Tufted Angioma Workup

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

Approach Considerations

A diagnosis of tufted angiomas is classically suspected in an infant or young child presenting with a red or violet vascular lesion with ill-defined borders present on the trunk or extremities. Hypertrichosis and/or excessive sweating overlaying the lesion further supports a diagnosis of tufted angioma.

Upon suspicion of tufted angioma, a biopsy specimen to obtain histological confirmation should be obtained whenever possible. However, there are times when a biopsy carries more risk than benefit and is deferred, such as when the lesion is highly vascular and/or the patient also presents with thrombocytopenia and coagulopathy. When a risky biopsy is pursued, there should be careful planning and communication between the surgeon, anesthesiologist, hematologist, and pathologist to ensure preparation with platelets, fresh frozen plasma, and other supportive measures if needed intraoperatively and postoperatively.

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Laboratory Studies

No specific laboratory study is useful in the diagnosis or treatment of tufted angioma. However, upon diagnosis, laboratory workup of tufted angioma should include a complete blood cell (CBC) count, fibrinogen, D-dimer, prothrombin time (PT), and activated partial thromboplastin time (aPTT). Further evaluation with kidney and liver function tests should also be ordered in severely ill patients. Urinary levels of basic fibroblast growth factor are of no value in the diagnosis of this condition, unlike juvenile capillary angioma.

If the patient has coexistent Kasabach-Merritt syndrome, laboratory tests reveal severe thrombocytopenia, low fibrinogen, and elevated d-Dimer.

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Imaging Studies

MRI studies have been proven useful in the examination of patients with deep and/or extensive tufted angiomas. MRI has been successful in evaluating the depth of invasion and extent of growth of tufted angiomas that extend deep into the muscle and fascia, as well as in documenting the response to treatment. [14]

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Procedures

Other than biopsy for diagnosis and the surgical and laser treatments described, no other procedures are indicated for tufted angioma.

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Histologic Findings

Tufted angiomas have a specific histologic pattern. They are characterized by the lobular arrangement of densely cellular capillaries (tufts), often referred to as a “cannonball” pattern, that are scattered throughout the dermis. [36] The vascular tufts consist of tightly packed hypertrophic endothelial cells with scanty cytoplasm and nuclei that are round, ovoid, or fusiform. Mitoses are rarely seen.

Immunohistochemistry usually reveals positive D2-40 and POX1 staining, while GLUT1 (a marker for infantile hemangiomas) is always negative.

Occasionally, hemosiderin deposits may be observed within the endothelial cells. Capillary spaces are narrow and elongated, and they are more noticeable in the periphery of the lobules, where they have a characteristic half-moon cleft shape. [37]

The capillary lobules are present throughout the dermis and subcutaneous tissue; the epidermis is uninvolved in most cases. Rare cases with fascial and muscular involvement are reported. [14] The surrounding pilosebaceous units of the dermis are unaffected, although hypertrophy of neighboring eccrine sweat glands is reported. No edema or inflammation surrounds the vascular lobules.

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