Rare Tumors in Children: Vascular Tumors
摘要
Vascular tumors are a heterogenous group of lesions, within the vascular anomalies spectrum. They are divided into three groups (benign, borderline/locally aggressive, and malignant). Infantile hemangioma is the most common benign tumor of infancy, it is a proliferative vascular lesion that appears within the first weeks of life, and it rapidly grows and then spontaneously begins to involute. Most lesions are cutaneous; however, they can develop in different body organs. Congenital hemangiomas are present at birth. These lesions proliferate intrautero and are fully developed when the baby is born. Tufted angioma and kaposiform hemangioendothelioma (KHE) are considered borderline tumors and belong to the same pathology spectrum, as they share clinical and histopathologic findings and some severe complications such as Kasabach-Merritt phenomenon. Even when they are histologically benign lesions, KHE can present a high grade of morbidity and mortality, and early diagnosis and treatment is essential. On the other end, malignant vascular tumors in childhood are extremely rare. Unlike hemangiomas, they do not regress or involute and have metastatic potential. This chapter is focused in the description, management, and treatment of the vascular tumors that present during childhood.