Tufted Angioma

Updated: Apr 26, 2017
  • Author: Alexandra R Vaughn; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Tufted angiomas are rare vascular tumors characterized by tightly packed capillaries (“tufts”) in discrete lobules scattered through the dermis and, sometimes, subcutaneous tissue. Although adult-onset cases have been described, tufted angiomas occur far more frequently in infants and children. Tufted angiomas usually have a benign course, with only rare reports of aggressive behavior or local invasion. [1] However, tufted angiomas seldom self-involute. [2]

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Pathophysiology

The pathogenesis of tufted angiomas is not well understood. Vascular markers (CD31 and CD34), vascular endothelial growth factor receptor-3 (VEGFR-3), and lymphatic markers (D2-40 and PROX1) on the neoplastic cells suggest they may be derived from the endothelial cells of lymphatic vessels. [9, 10]

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Epidemiology

Frequency:

Tufted angiomas are rare, and the true incidence is unknown. As of 2015, only 158 cases are described in the English-language literature. [3]

Race:

No racial predilection is recognized for tufted angioma.

Sex:

The prevalence of tufted angioma is equal in males and females.

Age:

Approximately 50% of cases of tufted angiomas present at birth, while most other cases develop within the first year of life. [4] Although rare, there are reports of onset later in childhood and even in adulthood.

Less than 10% of tufted angiomas develop in individuals older than 50 years, and tumors in persons older than 60-80 years are rare. [2, 5] 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. [6, 7]

One case report describes a 69-year-old man who was found to have an intracranial tufted angioma, suggesting potential for aggressive behavior and invasion into the brain. [1] Another case describes a 40-year-old man with a 2-year history of a lesion on his lower eyelid, which was confirmed on histology to be a tufted angioma. [8]

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Prognosis

Tufted angiomas may spontaneously regress or may regress after treatment, but they do not completely resolve. [4, 11, 12] In fact, in a study of patients 10 years after diagnosis and treatment of tufted angioma, presence of tumor was still evident on repeat biopsy. [13] Pain and tenderness are common associated symptoms, and hyperhidrosis is a frequent finding that occurs in 30% of patients. [14]

The growth pattern of tufted angioma tends to be slow and progressive (ie, occurring over 5 months to 10 years). 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. [15]

Residual lesions after treatment of tufted angioma usually looks like one of three patterns: (1) discoloration resembling a port-wine stain with superimposed papules, (2) an indurated area with telangiectasias, or (3) a fibrotic infiltrate within the subcutaneous tissue. Long-term symptoms can include chronic pain and swelling.

Kasabach-Merritt syndrome occurs in association with tufted angioma in approximately 10% of patients. Fortunately, most of these patients have a favorable response to treatment without further sequelae. However, there is a mortality rate of up to 24% in patients with Kasabach-Merritt syndrome. [16] Death in these cases results from bleeding, sepsis, invasion of a vital structure, or organ failure. [17]

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Patient Education

Tufted angioma is a benign tumor, with very rare reports of deaths directly or indirectly attributable to complications. However, pain and tenderness are common associated symptoms, and hyperhidrosis is a frequent finding that occurs in 30% of patients. [14]

The growth pattern of tufted angioma tends to be slow and progressive (ie, occurring over 5 months to 10 years). 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.

Kasabach-Merritt syndrome, characterized by severely low platelets and coagulopathy, has been reported to occur in 10% of patients with a diagnosis of tufted angioma. Fortunately, most of these patients have a favorable response to treatment.

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