Vascular Tumors of the Skin and Subcutaneous Tissue

Hemangiomas are benign vascular neoplasms that present soon after birth (Fig. 16-7). They
initially undergo rapid cellular proliferation over the first year of life, then undergo slow
involution throughout childhood.5860 Histologically, hemangiomas are composed of
mitotically active endothelial cells surroundings several, confluent blood-filled spaces.
Although these lesions may enlarge significantly in the first year of life, approximately 90%
involute over time.5860 Acute treatment is limited to hemangiomata that interfere with
function, such as airway, vision, and feeding. In addition, lesions resulting in systemic
problems, such as thrombocytopenia or high-output cardiac failure, should prompt resection.
The growth of rapidly enlarging lesions also can be halted with systemic prednisone or
interferon alpha-2a treatment use.5860 In the absence of acute surgical indications or
significant patient/parent concern, many lesions are allowed to spontaneously involute.
However, hemangiomata that remain into adolescence or involute to leave an unsightly
telangiectasia typically require surgical excision for optimal resolution.5860

Hemangiomas most often present at approximately 2 to 4 weeks after birth, rapidly

proliferate during infancy, reach a plateau phase, then involute over several years. Unless the
hemangiomatous mass obstructs the airway, visual axis, or imposes psychological harm to a
preschool age child, these tumors typically are allowed to spontaneously involute.

In contrast to neoplasms, vascular malformations are a result of structural abnormalities

formed during fetal development.61,62 Unlike hemangiomas, vascular malformations grow in
proportion to the body and never involute. Histologically, they contain enlarged vascular
spaces lined by nonproliferating endothelium.61,62 Arteriovenous malformations are high-flow
lesions that often present as subcutaneous masses associated with locally elevated
temperature, dermal stain, thrill, and bruit. In addition, overlying ischemic ulcers, adjacent
bone destruction, or local hypertrophy may occur.61,62 Very large malformations may cause
cardiac enlargement and congestive heart failure. Complications of arteriovenous
malformations, such as pain, hemorrhage, ulceration, cardiac effects, or local tissue
destruction, should prompt attempts at lesion destruction.61,62 Therapy consists of surgical
resection. Even when complete lesion resection is not possible, significant debulking may
greatly diminish symptomatology. In addition, angiography with selective embolization just
before surgery greatly facilitates operative removal.61,62
The capillary malformation, or port-wine stain, is a flat, dull-red lesion often located on the
trigeminal (cranial nerve V) distribution on the face, trunk, or extremities (Fig. 16-8).61,62
Presentation within the V1 or V2 facial regions should prompt concern of a possible link to
more systemic syndromes such as Sturge-Weber syndrome (leptomeningeal angiomatosis,
epilepsy, and glaucoma).61,62 Histologically, these nevi are composed of ectatic capillaries
lined by mature endothelium. Unsightly lesions may be treated with pulsed dye laser, covered
with cosmetics, or surgically excised.61,62

A capillary hemangioma (also known as a port-wine stain) present upon the midface may
signify Churg-Strauss syndrome, and computed tomography of the brain is appropriate to
rule out intracranial berry aneurysms.

Glomus tumor is an uncommon, benign neoplasm of the extremity. Representing less than
1.5% of all benign, soft-tissue extremity tumors, these lesions arise from dermal
neuromyoarterial apparatus (glomus bodies).63,64 Glomus tumor more commonly affects the
hand, and presentation within the subungual region of the toe is rare. Diagnosis of these
lesions is traditionally delayed, and atypical presentation on the foot or toes often leads to
even greater diagnostic challenges. In addition to the severe pain, point tenderness and cold
sensitivity are associated with these lesions and subungual glomus tumors typically appear as
blue, subungual discolorations of 1 to 2 mm. Tumor excision is the treatment of choice.63,64