Vascular tumor

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For information on vascular anomalies, click here.

For information on benign vascular tumors, Click here.

Vascular Tumor

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Overview

Classification

Benign Vascular tumor
Locally aggressive or borderline vascular tumors
Malignant vascular tumors

Benign Vascular Tumor Home Page

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2], Anmol Pitliya, M.B.B.S. M.D.[3]

Overview

Vascular tumors are growths (benign or malignant) formed from blood vessels; for example, hemangioma, hemangioendothelioma, Kaposi sarcoma, angiosarcoma. International Society for the Study of Vascular Anomalies (ISSVA) has classified vascular tumors into three main categories depending on their clinical and histological behavior. Some lesions related clinically and/or histologically to vascular tumors have been described in provisionally unclassified vascular anomalies and related lesions.

Classification

 
 
 
 
 
 
 
 
 
 
 
 
 
 
Vascular tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Benign
 
 
 
 
 
Locally aggressive or borderline
 
 
 
 
 
Malignant
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Infantile hemangioma / Hemangioma of infancy
 
 
 
 
 
 
Kaposiform hemangioendothelioma
 
 
 
 
 
 
Angiosarcoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Congenital hemangioma
 
 
 
 
 
 
Retiform hemangioendothelioma
 
 
 
 
 
 
Epithelioid hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Tufted angioma
 
 
 
 
 
 
Papillary intralymphatic angioendothelioma (PILA), Dabska tumor
 
 
 
 
 
 
Others
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Spindle-cell hemangioma
 
 
 
 
 
 
Composite hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Epithelioid hemangioma
 
 
 
 
 
 
Pseudomyogenic hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pyogenic granuloma (also known as lobular capillary hemangioma)
 
 
 
 
 
 
Polymorphous hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Others
Microvenular hemangioma
Anastomosing hemangioma
Glomeruloid hemangioma
Papillary hemangioma
Intravascular papillary endothelial hyperplasia
Cutaneous epithelioid angiomatous nodule
Acquired elastotic hemangioma
Littoral cell hemangioma of the spleen
 
 
 
 
 
 
Hemangioendothelioma not otherwise specified
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
'
Related lesions
Eccrine angiomatous hamartoma
Reactive angioendotheliomatosis
Bacillary angiomatosis
 
 
 
 
 
 
Kaposi sarcoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Others
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 


*congenital hemangioma (rapidly involuting type) and tufted angioma may be associated with thrombocytopenia and/or consumptive coagulopathy in some cases. Many experts consider tufted angioma and kaposiform hemangioendothelioma to be part of a spectrum rather than distinct entities
Adapted from International Society for the Study of Vascular Anomalies[1]

Benign vascular tumors

Benign vascular tumors, are benign growths formed from blood vessels; such as, hemangioma, hemangioendothelioma, Kaposi sarcoma. They exhibit a wide range of clinical manifestations, and may occur as isolated lesions or may occur as manifestation of multi-system syndromes and diseases. Their diagnosis and management depends on their clinical manifestations and coexistent anomalies. International Society for the Study of Vascular Anomalies (ISSVA) has classified these lesions into benign vascular tumors and related lesions.

For more information on benign vascular tumors, click here.

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

For more information on Kaposiform hemangioendothelioma, click here.

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

  • First described in 1969 by Dabska, this rare vascular neoplasm generally occurs in soft tissues but can also occur in bone. They usually appear as painless inflammatory irregular or nodular lesions below the skin surface.
  • The distinctive feature on histopathology is the intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration.[7]
  • They are locally aggressive but rarely metastasize. Locally recurrence after surgery is very common.
  • Diagnostic studies may include histopathological studies, fine needle aspiration, MRI, and CT scan.[8]
  • Wide local excision is the treatment of choice. However any combination of steroids, chemotherapy, radiation therapy, and invasive procedures can be used to treat this tumor.

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorphous hemangioendothelioma

Kaposi sarcoma

  • An AIDS-associated vascular malignancy that usually presents as mucocutaneous lesions but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.[19]
  • There are three known variants:
    • One variant occurs spontaneously in Jewish and Italian males in Europe and the United States.
    • Another more aggressive variant is endemic in young children is endemic in Africa.
    • A third form occurs in about 0.04% of kidney transplant patients. There is also a high incidence in AIDS patients. HHV-8 is the suspected cause.[20]

For more information on Kaposi sarcoma, Click here

Malignant vascular tumors

Angiosarcoma

For more information about angiosarcoma, click here.

Epithelioid hemangioendothelioma

References

  1. "Classification | International Society for the Study of Vascular Anomalies".
  2. Hu PA, Zhou ZR (June 2018). "Clinical and imaging features of Kaposiform Hemangioendothelioma". Br J Radiol. 91 (1086): 20170798. doi:10.1259/bjr.20170798. PMID 29536769.
  3. 3.0 3.1 "Letter: Prevention of coronary heart-disease". Lancet. 2 (7980): 313–4. August 1976. PMID 59885.
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  7. © 1999 Lippincott Williams & Wilkins, Inc.
  8. https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/
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  10. Shang Leen SL, Fisher C, Thway K (July 2015). "Composite hemangioendothelioma: clinical and histologic features of an enigmatic entity". Adv Anat Pathol. 22 (4): 254–9. doi:10.1097/PAP.0000000000000079. PMID 26050262.
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  14. Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V (2015). "Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma". Clin Sarcoma Res. 5: 22. doi:10.1186/s13569-015-0037-8. PMC 4615364. PMID 26500758.
  15. Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K (February 2018). "Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma". Pediatr Blood Cancer. 65 (2). doi:10.1002/pbc.26781. PMID 28843050.
  16. 16.0 16.1 El Hussein S, Omarzai Y (May 2017). "Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature". Int. J. Surg. Pathol. 25 (3): 266–270. doi:10.1177/1066896916679517. PMID 27913780.
  17. Tadros M, Rizk SS, Opher E, Thompson LD (June 2003). "Polymorphous hemangioendothelioma of the neck". Ann Diagn Pathol. 7 (3): 165–8. PMID 12808568.
  18. Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L (2009). "Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature". Tumori. 95 (1): 94–7. PMID 19366064.
  19. Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V (2018). "Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations". IDCases. 14: e00420. doi:10.1016/j.idcr.2018.e00420. PMC 6125769. PMID 30191128.
  20. Piccolo V, Russo T, Moscarella E, Brancaccio G, Alfano R, Argenziano G (October 2018). "Dermatoscopy of Vascular Lesions". Dermatol Clin. 36 (4): 389–395. doi:10.1016/j.det.2018.05.006. PMID 30201148.
  21. Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML (September 2018). "Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein". Ann Vasc Surg. doi:10.1016/j.avsg.2018.06.034. PMID 30217704.
  22. Udager AM, Ishikawa MK, Lucas DR, McHugh JB, Patel RM (December 2016). "MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience". Pathology. 48 (7): 697–704. doi:10.1016/j.pathol.2016.08.007. PMID 27780597.
  23. Priyakumari T, Chandar R, Jayasree K, Ramachandran K (September 2018). "Pediatric Primary Ovarian Angiosarcoma: From rarity to a realization". J Pediatr Adolesc Gynecol. doi:10.1016/j.jpag.2018.08.008. PMID 30179666.
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