2 Congenital Vascular Tumors



10.1055/b-0034-101158

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


(KHE)


Tufted angioma


(TA)


Multifocal lymphangioendotheliomatosis (MLT)


Time of presentation


Postnatal


Prenatal/newborn


Newborn


Prenatal/newborn/later


Newborn/later


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


pink-purple


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


Ultrasound


Hyperechoic


Variable flow depending on stage


Heterogeneous


Less defined than HOI


± High-flow


± calcifications


Heterogeneous


Less defined than HOI


± High flow


± calcifications


± High flow


Rarely imaged




MRI


T1 isointense or hypointense


and T2 hyperintense


early and homogeneous gadolinium enhancement; fatty tissue with involution


± flow


voids


Well-defined


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



Angiography


Well-circumscribed lobular masses with intense and persistent tissue uptake


± prominent early draining veins


Less intense vascularity during involution


Nonhomogeneous


Large, irregular feeding


Arteries


± arteriovenous


shunts


± arterial aneurysms



Hypervascular mass





Histology



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


hemosiderin


microthrombi



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


Immunohistochemistry


GLUT1+


GLUT1-


GLUT1-


GLUT1-


LYVE-1


GLUT1-


LYVE-1 +


LYVE-1 +


Ki-67+


Coagulopathy/thrombocytopenia



± Transient self-resolved modest thrombocytopenia, hypofibrinogenemia



± Profound thrombocytopenia, hypofibrinogenemia


± Mild thrombocytopenia, hypofibrinogenemia


Thrombocytopenia, normal Fibrinogen


Gender ratio


3–4:1


1:1






Anatomical location


Predominantly cervicofacial





Cervicofacial, trunk, extremities, retroperitoneal


Skin (multifocal) and gastrointestinal tract (multifocal)



Unifocal or multifocal


Usually unifocal



Usually unifocal


Usually unifocal


Multifocal


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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