Tufted angioma

ARTICLE

Tufted angioma is a rare skin tumour within the group of capillary haemangiomas [1, 2]. It may already be evident at birth, but usually develops during late infancy or adolescence. Even later manifestations are possible [1, 3]. Numerically, both sexes are affected to about the same extent [3, 4]. In one particular case, a high number of occurrences within one family has been reported [5].

Clinically, tufted angiomas appear as dull red, blue or purple macules or plaques, mostly involving the neck, shoulder or upper trunk regions, and sometimes also affecting the proximal limbs [1-3, 5-17]. Usually, the tumour is symptomless [1, 3]. However, in 30% of all cases, an increased sensitivity on palpation and tenderness are noticed [3, 4, 6, 8-10]. Some authors also report an intensified growth of vellus hair and hyperhidrosis around the angioma [6-8]. Tufted angioma undergoes a temporally limited phase of growth with mostly torpid, but occasionally rapid expansion [3, 4, 9]. Although the lesions may cover a wide anatomical area, they are entirely benign [3, 4, 9]. No malignant transformations have been reported to date. Only in a few cases, however, has spontaneous partial or complete regression been reported [7, 9]. In the extended form, on the other hand, the clinical course can be complicated by the Kasabach-Merritt syndrome [18]. Considering the similarity to malignant vascular tumours such as angiosarcoma or Kaposi's sarcoma, it is of considerable importance to be able to distinguish between these and tufted agioma.

Case reports

Case one

18 months before presenting at the hospital, an 11 year old boy had noticed a red tumour of 1.5 cm diameter on the left thoracic region. The tender cutaneous lesion had increased in size over the following months. Upon admittance to our hospital, a 1.5 x 4.5 cm, painful, erythematous, soft and movable tumour was found. The colour duplex revealed that the tumour penetrated to the subcutis. There was no bloodflow. The remaining clinical and laboratory findings were within the normal range.

The histological examination revealed well-circumscribed and cell-rich tufts peripherally enclosed by crescent-shaped, dilated vascular clefts that were scattered particularly throughout the reticular layer of dermis (Fig. 1). The epidermis and the subcutis were not involved. The proliferates were principally composed of uniform tumour cells with poorly outlined, eosiniphilic cytoplasm and partly fusiform, partly circular nuclei. The mitotic rate was not increased. Immunohistologically, an endothielial differentiation (CD31⁺ and CD34⁺) could be found. Apart from this, pericytes which were positive for smooth muscle actin (HHF 35) were identified. Up to 10% of the tumour cells were positive for Ki 67 (MIB-I).

Intralesional treatment with interferon-alpha 2b 1.5 Mio IE was administered three times per week over a period of three months. A partial remission was achieved with a decrease in size to 3.2 x 1.0 cm. After a follow-up of four months, no further improvement was observed. Therefore, we decided that the proper course was excision. To date, the patient is free from recurrence.

Case two

An 8 year old boy had had a livid tumour on the right side of the chest since birth. Over the years, this cutaneous lesion had increased in size. There were no subjective complaints. Upon admittance to our hospital, the boy presented a livid tumour on the right lateral thoracic wall, 2.5 cm in diameter (Fig. 2). It was enclosed by an anemic halo. The colour duplex examination revealed substantial vascularisation, with a depth of 5 mm.

The dominant histological feature was a cell proliferation with small clefts and luminae (Fig. 3). The lobuli that were scattered in the papillary and reticular layer of dermis were composed of endothelially-lined vessels and solid regions. In parallel to case one, the immunohistological analysis of this case revealed endothelial differentiation (CD31⁺ and CD34⁺). Apart from this, pericytes expressing smooth muscle actin (HHF 35) could be identified.

The tumour was surgically removed. To date (10 months after the surgery), we have not observed any relapse.

Discussion

The vascular tumour, tufted angioma, which was named because of its histological pattern, was first described by Wilson Jones (1976) [11, 12]. In Japan, however, a similar lesion had already been mentioned under the name angioblastoma (Nakagawa) in 1949 [12, 13]. Considering the slowly progressive, but benign character of the tumour, differentiation from other angioproliferative neoplasms is necessary. From the range of differential diagnoses (Table I) Kaposi's sarcoma and angiosarcoma have to be particularly differentiated [3].

In the cases described here, the tufted angioma manifested during childhood. However, its presence at birth, as seen in case 2, is observed quite rarely [3]. Both its localization at the thorax and its slow evolution are among the typical characterisitcs of this tumour [1, 3, 4]. In addition, the tenderness described in case one is quite often observed [3, 4, 6, 8-10].

In both cases, the histological analysis revealed well-circumscribed tufts composed of solid tumour cells under a slightly changed epidermis. These showed the "cannonball" aspect described by Wilson Jones [3]. At the periphery of the lobuli, crescent-shaped, dilated vascular clefts could be observed. The conglomerates of capillary spaces may resemble renal glomeruli, similar to the histological features found in glomeruloid haemangioma [3, 4, 8]. The tumour cells were cytologically bland, but with an increased proliferative activity (Ki 67-positive) in 5-100% of all cells. The immunopositivity for CD31 and CD34 indicates the endothelial differentiation of the solid tumour cells shaping the lobuli. At the same time, however, only in those parts of the tumour which were identifiable as vascular structures, could a positivity of the endothelial cells towards factor VIII-related antigen be found. This shows a certain immaturity of the neoplastic endothelium. Along with the identification of smooth muscle actin, the existence of a second cellular tumour component, the pericytes, could be shown. There was no inflammatory infiltrate.

To date no therapeutic concept for the treatment of tufted angioma has been established. However, the successful administration of interferon alpha has been recently reported [9, 19]. Therefore, in case 1 we administered interferon alpha s.c. for 3 months. The tumour stopped growing, although a complete remission was not achieved. Nevertheless, this facilitated the surgical intervention. Interferon therpay has been reported to be efficacious in other vascular tumours and malformations. For example, good results were described in relation to Kaposi's sarcoma, pulmonary haemangiomatosis as well as in some recalcitrant cases of haemangioma [20-22]. The exact mode of action, however, is still unknown. Results obtained from animal studies (mice) however, indicate direct inhibition of angiogenesis by interferon-alpha [23]. Other authors report a reduced motility of capillary endothelial cells [24]. Likewise, the inhibition of both the proliferation of smooth muscle cells and capillary endothelial cells could explain the effect of interferon-alpha in angiomatous neoplasms and malformations [25].

The response of tufted angioma to interferon therapy, however, varies greatly. Contrary to the almost complete remissions reported by Suarez et al. and Park et al. after interferon therapy, we observed only a partial remission [9, 19]. Munn et al. even reported a further progression of tufted angioma after an initial improvement [15]. A high-dose systemic steroid therapy, however, led to a good response in this case. In contrast, local application of clobetasol in the case described by Berstein et al. only reduced the tenderness and painfulness [8]. Léauté-Labrèze et al. treated a case of Kasabach-Merritt syndrome following congenital tufted angioma with acetylsalicylic acid [18]. As well as an increase in the thrombocyte cell count, the therapy also led to a dramatic reduction of the tumour. However, with interferon therapy, which was later administered into the remaining tumour, only a very slight improvement was achieved. Given the low number of case reports published so far, a general recommendation of interferon therapy is not yet justified, but it may be an alternative in cases where surgical treatment is not possible.

There have also been mixed results with laser therapy (argon-laser and dye-laser) [8, 16]. In our view, surgery is preferable in cases of smaller, circumscribed lesions, interferon alpha has a certain antiproliferative effect, but standardized treatments are not yet available.

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