Hemangiomas and Other Vascular Lesions

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DESCRIPTION

  • Vascular tumors: proliferative neoplasms of the vasculature include:
    • Infantile hemangioma (IH) (superficial, mixed, deep, IH with minimal or absent growth [IH-MAG])
    • Congenital hemangiomas: noninvoluting congenital hemangioma (NICH) and rapidly involuting congenital hemangioma (RICH)
    • Tufted angioma (TA)
    • Kaposi form hemangioendothelioma (KHE)
    • Pyogenic granuloma
  • Vascular malformations (VaMs): inborn errors of vascular morphogenesis; relatively static
    • Capillary malformations (CMs)
      • Nevus simplex (“salmon patch”)
      • Nevus flammeus (“port-wine stain”)
    • Venous malformations (VMs)
    • Arterial malformations: arteriovenous malformations (AVMs) or arteriovenous fistula (AVF)
    • Lymphatic malformations (LMs) (macrocystic and microcystic)

EPIDEMIOLOGY

  • Infantile hemangiomas
    • The most common pediatric vascular tumor; reported incidence is 2–10%.
    • Risk factors: female sex, prematurity, low birth weight, multiple gestation, family history of IH, progesterone therapy
  • Port-wine birthmarks
  • Estimated prevalence is 3 to 5 per 1,000 live births.
  • No sex predilection; sporadic inheritance pattern

COMMONLY ASSOCIATED CONDITIONS

  • Infantile hemangiomas
    • PHACE syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects or aortic coarctation, eye anomalies)
      • Cerebral malformations occur in >50% and cerebrovascular anomalies in 33%.
    • LUMBAR syndrome (lower body IH and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, renal anomalies)
  • Port-wine birthmarks
    • Sturge-Weber syndrome
    • Klippel-Trenaunay syndrome
    • Parkes Weber syndrome
  • KHE
    • Kasabach-Merritt phenomenon (consumptive coagulopathy, severe thrombocytopenia)

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