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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 TumorsVascular Malformations
Infantile hemangiomasSlow-flow malformations
Congenital hemangiomasCapillary malformation (CM)
Rapidly involuting congenital hemangiomaPort wine stain
Noninvoluting congenital hemangiomaTelangiectasia
Tufted angioma Angiokeratoma
Kaposiform hemangioendothelioma Venous malformation (VM)
Spindle cell hemangioendotheliomaCommon 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
TumorsMalformations
Clinical Present shortly after birth, usually in infancyPresent at birth with slow expansion (not proliferation) over time
May have premonitory/precursor lesion: vasoconstriction, telangiectasia
Sex prevalenceInfantile hemangiomas (IHs) have female predilection; female-to-male ratio of 3:1No gender predilection
Other vascular tumors (kaposiform hemangioendothelioma [KHE] and tufted angioma [TA]); female-to-male ratio of1:1
Natural historyRapid proliferation after birth in IH, followed by slow involution over yearsPersist through life with commensurate growth or progressive worsening due to vessel dilatation
Slower proliferation of other vascular tumorsArteriovenous malformation and venous malformation may flare with trauma, puberty, menses, or pregnancy
PathologyIH: endothelial cell hyperplasia, mast cells during proliferation, fibrofatty residua after involutionDependent 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 proliferationVascular ectasia or irregular vascular channels can often be present
ImmunohistochemistryGLUT-1 positivity in IHs but not in other vascular tumorsGLUT-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 nodule or small area ...

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