endovascular papillary angioendothelioma

血管内乳頭状血管内皮腫

WordNet

1. (botany) a tiny outgrowth on the surface of a petal or leaf
2. a small nipple-shaped protuberance concerned with taste, touch, or smell; "the papillae of the tongue"
3. a small projection of tissue at the base of a hair or tooth or feather
4. of or relating to or resembling papilla (同)papillose

PrepTutorEJDIC

1. 乳頭;(毛根・歯根などにある)微小乳頭状突起物

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/10/09 23:41:01」(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, heel, 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. 胸部大動脈の動脈瘤修復 endovascular repair of the thoracic aorta
• 2. 血管内腹部大動脈修復術の合併症 complications of endovascular abdominal aortic repair
• 3. Endovascular methods for aortic control in trauma
• 4. 膝窩動脈瘤の手術および血管内修復 surgical and endovascular repair of popliteal artery aneurysm
• 5. 胸部大動脈の鈍的損傷の手術および血管内修復 surgical and endovascular repair of blunt thoracic aortic injury

English Journal

• CASE REPORT Successful Treatment of a Rare Massive Dabska Tumor.

• Eng OS, Borah GL, Gannon CJ.Author information Divisions of General Surgery, Rutgers-Robert Wood Johnson Medical School, New Brunswick.AbstractObjective: The Dabska tumor is a rare low-grade angiosarcoma first described in 1969 by Maria Dabska. Approximately 30 cases of varying presentations have been reported since its initial description. Methods: We describe a case of a 23-year-old woman presenting with a massive recurrent left flank hemangioendothelioma, at final resection diagnosed to be an endovascular papillary angioendothelioma (Dabska tumor). The sheer size of the tumor necessitated reconstructive surgery. Results: Successful abdominal reconstruction after radical resection of a Dabska tumor was achieved using local fasciocutaneous-type flaps. Conclusion: To our knowledge, this is the first case report describing reconstructive surgery following resection of an abdominal Dabska tumor.
• Eplasty.Eplasty.2013 Sep 10;13:e46.
• Objective: The Dabska tumor is a rare low-grade angiosarcoma first described in 1969 by Maria Dabska. Approximately 30 cases of varying presentations have been reported since its initial description. Methods: We describe a case of a 23-year-old woman presenting with a massive recurrent left flank he
• PMID 24058717

• An unusual mole: an adult case of Dabska tumour.

• Bernić A, Novosel I, Krizanac S.Author information Dr. Ivo Pedisić, General Hospital, Department of ENT Surgery, Sisak, Croatia. abernic@gmail.comAbstractIn 1969 Dabska and her colleagues described for the first time this rare malignant tumour, also later known as a malignant endovascular papillary angioendothelioma of childhood. Overall, depending amongst other factors on its location, it is thought to have a generally favourable prognosis and a wide local excision seems to be the treatment of choice. We here present a very rare and unusual case of a 63 year old woman with a 20 year history of slow-growing right buccal dermatological lesion which resembled a common mole. The histopathological diagnosis of Dabska Tumour was made following the hematoxylin and eosin (H&E) biopsy. The analysis revealed multiple delicate interconnecting vascular channels with papillary plugs, some of which containing hyalinized core, projecting into the lumen lined by atypical plumped endothelial cells.
• Collegium antropologicum.Coll Antropol.2012 Nov;36 Suppl 2:171-2.
• In 1969 Dabska and her colleagues described for the first time this rare malignant tumour, also later known as a malignant endovascular papillary angioendothelioma of childhood. Overall, depending amongst other factors on its location, it is thought to have a generally favourable prognosis and a wid
• PMID 23397780

• Fine needle aspiration cytology of malignant endovascular papillary angioendothelioma.

• Banik T, Sinha AK, Rai MK, Prasad J.Author information Department of Pathology, Rajendra Institute of Medical Sciences, Ranchi, India. tbanik.rnj@gmail.comAbstractHere we described a rare case of malignant endovascular papillary angioendothelioma (Dabska tumor) in an adult female. On fine needle aspiration, the smear showed many small clusters of tumor cells with rosettoid arrangement along with papillary fragments with fibrovascular core and hobnail like arrangement of the cells. Histopathological examination revealed a vascular tumor in the form of papillary projection into the vascular lumina, lined by atypical endothelial cells.
• Diagnostic cytopathology.Diagn Cytopathol.2011 Jul;39(7):514-6. doi: 10.1002/dc.21475. Epub 2010 Nov 2.
• Here we described a rare case of malignant endovascular papillary angioendothelioma (Dabska tumor) in an adult female. On fine needle aspiration, the smear showed many small clusters of tumor cells with rosettoid arrangement along with papillary fragments with fibrovascular core and hobnail like arr
• PMID 21695802

Japanese Journal

• Endovascular papillary angioendothelioma (Dabska tumor)

• FUKUNAGA Masaharu
• Pathology international 48(10), 840-841, 1998-10
• NAID 10008343662

• Endovascular papillary angioendothelioma (Dabska tumor) in an elderly woman

• Yamada Akihiko,Uematsu Kunio,Yasoshima Hitoshi [他],SAKURAI Kazunari,HORI Kazutoshi,OHYA Munehiko,OHKUBO Eriko,OGASAWARA Hiroshi
• Pathology international 48(2), 164-167, 1998-02
• NAID 10008340899

• Endovascular Papillary Angioendothelioma (Dabska Tumour) : Like Tumour Associated with Lymphoedema

• FUKUNAGA Masaharu,SHISHIKURA Yuri,ISHIKAWA Eisei,ENDO Yasuhiko,USHIGOME Shinichiro
• 日本整形外科學會雜誌 = The Journal of the Japanese Orthopaedic Association 70(6), S1079, 1996-06-25
• NAID 10016492507

Related Links

• Endovascular papillary angioendothelioma (Dabska tumor ...

1. J Pediatr Surg. 2011 Jan;46(1):e25-8. doi: 10.1016/j.jpedsurg.2010.09.046. Endovascular papillary angioendothelioma (Dabska tumor): underrecognized malignant tumor in childhood. Neves RI(1), Stevenson J, Hancey ...

• Endovascular papillary angioendothelioma (Dabska tumor ...

Endovascular papillary angioendothelioma, or Dabska tumor, was first presented in 1969 by Maria Dabska where she presented cases occurring in 6 children. The tu ... At his 30-month follow-up visit, the patient has no cervical ...

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★リンクテーブル★

リンク元	「血管内乳頭状血管内皮腫」
関連記事	「endovascular」「papillary」「papilla」

「血管内乳頭状血管内皮腫」

　　[★]

英

endovascular papillary angioendothelioma

同

ダブスカ腫瘍 Dabska tumor

• 血管内乳頭状血管内皮腫は、1969年Dabskaによって最初に定義されたまれな血管腫。
• 小児ないし若年成人の皮膚または皮下に生じる
• 顕微鏡的には血管の吻合から構成され、それらのうちのいくらかは、時に乳頭状突起あるいは腎糸球体に似た房のような構造からなる。
• 領域リンパ節への転移例で報告されている例があるが、長期予後は一般によい。

参考

• [原著]Malignant endovascular papillary angioendothelioma of the skin in childhood. Clinicopathologic study of 6 cases.

• Dabska M.
• Cancer.Cancer.1969 Sep;24(3):503-10.
• PMID 5343389

http://emedicine.medscape.com/article/1112873-overview

「endovascular」

　　[★]

• adj.

• 血管内の

関

endovascularly、intravascular、intravascularly

「papillary」

　　[★]

• adj.

• 乳頭の、乳頭部の

関

nipple、papilla、papillae

　

「papilla」

　　[★]

• n.

• 乳頭