Chapter 364. Vascular Tumors and Malformations

Ilona J. Frieden; Sheilagh M. Maguiness

The International Society of the Study of Vascular Anomalies (ISSVA) divided vascular anomalies into malformations or tumors, and the vast majority of vascular lesions can be classified using this schema (Table 364-1). Vascular tumors and malformations can usually be distinguished from one another based on clinical appearance and growth characteristics (Table 364-2). If diagnosis is unclear observation over time, imaging studies or even biopsy may be required.

Table 364-1. Updated International Society of the Study of Vascular Anomalies Classification of Vascular Anomalies

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Table 364-1. Updated International Society of the Study of Vascular Anomalies Classification of Vascular Anomalies

Vascular Tumors

Vascular Malformations

Infantile hemangiomas

Slow-flow malformations

Congenital hemangiomas

Capillary malformation (CM)

Rapidly involuting congenital hemangioma

Port wine stain

Noninvoluting congenital hemangioma

Telangiectasia

Tufted angioma

Angiokeratoma

Kaposiform hemangioendothelioma

Venous malformation (VM)

Spindle cell hemangioendothelioma

Common sporadic VM

Other, rare hemangioendotheliomas (epithelioid, composite, retiform, polymorphous, Dabska tumor, lymphangioendotheliomatosis, etc)

Bean syndrome

Familial cutaneous and mucosal VM (VMCM)

Glomuvenous malformation (GVM)

Dermatologic acquired vascular tumors

Lymphatic malformation (LM)

Pyogenic granuloma

Macrocystic

Targetoid hemangioma

Microcystic

Glomeruloid hemangioma

Fast-flow malformations

Microvenular hemangioma

Arterial malformation (AM)

Arteriovenous fistula (AVF)

Arteriovenous malformation (AVM)

Complex-combined vascular malformations

CVM, CLM, LVM, CLVM, AVM-LM, CM-AVM

AVM-LM, arteriovenous and lymphatic malformation, CLM, capillary lymphatic malformation; CLVM, capillary lymphatic venous malformation; CM-AVM, capillary and arteriovenous malformation; CVM, capillary venous malformation; LVM, lymphatic venous malformation.

Table 364-2. Major Differences between Vascular Tumors and Malformations

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Table 364-2. Major Differences between Vascular Tumors and Malformations

Tumors

Malformations

Clinical

Present shortly after birth, usually in infancy

Present at birth with slow expansion (not proliferation) over time

May have premonitory/precursor lesion: vasoconstriction, telangiectasia

Sex prevalence

Infantile hemangiomas (IHs) have female predilection; female-to-male ratio of 3:1

No gender predilection

Other vascular tumors (kaposiform hemangioendothelioma [KHE] and tufted angioma [TA]); female-to-male ratio of1:1

Natural history

Rapid proliferation after birth in IH, followed by slow involution over years

Persist through life with commensurate growth or progressive worsening due to vessel dilatation

Slower proliferation of other vascular tumors

Arteriovenous malformation and venous malformation may flare with trauma, puberty, menses, or pregnancy

Pathology

IH: endothelial cell hyperplasia, mast cells during proliferation, fibrofatty residua after involution

Dependent on type, but usually comprised of increased numbers of the dominant vessel type

KHE/TA: spindle cell tumor comprised of lymphatic and endothelial cell lines, aggressive local proliferation

Vascular ectasia or irregular vascular channels can often be present

Immunohistochemistry

GLUT-1 positivity in IHs but not in other vascular tumors

GLUT-1 negative

Clinical Manifestations and Subtypes

The most common vascular tumor is infantile hemangioma (IH), present in approximately 4% of infants and more common in females, Caucasians, preterm infants (particularly in those weighing < 1500 g) and infants of multiple gestations.2 A key clinical feature is whether hemangiomas are localized (eg, arising as a ...

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