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.^[44]

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.^[45]Vincristine administered via a central line is usually given in conjunction with corticosteroids.^[46]Sirolimus is an oral chemotherapy agent that is still under investigation for the treatment of infantile vascular tumors.^[47]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.^[48]

In a clinical trial comparing vincristine and corticosteroids for the treatment of tufted angioma and kaposiform hemangioendothelioma, investigators found that vincristine had a significantly better effect than corticosteroids on platelet counts and tumor texture. The authors recommended that vincristine be considered as a first-line therapy for tufted angioma.^[49]

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.^[50]Fresh frozen plasma may be required in infants with DIC. Packed red blood cells should be given for symptomatic anemia.

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.^[51]Surgical excision can be tried as second-line therapy after failure of medical management.^[52]

Cryosurgery, radiation therapy, and embolization are successful to varying degrees in tufted angioma patients.^[53]Use of the pulsed-dye laser has had variable results in both children and adults with tufted angioma.^[23]

Guidelines

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Media Gallery

This lesion is a tufted angioma in a 1-year-old girl. Present since birth, this bluish-red hemangioma-like plaque on the patient's right upper leg slowly enlarged and became tender during the month prior to presentation. The child was otherwise well. A skin biopsy specimen revealed dilated congested capillaries in the lower dermis and subcutis. Clusters of capillaries with hyperplastic endothelial cells were also scattered in the mid and upper parts of the dermis. The epidermis revealed a slight basket-weave orthokeratosis, with minimal acanthosis, and papillomatosis. The patient was treated conservatively with only observation. The lesion remained stable and did not progress since presentation. Courtesy of National Skin Centre, Singapore.

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Author

Alexandra R Vaughn Drexel University College of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Amanda T Moon, MD Clinical Assistant Professor of Dermatology, Children's Hospital of Philadelphia

Amanda T Moon, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

John G Albertini, MD Private Practice, The Skin Surgery Center; Clinical Associate Professor (Volunteer), Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine; Past President, American College of Mohs Surgery

John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: QualDerm Partners; Novascan<br/>Have a 5% or greater equity interest in: QualDerm Partners - North Carolina.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mitchel P Goldman, MD Voluntary Clinical Professor of Dermatology, University of California, San Diego, Medical Center; Dermatologist, West Dermatology and Cosmetic Laser Dermatology

Mitchel P Goldman, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Cosmetic Surgery, American Academy of Dermatology, American College of Phlebology, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, American Society of Lipo-Suction Surgery, California Medical Association, Dermatology Foundation, Phi Beta Kappa, San Diego County Medical Society

Disclosure: Nothing to disclose.

Leyda E Bowes, MD Cosmetic Surgery Fellow, Department of Dermatology, Cosmetic Laser Associates of San Diego County

Leyda E Bowes, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Florida Medical Association

Disclosure: Nothing to disclose.

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Tufted Angioma Treatment & Management

Medical Care

Hemostasis Support

Surgical Care

References

Tables

Contributor Information and Disclosures

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