Background
Tufted angiomas are rare vascular tumors most commonly localized to the skin and subcutaneous tissues and characterized by slow angiomatous proliferation. Although adult-onset cases have been described, tufted angiomas occur far more frequently in children. Tufted angiomas have a benign course, with no reported cases of aggressive behavior or metastases. However, tufted angiomas seldom self-involute.[1]
Epidemiology
Frequency
United States
The incidence of tufted angiomas is low. As of the year 2000, only 54 cases are described in the English-language literature in the United States and Europe.
International
The international incidence of tufted angioma is also low. However, after Nakagawa identified the condition, angioblastoma, in 1949, more cases have been described in Japan. By 2000, 157 cases had been reported.[2, 3, 4]
Mortality/Morbidity
Tufted angioma is a benign entity. No deaths directly or indirectly attributable to complications have been reported. However, pain and tenderness are common associated symptoms, and hyperhidrosis is a frequent finding that occurs in 30% of patients.[5]
The growth pattern of tufted angioma tends to be slow and progressive (ie, occurring over 5 mo to 10 y). However, an occasional tufted angioma undergoes a rapid expansion phase that leads to localized swelling and a decreased range of motion of the structures in the affected area. One report described a patient with a painful tufted angioma on the lateral neck and jaw. The tumor also had deep extension into the underlying fascia and muscle, a rare finding.[6]
Kasabach-Merritt syndrome (KMS), also known as platelet-trapping syndrome (PTS), occurs in association with tufted angioma in some patients. Fortunately, most of these patients have a favorable response to the treatment of platelet-trapping syndrome with aspirin and/or ticlopidine, oral betamethasone, or vincristine, without further sequelae.
Race
No racial predilection is recognized for tufted angioma.
Sex
The prevalence of tufted angioma is equal in both sexes.
Age
Most series report that 60-70% of tufted angiomas develop before the patient is aged 5 years, and 25% of tumors appear by the time the patient is aged 1 year. The Japanese-language literature, however, reports that more than 50% of tufted angiomas appear in the patient's first year of life. Of these patients, 15% are believed to have had a blemish in the area where the tufted angioma later developed; this observation suggests a congenital mode of presentation.
Less than 10% of tufted angiomas develop in individuals older than 50 years, and tumors in persons older than 60-80 years are rare.[1, 4] One 81-year-old patient with a tufted angioma of 2 years' duration is reported in the French-language literature, and a case has been described in an 84-year-old man in Japan.[7, 8]
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Nakagawa K. Case report of angioblastoma of the skin. Jpn J Dermatol. 1949;59:92-4.
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