Tufted Angioma Treatment & Management

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

Approach Considerations

The treatment for tufted angioma is multimodal and individualized, depending on the extent of tumor invasion, tumor size, presence of symptoms, and presence or absence of Kasabach-Merritt syndrome. Treatment generally consists of one or multiple approaches, including surgical excision, pulsed-dye laser (for superficial tumors), and several medications and chemotherapy agents. The efficacy of one treatment approach over another has not been established in large randomized clinical trials, and current available evidence is based on anecdotal clinical experience, case reports, and small case series.

For patients with small tufted angiomas who are asymptomatic, observation only can be considered. There have been a few reports of patients whose tufted angiomas spontaneously regressed in infancy and childhood. [6, 38, 39]

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Medical Care

For patients without Kasabach-Merritt syndrome who are symptomatic and who opt not to have surgical excision or who are not surgical candidates, pharmacological management with aspirin 5 mg/kg/day is first-line treatment. [40]

In patients with unresectable tumors owing to size, location, or surgical risks, pharmacotherapy can be used to decrease the tumor size. Although no single agent or combination of agents has been proven better than another, several chemotherapy agents haven been used, including vincristine, sirolimus (an mTOR inhibitor), interferon-alfa, and systemic corticosteroids. Systemic corticosteroids are often used as first-line therapy to stabilize the platelet count and then slowly tapered. [41] Vincristine administered via a central line is usually given in conjunction with corticosteroids. [42] Sirolimus is an oral chemotherapy agent that is still under investigation for the treatment of infantile vascular tumors. [43] It has been effectively used to treat several cases of tufted angioma, demonstrating it to be a possible promising treatment with good preliminary results. According to a 2016 meta-analysis of 15 clinical studies of various therapies used to treat tufted angioma, results suggest vincristine may be most effective relative to other agents. [44]

 

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Hemostasis Support

For patients with coexistent Kasabach-Merritt syndrome, the coagulopathy must be carefully managed with hemostasis support. In these patients, hemorrhage is rare but can occur in disseminated intravascular coagulation (DIC) and multiorgan failure. Despite severely low platelet counts in these patients, platelets should only be given prior to surgery and in patients with active bleeding; platelets administration can rapidly increase the size of and pain associated with the tumor, leading to increased platelet trapping. In patients with low fibrinogen levels, cryoprecipitate should be given if one of the following four conditions is present: (1) active bleeding, (2) in preparation for surgery, (3) platelet count less than 10,000/µL, or (3) a fibrinogen level less than 1 g/L. Recombinant human factor VIIa can be given in cases of active bleeding. [45] Fresh frozen plasma may be required in infants with DIC. Packed red blood cells should be given for symptomatic anemia.

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Surgical Care

Complete surgical excision, if chosen, allows permanent removal of small or localized tufted angiomas, although local recurrence of some surgically excised tumors is observed. [46] Surgical excision can be tried as second-line therapy after failure of medical management. [47]

Cryosurgery, radiation therapy, and embolization are successful to varying degrees in tufted angioma patients. [48] Use of the pulsed-dye laser has had variable results in both children and adults with tufted angioma. [11]

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Consultations

If Kasabach-Merritt syndrome is suspected in a child, consultation with a hematologist/oncologist and a dermatologist may be indicated.

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Prevention

Because the cause of tufted angioma is poorly understood, no specific method of prevention or deterrence is recommended.

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Long-Term Monitoring

In patients who opt not to undergo medical or surgical management, tumors should be carefully monitored for size expansion and the development of Kasabach-Merritt syndrome.

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