Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ 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 ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.