Pediatric Clinics of North America - CIPERJ

CARE OF PATIENTS WITH VASCULAR ANOMALIES
341
Vascular tumors
Classification
As illustrated in Fig. 1, vascular tumors represent the first large category
of vascular anomalies observed in children. The majority of these vascular
tumors are hemangiomas (often called infantile hemangiomas) that appear
shortly after birth. Most hemangiomas are simple lesions and require only
observation. Some hemangiomas are more complex and cause injury or disfigurement
to vital organs, whereas others are associated with other physical
abnormalities and syndromes. In contrast, certain lesions are identified as
congenital hemangiomas, which are present at birth but are pathologically
and clinically different from infantile hemangiomas. Lesions classified as
‘‘other’’ vascular tumors are endothelial cell derived but are not true hemangiomas;
these have a more complicated picture (discussed later).
Diagnosis
Hemangiomas are the most common vascular tumor of infancy. Hemangiomas
occur more commonly in white newborns, with a higher incidence in
female and premature infants [2–5]. They are observed most commonly in
the head and neck area followed by the trunk and extremities. The majority
occur as single tumors, but as many as 20% of affected infants have multiple
lesions [2–5]. Most hemangiomas are not seen at birth but appear during the
first several weeks of life. Hemangiomas can be deep or superficial or a combination
of the two types. Deep hemangiomas are soft, warm masses with
a bluish color. Superficial hemangiomas are red and raised or, rarely,
telangiectatic.
Hemangiomas have several phases of growth. The first is the proliferating
phase during which they expand rapidly. This phase lasts for 4 to 6 months.
In this phase, the hemangioma’s superficial component becomes more
erythematous or violacious. Expansion can occur superficially and deeply.
Deep hemangiomas may proliferate through up to 2 years of age. A stationary
phase follows during which the hemangioma grows in proportion to the
child. This phase is followed by an involuting phase that can last up to 5 to
6 years. Involuting hemangiomas become more gray in color. Maximum involution
occurs in approximately 50% of children by age 5 years and in 90%
of children by age 9 [2–5]. The majority of patients do not have sequelae, but
20% to 40 % of patients have residual changes of the skin, such as laxity,
discoloration, telangiectasias, fibrofatty masses, or scarring.
Congenital hemangiomas are an entity distinguished from infantile
hemangiomas because they are fully developed at birth and even can be
diagnosed in utero. There are two subgroups: rapidly involuting congenital
hemangiomas (RICH) and noninvoluting congenital hemangiomas (NICH)
[6]. The RICH lesions can look very violacious at birth but regress rapidly
during the first year of life. In contrast, NICH lesions are fully developed at