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Vascular anomalies: Description, classification and nomenclature

It is hoped that the utilization of archaic and inaccurate terminology will decline with improved understanding of the fundamental differences between the various vascular anomalies and with more widespread familiarity with the ISSVA classification scheme. In addition to the stubborn persistence of the term “cavernous hemangioma” throughout the literature and within everyday practice, other terms such as “port wine stain” and “lymphangioma” remain pervasive. The suffix “oma” is typically associated with tumors, and is appropriately reserved for the vascular tumors such as hemangioma and hemangioendothelioma.

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 Vascular malformations and tumors

Vascular malformations and tumors are a heterogeneous group of lesions that may affect the arterial, capillary, venous or lymphatic system or any combination thereof. They encompass a bewildering range of lesions, syndromes, and masses ranging from the relatively common (e.g. infantile hemangioma and arteriovenous malformations (AVM)) to the rare (e.g. Dabska tumor and Bean syndrome).

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The abnormal growth of blood vessels are called vascular tumors. They may look like a birthmark or infantile hemangioma. Examples of other vascular tumors include kaposiform hemangioendothelioma and tufted angioma.

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Both vascular tumors and malformations may occur anywhere on the body. In brief, hemangiomas are vascular tumors that are rarely apparent at birth, grow rapidly during the first 6 months of life, involute with time and do not necessarily infiltrate but can sometimes be destructive. Vascular malformations are irregular vascular networks defined by their particular blood vessel type. In contrast to hemangiomas, they are present at birth, slow growing, infiltrative, and destructive. Almost all vascular malformations and nearly 40% of hemangiomas eventually require intervention.

Malignant vascular tumors—an update

Although benign hemangiomas are among the most common diagnoses amid connective tissue tumors, sarcomas showing endothelial differentiation (ie, angiosarcoma and epithelioid hemangioendothelioma) represent under 1% of all sarcoma diagnoses, and thus it is likely that fewer than 500 people in the United States are affected each year.

Vascular Anomalies: Hemangiomas and Beyond—Part 1, Fast-Flow Lesions

The purpose of this study was to review the medical literature and the current classification of vascular anomalies to clarify common misconceptions and provide guidance for imaging and treatment. In this first article of a two-part series, we focus on the fast-flow vascular anomalies.

Vascular Malformations & Tumors - Pediatric

Treatment of vascular malformations depends on the size and location and the extent to which it disturbs the child. Treatment options vary from trying to stop the blood flow to the malformation to surgically removing it.

Vascular Malformations and Hemangiomas: A Practical Approach

Vascular malformations and hemangiomas can cause significant morbidity and even mortality in both children and adults. For a number of reasons, physicians often confuse these lesions. The nomenclature for classifying these lesions is often used interchangeably and inappropriately. Clinically significant malformations are uncommon, and patients with these malformations are rarely encountered in primary medical facilities, rendering most physicians inexperienced in providing optimal care.

Vascular Tumors

Vascular anomalies can be broken down into two major categories: tumors and malformations. These clinical entities are extremely different, but often confused, as evidenced by the history and medical literature. A thorough medical history and physical exam is essential when it comes to defining any vascular anomaly. There are several different vascular tumors, but infantile hemangiomas are the most common. Others tumors that will be discussed include tuft angiomas, pyogenic granulomas, angiosarcomas, and kaposiform hemangioendotheliomas. Management of vascular tumors depends on their medical history, diagnosis, and most importantly their location.

Venous Malformations

Venous malformations, formerly referred to as hemangiomas, are the most common vascular soft tissue abnormality and comprise 7% of benign soft tissue tumors. The terminology for vascular anomalies in the medical literature is confusing and inconsistent.

Resources

Radiology Key

In 1982, Mulliken and Glowacki proposed a useful classification for vascular anomalies, which was then adopted by the International Society for the Study of Vascular Anomalies (ISSVA) in 1996 and updated in 2014. This classification is based on the cellular turnover, histologic features, natural history, and physical findings of the vascular anomalies.