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OVERVIEW

  • Vascular tumors of infancy and childhood

    • Infantile hemangioma

    • “Congenital hemangiomas” (noninvoluting, or NICH; rapidly involuting, or RICH)

    • Kaposiform hemangioendothelioma

    • Tufted angioma

    • Pyogenic granuloma

  • Vascular tumors of adulthood

    • Kaposi sarcoma

    • Angiolymphoid hyperplasia with eosinophilia

    • Endovascular papillary angioendothelioma (Dabska tumor)

    • Intravascular papillary endothelial hyperplasia (Masson tumor)

    • Low-grade angiosarcomas

      • – Epithelioid hemangioendothelioma

      • – Spindle cell hemangioendothelioma

      • – Retiform hemangioendothelioma

    • Angiosarcoma

  • Vascular malformations

    • Capillary

      • – Salmon patch

      • – Port-wine stain

      • – Cutis marmorata telangiectatica congenital

      • – Phakomatosis pigmentovascularis

      • – Unilateral nevoid telangiectasia

      • – Angiokeratomas

    • Lymphatic: microcystic, macrocystic, or combined

    • Venous

      • – Glomuvenous malformations: glomus tumors, glomangiomas, and glomangiomatosis

    • Arterial

    • See complex vascular malformation/overgrowth syndromes

VASCULAR TUMORS OF INFANCY AND CHILDHOOD

Infantile Hemangioma (IH)

  • Characteristics

    • Most common tumor of infancy

    • Characterized by endothelial cell proliferation

    • Endothelial cells derived from hemangioma express vascular endothelial growth factor (VEGF-A), VEGF receptors, TIE2, and angiopoietin-2

    • GLUT-1 (glucose transporter) is an immunohistochemical stain specific for IH in all phases of growth and involution

    • Positive staining occurs only with IH and not with any other vascular tumor or malformation

    • Proliferative phase: 6 to 12 months; rarely longer

    • Involution phase: gradual over several or more years

    • Risk factors: Caucasian, female, low birth weight, multiple gestation

  • Location: more than 60% occur on head or neck, most commonly midcheek, lateral upper lip, and upper eyelid

  • Types

    • Superficial, deep, or combined

      • – Superficial

        • ▴ Most common

        • ▴ Raised, bright-red papule, nodule, or plaque

      • – Deep: soft, flesh-colored nodule that often has a bluish hue and/or central telangiectasias

      • – Combined (Fig. 13-1)

  • Localized, segmental, or multiple

  • Classification by morphology

    • Localized: papules or nodules that appear to arise from a single focal point and demonstrate clear spatial containment

    • Segmental (Fig. 13-2)

      • – Plaque-like and show a linear and/or geographic pattern over a cutaneous territory

      • – Much more likely to be complicated, require more intensive and prolonged therapy, and have a poorer overall outcome

    • Multifocal

      • – Generally defined as five or more small, localized lesions

      • – Multiple hemangiomas are associated with multiple births

  • Complications

    • Ulceration

      • – Most common in proliferative phase

      • – Often leads to pain, scarring, bleeding, and secondary infection

      • – Favors IH in trauma-prone sites: lip, perineum, intertriginous, posterior scalp, and back

    • Scarring

      • – More common with segmental IH, localized IH of superficial, raised morphology with sharp “cliff drop” border, ulceration

      • – High-risk locations: lip (especially when crossing the vermillion border), nasal tip, and ear

    • Vital organ compromise

      • – Visual obstruction

        • ▴ Amblyopia from stimulus deprivation or astigmatism

        • ▴ Most common when IH involves upper eyelid (Fig. 13-3)

        • ▴ Refer to ophthalmology

      • – Airway (especially subglottic) IH

        • ▴ Associated with segmental IH in a cervicofacial or “beard” distribution

        • ▴ Watch for development of stridor

        • ▴ Endoscopy for definitive diagnosis

    • Visceral IH

      • – Associated with multifocal and solitary segmental IH

      • – Liver most worrisome site

      • – Complications of multifocal and diffuse liver hemangiomas include acquired hypothyroidism due to hemangioma expression of type 3 thyronine deiodinase and macrovascular high-flow shunting that can lead to low- or high-output cardiac failure

      • – Gastrointestinal IH can rarely lead to significant bleeding

      • – Brain IH can very rarely lead to mass effects/neurologic sequelae

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