.3;q25) is a nonrandom aberration in epithelioid hemangioendothelioma". Am J Surg Pathol. 25 (5): 684–7. doi:10.1097/00000478-200105000-00019. PMID 11342784. S2CID 26397433.
  4. ^ Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, et al. (August 2011). "Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma". Sci Transl Med. 3 (98): 98ra82. doi:10.1126/scitranslmed.3002409. PMID 21885404. S2CID 206678129.
  5. ^ "Epithelioid Hemangioendolthelioma (EHE) - Diagnosis & Treatment". Boston Children's Hospital. Retrieved June 17, 2021.
  6. ^ Crotty EJ, McAdams HP, Erasmus JJ, Sporn TA, Roggli VL (December 2000). "Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features". AJR Am J Roentgenol. 175 (6): 1545–9. doi:10.2214/ajr.175.6.1751545. PMID 11090371.
  7. ^ "Epithelioid Hemangioendothelioma in children". Boston Children's Hospital. Archived from the original on August 18, 2017.
  8. ^ Paulson, Kelly G.; Ravi, Vinod; Rubin, Brian P.; Park, Min; Loggers, Elizabeth T.; Cranmer, Lee D.; Wagner, Michael J. (2023). "Incidence, demographics, and survival of malignant hemangioendothelioma in the United States". Cancer Medicine. 12 (14): 15101–15106. doi:10.1002/cam4.6181. PMC 10417180. PMID 37260142.
  9. ^ "Epithelioid Hemangioendothelioma (EHE) Cancer". Facebook.
  10. ^ "Home".
  11. ^ "Epithelioid Hemangioendothelioma Cancer Foundation".
  12. ^ generator, metatags. "EHE Rare Cancer Foundation Australia". www.ehefoundation.com.au. Retrieved 2016-07-06.
  13. ^ generator, metatags. "EHE Rare Cancer Foundation Australia". www.ehefoundation.com.au. Retrieved 2016-07-06.
  14. ^ Stein L (July 16, 2008). "Living with Cancer: The Kris Carr's Story". Scientific American.


[edit]
Retrieved from "https://en.wikipedia.org/enwiki/w/index.php?title=Epithelioid_hemangioendothelioma&oldid=1191315869"