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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/09/19 20:18:44」(JST)

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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. Some now refer to it as low grade epithelioid angiosarcoma. It typically occurs in the 20 - 40 age range with no sex predilection, although the overall age range involved is much broader. It has an indolent course, some have survived for decades with multi-organ disease.^[1]^:601 Before their description 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 liver). In the lung and liver, common sites of metastatic tumor, it was most likely to be confused with carcinoma (tumor with epithelial differentiation), a far more common type of tumor.

It is a vascular cancer in the lining (intima) of blood vessels, originally described 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.

Genetics

The cytogenetics of eHAE are not well characterized, as yet: t(1;3)(p36.3;q25) and a small supernumerary marker chromosome involving 22qll have been described in some patients.

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.^[2] Around 20 cases are diagnosed in America every year - the cause is unknown.^[3] It is unresponsive to any known strain of chemotherapy, making treatment very difficult.

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.^[4] 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.

People with eHAE

There is a Facebook site set up for people with EHE.^[5] There is also a Registry for patients to enter their medical history.^[6] CRAVAT Center for Research and Analysis of VAscular Tumors is a website for the EHE community. ^[7] 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.^[8]

References

^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
^ http://www.childrenshospital.org/az/Site844/mainpageS844P1.html
^ http://www.heardsupport.org/whatisHEARD.html
^ http://www.ajronline.org/content/175/6/1545.long
^ [1]
^ [2]
^ [3]
^ Living with Cancer: The Kris Carr's Story

UpToDate Contents

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1. 悪性肝腫瘍の病理 pathology of malignant liver tumors
2. 固形肝病変：鑑別診断および評価 solid liver lesions differential diagnosis and evaluation
3. 転移性軟部組織肉腫の全身治療 systemic treatment of metastatic soft tissue sarcoma
4. 悪性胸膜中皮腫の病理 pathology of malignant pleural mesothelioma
5. 肝血管腫 hepatic hemangioma

English Journal

Pleural epithelioid hemangioendothelioma: literature summary and novel case report.

Salijevska J1, Watson R1, Clifford A2, Ritchie AI2, Mauri F3, Adeboyeku D2.
Journal of clinical medicine research.J Clin Med Res.2015 Jul;7(7):566-70. doi: 10.14740/jocmr2174w. Epub 2015 May 8.
Epithelioid hemangioendothelioma (EHE) is a rare malignant cancer of vascular origin that can affect multiple and varied tissue sites. A subtype of EHE, pulmonary epithelioid hemangioendothelioma (PHE), is more unusual with only 200 reported cases. Of these, only 27 have been classified as pleural i
PMID 26015825

A novel treatment for bone lesions of multifocal epithelioid sarcoma-like hemangioendothelioma.

Davis AT1, Guo AM, Phillips NJ, Greenberg DD.
Skeletal radiology.Skeletal Radiol.2015 Jul;44(7):1013-9. doi: 10.1007/s00256-014-2089-x. Epub 2015 Jan 8.
Epithelioid sarcoma-like hemangioendothelioma (ES-H) is a rare, indolent vascular neoplasm with characteristics similar to epithelioid sarcoma. It typically affects young males who present with skin and subcutaneous lesions in the extremities. Bone lesions, occurring in approximately 20 % of patien
PMID 25564226

Japanese Journal

経気管支肺生検で診断し3年間観察しえた pulmonary epithelioid hemangioendothelioma の1例

野口真吾,矢寺和博,西田千夏,川波敏則,石本裕士,迎寛
日本呼吸器学会誌 = Annals of the Japanese Respiratory Society 1(2), 124-128, 2012-02-10
NAID 10030269185

腕頭静脈合併切除を要した縦隔原発類上皮血管内皮腫の1手術例

鈴木仁之,真栄城亮,井上健太郎,近藤智昭,高尾仁二,島本亮
日本呼吸器外科学会雑誌 26(6), 633-637, 2012
症例は54歳男性．検診にて胸部異常陰影を指摘されたため当院紹介となった．精査により右腕頭静脈への浸潤が疑われる縦隔腫瘍と診断し，手術を施行した．腫瘍は横隔神経を巻き込み右腕頭静脈と強く癒着していたため横隔神経は離断し，右腕頭静脈を含めて腫瘍を摘出した．病理組織学的検査では，血管内皮類似の異型細胞が血管腔を形成しCD34, von Willebrand factor（vWF）陽性，TTF-1, αS …
NAID 130002114649

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★リンクテーブル★

リンク元	「類上皮血管内皮腫」
拡張検索	「pulmonary epithelioid hemangioendothelioma」
関連記事	「epithelioid」

「類上皮血管内皮腫」

Epithelioid Hemangioendothelioma
	Classification and external resources
	Micrograph of an epithelioid hemangioendothelioma of the liver.
ICD-O:	M9133/
DiseasesDB	34264
MeSH	D000230