hemangioendothelioma

血管内皮腫

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/08/01 08:24:18」(JST)

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Hemangioendothelioma
Micrograph of a kaposiform hemangioendothelioma with "glomeruloid" nodules of endothelial cells.
Classification and external resources
ICD-O	9130-9133
DiseasesDB	33472
MeSH	D006390

Hemangioendothelioma is used to describe a group of vascular neoplasms that may be considered benign as well as malignant, depending on the specific group member's activity.

Classification

Hemangioendotheliomas may be classified as:

• Epithelioid sarcoma-like hemangioendothelioma is an exceedingly rare vascular tumor of intermediate grade that was first described by Steven Billings, Andrew Folpe, and Sharon Weiss in 2003. These tumors are so named because their histologic appearance is very similar to that of epithelioid sarcoma, a more malignant tumor with which they are commonly mistaken.
• Composite hemangioendothelioma is a low-grade angiosarcoma typically occurring in adults, although it has been described in infancy.
• Spindle-cell hemangioendothelioma) is a vascular tumor that was first described in 1986 by Sharon Weiss, M.D., and commonly presents in a child or young adult who develops blue nodules of firm consistency on a distal extremity. These tumors were reclassified by Dr. Weiss in 1996 as "spindle cell hemangioma", rather than hemangioendothelioma, due to the excellent prognosis observed in a group of 78 patients.

• Retiform hemangioendothelioma (also known as a "Hobnail hemangioendothelioma") is a low-grade angiosarcoma, first described in 1994, presenting as a slow-growing exophytic mass, dermal plaque, or subcutaneous nodule.

• Kaposiform hemangioendothelioma (also known as "Infantile kaposiform hemangioendothelioma") is an uncommon vascular tumor, first described by Niedt, Greco, et al (Hemangioma with Kaposi's sarcoma-like features: report of two cases.(Niedt GW, Greco MA, Wieczorek R, Blanc WA, Knowles DM 2nd. that affects infants and young children, with rare cases having also been reported in adults.Pediatr Pathol. 1989;9(5):567-75.)

• Endovascular papillary angioendothelioma, also known as "Dabska tumor", "papillary intralymphatic angioendothelioma" (PILA), "Dabska-type hemangioendothelioma", "hobnail hemangioendothelioma", and "malignant endovascular papillary angioendothelioma", is a rare low-grade angiosarcoma of lymphatic channels. Approximately 30 such tumors have been described in the medical literature. Although included in the World Health Organization tumor classification, there is uncertainty as to whether EPA is a distinct entity or a heterogenous group of tumours. The lesion usually presents as a slow-growing tumor of the skin and subcutaneous tissues of the head, neck, or extremity, of infants or young children. However, EPA has involved the testicle, deep muscle tissue as a neoplastic transformation of a larger existing benign cavernous hemangioma, bone and spleen, and has been found in adults. Some reports indicate a good prognosis but metastasis is occasionally seen.

• Infantile hemangioendothelioma is a rare benign vascular tumour arising from mesenchymal tissue and is usually located in the liver. It often presents in infancy with cardiac failure because of extensive arteriovenous shunting within the lesion. It is the third most common liver tumor in children, the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the first 6 months of life. These hemangioendotheliomas have 2 growth phases: an initial rapid growth phase, which is followed by a period of spontaneous involution (usually within the first 12 to 18 months of life). Detection of the hemangioendothelioma within the first 6 months of life is attributed to the initial rapid growth during this time; however, the tumor has been detected with fetal ultrasonography. Histopathologically, there are 2 types of hepatic hemangioendotheliomas:
  • Type I: Hemagioendotheliomas of this type have multiple vascular channels that are formed by an immature endothelial lining with stromal separation from bile ductules.
  • Type II: These hemangioendotheliomas have an appearance that is more disorganized and hypercellular, and there are no bile ductules.

In children, distinguishing between a primary malignant liver tumor (hepatoblastoma) and a benign primary hepatic lesion (hemangioendothelioma) is crucial. The absence of urinary catecholamines supports the diagnosis of hemangioendothelioma. In patients with hemangioendotheliomas, elevations in α₁-fetoprotein levels are milder than those found in patients with hepatoblastomas. Infantile hepatic hemangioendothelioma is strongly suggested by the presence of a vascular lesion on imaging studies. A complex, heterogeneous mass is often seen on ultrasonograms; a complex tumor that lacks central enhancement can be see on CT scans; and the vascular nature of the lesion along with dilation of the aorta proximal to the origin of the celiac artery and a decrease in the diameter distally, indicating significant shunting, is seen on angiograms. Because most hemangioendotheliomas in infants sponanteously involute and regress within the first 12 to 18 months of life, asymptomatic lesions are generally managed conservatively. Infants who have severe anemia and/or thrombocytopenia can be given blood products; for those who have cardiac failure, diuretics and digoxin are often given. To stop further growth and to speed regression of lesions in infants with more significant clinical sequelae, treatment with corticosteroids or interferon-α-2a is administered. To slow the growth of tumors that are rapidly enlarging, chemotherapy and radiation therapy have been used. Surgical resection, partial hepatectomy, and embolization of afferent vessels should be considered for severe cases.

• Low power photomicrograph of an endovascular papillary angioendothelioma showing papillae with hyalinized cores

• High power view showing a vascular tumor with cuboidal endothelium lining the vessels. Few entrapped seminiferous tubules are also noted (arrow).

• Characteristic budding, hobnail-like endothelial cells visible.

Signs and symptoms

They have been described as masses that fall between a hemangioma and angiosarcoma. They are vascular tumors that commonly present with an enlarging mass and most commonly involve the lungs, liver, and musculoskeletal system, although many other body sites have been reported, including the head and neck, intestines, lymph nodes, pleura, retroperitoneum, stomach.

Treatment

Treatment is varied and depends on the site and extent of tumor involvement, site(s) of metastasis, and specific individual factors. Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens.

See also

References

External links

• Hemgioendothelioma Support Group
• Maxillofacialcenter
• H.E.A.R.D. Hemangioendothelioma, Epithelioid Hemangioendothelioma And Related vascular Disorders Support Group and International H.E.A.R.D. Registry
• Infantile Hemangioendothelioma Support Group

UpToDate Contents

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• 1. 房状血管腫、カポジ肉腫様血管腫、およびKasabach‐Merritt現象 tufted angioma kaposiform hemangioendothelioma and the kasabach merritt phenomenon
• 2. 肝血管腫 hepatic hemangioma
• 3. 先天性血管腫：急速退縮性先天性血管腫（RICH）および非退縮性先天性血管腫（NICH） congenital hemangiomas rapidly involuting congenital hemangioma rich and noninvoluting congenital hemangioma nich
• 4. 肝肥大：鑑別診断および評価 hepatomegaly differential diagnosis and evaluation
• 5. 固形肝病変：鑑別診断および評価 solid liver lesions differential diagnosis and evaluation

English Journal

• Real-time contrast-enhanced ultrasound in diagnosing of focal spleen lesions.

• Yu X, Yu J, Liang P, Liu F.SourceDepartment of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China.
• European journal of radiology.Eur J Radiol.2012 Mar;81(3):430-6. Epub 2011 Jan 26.
• OBJECTIVE: To investigate the features of focal spleen lesions (FSLs) on contrast-enhanced ultrasound (CEUS) imaging.MATERIALS AND METHODS: CEUS with a blot injection of SonoVue was performed in 48 patients with 75 FSLs (median diameter 2.6cm) and their perfusion characteristics were analyzed by usi
• PMID 21273021

• Right Portal Vein Ligation Combined With In Situ Splitting Induces Rapid Left Lateral Liver Lobe Hypertrophy Enabling 2-Staged Extended Right Hepatic Resection in Small-for-Size Settings.

• Schnitzbauer AA, Lang SA, Goessmann H, Nadalin S, Baumgart J, Farkas SA, Fichtner-Feigl S, Lorf T, Goralcyk A, Hörbelt R, Kroemer A, Loss M, Rümmele P, Scherer MN, Padberg W, Königsrainer A, Lang H, Obed A, Schlitt HJ.Source*Departments of Surgery and †Radiology and ‡Institute for Pathology, University Hospital Regensburg, Regensburg; §Eberhard Karls University of Tübingen, Department of General Visceral and Transplant Surgery, Tübingen; ‖Johannes-Gutenberg University Mainz, Department of General and Abdominal Surgery, Mainz; ¶Georg-August Univeristy Göttingen, Department of General and Visceral Surgery, Göttingen; and #Justus Liebig University of Giessen, Department of Thoracic and General Surgery, Giessen, Germany.
• Annals of surgery.Ann Surg.2012 Feb 10. [Epub ahead of print]
• OBJECTIVE: To evaluate a new 2-step technique for obtaining adequate but short-term parenchymal hypertrophy in oncologic patients requiring extended right hepatic resection with limited functional reserve.BACKGROUND: Patients presenting with primary or metastatic liver tumors often face the dilemma
• PMID 22330038

• Propranolol As A First-Line Treatment For Diffuse Infantile Hepatic Hemangioendothelioma: Case Report And Review Of Literature.

• Avagyan S, Klein M, Kerkar N, Demattia A, Blei F, Lee S, Rosenberg HK, Arnon R.Source*Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY †Division of Pediatric Endocrinology and Diabetes, Mount Sinai Hospital, New York, NY ‡Division of Pediatrics & Recanati-Miller Transplant Institute, Mount Sinai Hospital, New York, NY §Westside Pediatrics, New York, NY ||Vascular Birthmark Institute of NY, Roosevelt Hospital, New York, NY ¶Department of Radiology, Division of Pediatric Radiology, Mount Sinai Hospital, New York, NY.
• Journal of pediatric gastroenterology and nutrition.J Pediatr Gastroenterol Nutr.2012 Feb 10. [Epub ahead of print]
• PMID 22331019

Japanese Journal

• Pulmonary epithelioid hemangioendothelioma

• MIZUNO Yoshimasa,IWATA Hisashi,SHIRAHASHI Koyo,HIROSE Yoshinobu,TAKEMURA Hirofumi
• General thoracic and cardiovascular surgery 59(4), 297-300, 2011-04-10
• NAID 10029019187

• 原発性肺癌が疑われた高齢者肺類上皮血管内皮腫の1例

• 坪井 光弘,古澤 潔,環 正文,三浦 一真
• 日本呼吸器外科学会雑誌 = The journal of the Japanese Association for Chest Surgery 25(2), 229-234, 2011-03-15
• 肺類上皮血管内皮腫（PEH）は稀な肺腫瘍の1つであり，かつて血管内細気管支肺胞上皮腫瘍と呼称されていた疾患である．PEHの多くは，画像上境界明瞭な多発小結節陰影を示すとされているが，今回我々は原発性肺癌と類似する画像所見を呈したPEH症例を経験したので，文献的考察を加えて報告する．症例は78歳，女性．咳嗽を主訴に近医を受診した．胸部CTで左S1+2に石灰化を伴う境界明瞭な15mm大の腫瘤影，右S9 …
• NAID 10027955473

• 臨床室 環指基節骨に発生した骨血管内皮腫の1例

• 佐藤 央,吉田 雅博,中島 浩敦 [他]
• 整形外科 62(11), 1188-1191, 2011-10
• NAID 40019002650

Related Links

• Hemangioendothelioma - Wikipedia, the free encyclopedia

Hemangioendothelioma is used to describe a group of vascular neoplasms that may be considered benign or malignant in their activity. Contents. 1 Presentation; 2 Treatment; 3 Types of hemangioendotheliomas; 4 See also; 5 References ...

• Hemangioendothelioma - The Doctor's Doctor

28 Jan 2008 ... Features information about this rare vascular tumor. Includes differentials, prognosis, and treatments.

Related Pictures

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★リンクテーブル★

リンク元	「血管内皮腫」「haemangioendothelioma」
拡張検索	「malignant hemangioendothelioma」「spindle-cell hemangioendothelioma」

「血管内皮腫」

　　[★]

英

hemangioendothelioma、haemangioendothelioma, angioendothelioma

「haemangioendothelioma」

　　[★] 血管内皮腫、hemangioendothelioma

「malignant hemangioendothelioma」

　　[★] 血管肉腫。悪性血管内皮腫

「spindle-cell hemangioendothelioma」

　　[★] 紡錘細胞血管内皮腫