Benign Vascular Tumors

Published on 22/03/2015 by admin

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Chapter 499 Benign Vascular Tumors

499.1 Hemangiomas

Hemangiomas, the most common benign tumors of infancy, occur in about 5% of term infants (Chapter 642). The risk of hemangioma is 3-5 times higher in girls than boys. The risk is doubled in premature infants and 10 times higher in offspring of women who had chorionic villus sampling. Hemangiomas can be present at birth but usually arise shortly after birth and grow rapidly during the 1st yr of life, with slowing of growth in the next 5 yr and involution by 10-15 yr of age.

Clinical Manifestations

More than 50% of all hemangiomas are located in the head and neck region. Most are solitary lesions, but the presence of more than one cutaneous lesion increases the likelihood of visceral hemangiomas. The liver is the primary site of visceral involvement; other involved organs include the brain, intestines, and lung. Most hemangiomas require no therapy, but approximately 10% of hemangiomas cause significant impairment and 1% are life-threatening because of their location. Hemangiomas around the airway can cause airway obstruction, and those around the eyes can result in loss of vision. Ulceration is a common complication and can lead to secondary infection. Large hepatic hemangiomas or hemangioendotheliomas may result in hepatomegaly, anemia, thrombocytopenia, and high-output heart failure.

Kasabach-Merritt syndrome (Chapter 642) is characterized by a rapidly enlarging lesion, thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy as a result of platelet and red blood cell trapping and activation of the clotting system within the vasculature of the hemangioma. This syndrome has been shown to be associated with kaposiform hemangioendotheliomas or tufted angiomas.

Cutaneous lesions usually can be diagnosed by typical appearance and rapid proliferation. Segmental hemangiomas, or those with geographic localization and some plaquelike features, recently have been shown to have a higher risk of complications and association with developmental abnormalities. A deep lesion may require imaging studies to help differentiate it from a lymphangioma. The presence of a midline hemangioma in the lumbosacral area indicates the need for an MRI to search for underlying asymptomatic neurologic abnormalities. Location also may dictate the need for an ophthalmologic or surgical consultation. An ultrasonographic scan or MRI of the liver should be performed if multiple cutaneous lesions are present.

499.2 Lymphangiomas and Cystic Hygromas

Lymphatic malformations, including lymphangiomas and cystic hygromas, which arise in the embryonic lymph sac, are the 2nd most common benign vascular tumors in children. About half are located in the head and neck area. Approximately 50% are present at birth, with most presenting by 2 yr of age. There is no gender predisposition. Spontaneous regression has been reported but is not typical.

Lymphatic malformations present as soft, painless masses that transilluminate if superficial. Intrathoracic lymphatic malformation can present as symptoms related to a mediastinal mass or pericardial or pleural effusion. Rapid enlargement can occur with infection or hemorrhage. Localized lesions may be surgically resected, but this can be difficult, owing to their infiltrative nature. Recurrence is common with incompletely resected lesions. Aspiration can provide temporary relief in an emergency, such as in the presence of dyspnea, but reaccumulation will occur. Treatment by injection of sclerosing agents, laser therapy, and systemic interferon therapy also has been used. The streptococcal immunotherapeutic agent OK-432 (Picibanil) is the sclerosing agent of choice, avoiding the need for surgery in most cases. Bleomycin is also an effective sclerosing agent but has a risk of pulmonary toxicity. It appears to be especially effective for the treatment of macrocystic lymphangiomas of the head and neck.