Vascular tumor

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For information on vascular anomalies, click here.

For information on benign vascular tumors, Click here.

Vascular Tumor




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]


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.


Vascular tumors
Locally aggressive or borderline
Infantile hemangioma / Hemangioma of infancy
Kaposiform hemangioendothelioma
Congenital hemangioma
Retiform hemangioendothelioma
Epithelioid hemangioendothelioma
Tufted angioma
Papillary intralymphatic angioendothelioma (PILA), Dabska tumor
Spindle-cell hemangioma
Composite hemangioendothelioma
Epithelioid hemangioma
Pseudomyogenic hemangioendothelioma
Pyogenic granuloma (also known as lobular capillary hemangioma)
Polymorphous hemangioendothelioma
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

*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


For more information about angiosarcoma, click here.

Epithelioid hemangioendothelioma


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