Introduction
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
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.
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.6,7
Clinical
History
- Most tufted angiomas develop by the time the patient is aged 5 years, although a number of juvenile and adult cases have been reported.
- A congenital linear tufted angioma also is reported.8 It is localized to the skin, with no documented systemic or metastatic involvement.
- Most reported tumors occur on the upper trunk, neck, and shoulders, and fewer tumors occur on the face, scalp, and proximal extremities.
- Involvement of the feet and oral mucosa is observed, but only in rare cases.9,10 In the former, the tufted angioma was located intravenously.
- A tufted angioma is generally described as a dull red, brown-red, or purple cutaneous patch or plaque.
- The patch or plaque slowly enlarges for 5 months to 10 years, after which no further growth occurs.
- Generally, the lesion is described as solitary. However, multifocal presentations are reported, although these lesions are always localized to the skin and rarely involve the subcutaneous tissues.
- Partial regression is reported, but complete regression is rare.4,11,12
- Many tufted angiomas have associated painful episodes.
- No history of trauma preceding the appearance of the tufted angioma is reported, although 1 case report claims that a tufted angioma developed at the site of an arthropod bite.
Physical
- A tufted angioma appears as a purplish red to red-brown patch or plaque that predominantly appears on the upper thorax; neck; shoulders; and, less commonly, on the face, scalp, and proximal extremities.13,14
- The diameter of the patch of plaque generally ranges from less than 1 cm to several centimeters.
- The tufted angioma may be solitary or multifocal, and it can be round to polycyclic in more extensive cases (see Media File 1).
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 progressed since presentation. Courtesy of National Skin Centre, Singapore.
- On palpation, tufted angiomas often have a rubbery consistency, and they may be painful.
- Ulceration is rare; this finding may be predicted from its slow rate of growth.
- Hyperhidrosis of the skin overlying the area of the tufted angioma is reported. Histologically, hyperhidrosis corresponds with areas of abundant eccrine glands.4
- Peripheral extension occurs, and lesions of tufted angioma are described as having a thick indurated border and a central depression, with an appearance similar to that of a doughnut.5
- Tufted angioma lesions may also appear as deep red or purple patches with scattered round-to-oval dark-red nodules on their surface.
- Occasionally, tufted angiomas may be associated with the more serious platelet-trapping syndrome or Kasabach-Merritt syndrome. In this setting, the presence of petechiae and ecchymotic patches should alert the physician to the development of these entities.15
Causes
- No causes of tufted angioma have been established. Trauma does not appear to be a predisposing factor, although a report describes the appearance of a lesion of tufted angioma at the site of a previous arthropod bite.
- Several authors have noted the development of tufted angioma within port-wine stains or in the context of these stains.16,17,18
- Some authors have postulated that high hormonal levels during pregnancy and puberty may induce the development of tufted angiomas.19,20
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| References |
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References
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Further Reading
Keywords
tufted angioma, tufted angiomas, Nakagawa's angioma, Nakagawa's angioblastoma, Nakagawa angioma, Nakagawa angioblastoma, progressive capillary hemangioma, acquired tufted angioma, skin and subcutaneous tissue vascular tumor, vascular tumor, angioblastoma
