2 Congenital Vascular Tumors


2 Congenital Vascular Tumors

Francine Blei

2.1 Introduction

Congenital vascular masses may be benign or malignant and are reviewed in this chapter. Vascular lesions that are present at birth may also be detected antenatally via routine ultrasound examination, with further assessment by fetal magnetic resonance imaging if considered necessary. Serial monitoring is necessary to maintain a safe pregnancy and to evaluate flow characteristics, fetal growth or compromise, and size of the abnormality, all of which contribute to rational decision making regarding the timing and type of delivery. In some cases, the affected fetus has been treated medically by transplacental passage of medication administered to the mother (e.g., corticosteroids for high-flow hepatic hemangiomatosis, digoxin for a high-flow congenital hemangioma of the nape ( Fig. 2.1 ).

Fig. 2.1 Rapidly involuting congenital hemangioma (RICH) of the scalp. (a,b) RICH of nape was diagnosed prenatally as highly vascular mass at birth. (c,d) Patient 2 weeks later (untreated). Note improvement in color. Lesion was removed surgically because of its large size and difficulty moving the neck.

Whereas “typical” hemangiomas are not apparent at birth and proliferate postnally, rapidly involuting congenital hemangioma (RICH) lesions are present prenatally and gradually involute after birth ( Fig. 2.2 ). 1 ,​ 2 ,​ 3 ,​ 4 ,​ 5 The clinical features of the subtypes of hemangiomas are summarized in Table 2.1 . Some RICH lesions are very high flow, even in utero, and can cause a high-output state and fetal or infant distress. Transient thrombocytopenia may also be present postnatally and self-resolves. 6 ,​ 7 ,​ 8 Many RICH lesions have a circumferential pale “halo.” Most do not require therapy and improve naturally; however, the subgroup with a symptomatic high-flow component may benefit from embolization. Lesions that ulcerate require medical and local attention. RICHs are often on the nape, scalp, or extremities and have characteristic radiologic findings, with less well-defined margins than typical hemangiomas and high flow, 9 ,​ 10 ,​ 11 ,​ 12 and they are benign histologically, typically with plump lobular endothelial cells, which are typically GLUT-1 negative.

Fig. 2.2 The three types of hemangiomas: the common, typical infantile hemangiomas, rapidly involuting congenital hemangiomas (RICHs), and the rare noninvoluting congenital hemangiomas (NICHs). (Used with permission from Mulliken JB, Enjolras O. Congenital hemangiomas and infantile hemangioma: missing links. J Am Acad Dermatol. 2004;50:875–882.)

Table 2.1 Features of different types of proliferative vascular lesions

Typical hemangioma of infancy (HOI)

Rapidly involuting congenital hemangioma (RICH)

Noninvoluting congenital hemangioma (NICH)

Kaposiform hemangioendothelioma


Tufted angioma


Multifocal lymphangioendotheliomatosis (MLT)

Time of presentation






Newborn, early infancy

Clinical appearance

Initially macular pale area, rapidly proliferates

± ulceration

Large vascular well-defined mass

circumferential halo

May have high flow with cardiac failure

± ulceration

Plaquelike, with overlying telangiectasia


pale central or periphery

~ 5 cm diameter

Boggy, edematous purpuric mass

± Hyperhidrosis of overlying skin

Blue, deep red, purple or brownish erythematous leathery plaque or nodule

± Hyperhidrosis of overlying skin

Numerous red-brown skin plaques with gastrointestinal involvement, severe GI bleeding



Variable flow depending on stage


Less defined than HOI

± High-flow

± calcifications


Less defined than HOI

± High flow

± calcifications

± High flow

Rarely imaged


T1 isointense or hypointense

and T2 hyperintense

early and homogeneous gadolinium enhancement; fatty tissue with involution

± flow



Similar to hemangioma of infancy but may have ill-defined margin

High signal intensity on the T2

High-flow vessels

± fat stranding

Similar to HOI

± fat stranding

Infiltrative, irregular borders, invades muscle and fat, stranding, reticulatd subcutaneous pattern, thickened overlying skin

Hemosiderin seen on gradient echo

May have high flow (less than RICH for HOI)

± involvement of adjacent bone

Rarely imaged

Superficial lesions ± intramuscular extension


Well-circumscribed lobular masses with intense and persistent tissue uptake

± prominent early draining veins

Less intense vascularity during involution


Large, irregular feeding


± arteriovenous


± arterial aneurysms

Hypervascular mass


Small-to-large capillary lobules with fibrous tissue, moderately plump endothelial cells, pericytes

± central involuting zone

hemosiderin, thrombosis, cyst formation, areas of calcification, extramedullary hematopoiesis

Lobular thin-walled vessels lined with hobnailed endothelial cells

large central vessel

dysplastic veins

arterial shunting

mast cells

Infiltrating slitlike spindle-shaped endothelial cells with fibrous bands; low mitotic activity



Lobular endothelial cells forming “cannonball” appearance; tightly packed hypertrophic endothelial cells with scant cytoplasm, forming “tufts”

Dilated, thin-walled dermal and subcutaneous vessels lined with hobnailed, endothelial cells








LYVE-1 +

LYVE-1 +



± Transient self-resolved modest thrombocytopenia, hypofibrinogenemia

± Profound thrombocytopenia, hypofibrinogenemia

± Mild thrombocytopenia, hypofibrinogenemia

Thrombocytopenia, normal Fibrinogen

Gender ratio



Anatomical location

Predominantly cervicofacial

Cervicofacial, trunk, extremities, retroperitoneal

Skin (multifocal) and gastrointestinal tract (multifocal)

Unifocal or multifocal

Usually unifocal

Usually unifocal

Usually unifocal


Abbreviations: GI, gastrointestinal; GLUT1, glucose transporter type 1; LYVE1, lymphatic vessel endothelial hyaluronan receptor 1; MRI, magnetic resonance imaging.

Noninvoluting congenital hemangiomas (NICHs), also termed congenital nonprogressive hemangiomas, are histologically and immunophenotypically distinctive from typical hemangiomas of infancy and RICH and are considered to have a differing pathogenesis. 13 ,​ 14 They are present at birth and do not involute. On Doppler ultrasound, NICH-type lesions may have high flow clinically. 12 Histologically benign, these are glucose transporter type 1 (GLUT1) negative and have thin-walled, lobular endothelial cells with arterial shunting.

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Jun 15, 2020 | Posted by in HEAD AND NECK SURGERY | Comments Off on 2 Congenital Vascular Tumors
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