Hemangiomas and Other Vascular Lesions
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- Vascular tumors: neoplasms of the vasculature
- Infantile hemangioma (IH)
- Congenital hemangiomas: noninvoluting congenital hemangioma (NICH) and rapidly involuting congenital hemangioma (RICH)
- Tufted angioma (TA)
- Kaposiform hemangioendothelioma (KHE)
- Pyogenic granuloma
- Vascular malformations (VaM): anomalous blood vessels without endothelial proliferation
- Capillary malformations (CM) (e.g., salmon patch, port-wine stain, nevus flammeus)
- Venous malformations (VM)
- Arterial malformations: arteriovenous malformations (AVM) or arteriovenous fistula (AVF)
- Lymphatic malformations (LM) (macrocystic and microcystic)
- Combined malformations (e.g., capillary-venous-lymphatic)
- Other types of VaM may occur in any part of the body and may be associated with soft tissue overgrowth of the involved part.
- 4–5% of infants
- ∼10% of Caucasian infants by age 12 months
- Increased incidence in low-birth-weight and premature infants
- Other demographic risk factors include white non-Hispanic race, female sex, multiple gestation pregnancy, advanced maternal age, chorionic villus sampling during pregnancy, and positive family history.
Commonly Associated Conditions
- Infantile hemangiomas
- Segmental hemangioma (usually facial) associated with other developmental anomalies (Posterior fossa malformations; hemangiomas; arterial anomalies; cardiac anomalies, including aortic coarctation; eye abnormalities; sternal defects/supraumbilical raphe)
- Cerebral malformations occur in >50% and cerebrovascular anomalies in 33%.
- An underlying spinal dysraphism may be present.
- PHACE-like syndromes of the lower body with associated GI and GU anomalies have been described.
- Commonly located on the face and involving a developmental unit (segment) and frequently associated with complications, even without meeting full PHACES criteria
- Segmental IHs are much more likely to have complications and to receive treatment than focal IHs.
- Kaposiform hemangioendothelioma
- Kasabach-Merritt phenomenon (consumptive coagulopathy, severe thrombocytopenia)
- CM may be present as part of syndromes (e.g., Sturge-Weber, RASA-1 mutations associated with fast-flow VaM, von Hippel-Lindau, Rubinstein-Taybi, Beckwith-Wiedemann, Cobb syndrome).
- VM can be inherited autosomal dominantly due to TIE2/TEK gene mutations.
- CM-AVM: due to RASA-1 gene mutation and associated with fast-flow VaM
- Generalized LM and lymphedema have been associated with VEGFR3 mutations.