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Congenital, Self-regressing Tufted Angioma
John Browning, MD;
Ilona Frieden, MD;
Eulalia Baselga, MD;
Annette Wagner, MD;
Denise Metry, MD
Arch Dermatol. 2006;142:749-751.
ABSTRACT
Background Tufted angioma (known in Japanese literature as angioblastoma of Nakagawa) is an uncommon, histologically benign, vascular tumor. Lesions typically present during infancy or early childhood and are most commonly reported to persist and/or expand over time. Congenital presentations are rare, as are reports of spontaneous regression.
Observations We present a series of 5 histopathologically confirmed cases of congenital tufted angioma that spontaneously regressed during infancy or early childhood. We also review the literature, focusing on both congenital and early-onset cases in infants.
Conclusion We recommend that observation for potential regression be considered for otherwise uncomplicated congenital or early infantile cases of tufted angioma.
INTRODUCTION
Tufted angioma (TA), also known in the Japanese literature as angioblastoma of Nakagawa, is an uncommon, histologically benign, vascular tumor that most often manifests during infancy or early childhood. It shows characteristic histopathologic findings, with lobules or tufts of capillaries in the dermis. Within the tufts are benign spindle cells, which appear to push against the adjacent vessels, giving them a slitlike appearance. On low-power microscopy, the tufts have the appearance of cannonballs in the dermis.
Most cases of TA are sporadic, although familial cases have been rarely reported.1-2 There is no sex predominance, and the clinical presentation is variable. Lesions most often present as solitary tumors or large, infiltrated plaques that are dusky red or violaceous in color. Other characteristic features include increased lanugo hair, an overlying port-wine–like stain, nodularity, or cobblestoning. Tufted angiomas are most commonly reported to persist, often slowly enlarging over years, and may be tender. Kasabach-Merritt phenomenon (KMP) seems to be a rare complication of TA, more common to the kaposiform hemangioendothelioma, although these 2 lesions are considered by many to be within the same spectrum.
Congenital cases of TA are unusual, as are reports of spontaneous regression. We present a series of 5 histopathologically confirmed cases of congenital TA, 4 of which completely regressed clinically during infancy and 1 that had partially regressed clinically by the time the child was 4 years old. We also review 10 prior reports of congenital or early-onset TA.
REPORT OF CASES
In Table 1 we present 5 new cases of congenital TA that spontaneously regressed, 4 during infancy and 1 by the time the child was 4 years old. Biopsy specimens were taken from all patients, and histopathologic findings from all specimens showed classic features of TA. Complete regression of the TA during infancy was observed in 4 patients, 3 of whom had residual skin changes. One patient exhibited partial regression by age 4 years. None of our cases was complicated by KMP. One patient received an intralesional corticosteroid injection whereas the other 4 were observed without intervention.
Ten reports of congenital or early-onset TA, all confirmed by histologic results, have been previously published in the literature3-7; 4 of the lesions were congenital, 5 developed within the first 3 months of life, and 1 was noted by age 9 months. Tenderness or pain was noted in all. None were complicated by KMP. Eight of the 10 patients showed complete or partial regression. The 2 lesions that did not regress were noted to be less tender over time.
COMMENT
Tufted angioma is uncommon but not rare; there are more than 200 reports in the English and Japanese literature.8 However, presentation at birth is unusual, and reports of spontaneously regressing TA even more so; there are only 4 cases confirmed by histologic results reported prior to the series described herein. Regression may be a phenomenon more common to lesions that present at birth or early infancy because there are even fewer reports of spontaneous regression in patients with later presentation. In contrast, to our knowledge there is only 1 report of a congenital TA that failed to improve over time.9 Other than early onset, we found no other clinical or histopathologic features more common to spontaneously regressing vs persistent lesions.
In the cases reported herein and in prior cases, regression occurred over months to a few years. Most cases completely regressed, whereas in 3 cases partial regression was observed, with decreased induration and tenderness. Prior to regressing, slow growth often occurs (Table 1). Three of our patients had residual skin changes following complete involution, although this finding is not described in previous reports and is common to other spontaneously regressing vascular tumors such as infantile hemangioma and rapidly involuting congenital hemangioma. One child had cutaneous atrophy with prominent vasculature following involution of the TA (Figure). Another child had a residual erythematous macule following involution. The third child had an overlying eczematous dermatitis after resolution of the TA. Although it is hypothesized that local secretion of growth factors important in angiogenesis, such as interleukin 8, play a role in the development of TA,10 the mechanism for regression is unknown.
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Figure. A, Tufted angioma (TA) involving the right temple at age 3 days; B, the same child at age 5 months following rapid involution of the TA with mild residual erythema, and C, at age 2 years with further resolution of the erythema, revealing cutaneous atrophy with prominent underlying vasculature.
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The differential diagnosis of TA includes infantile hemangioma, vascular (particularly venous) malformation, infantile myofibroma (hemangiopericytoma), and malignant vascular tumors, such as infantile fibrosarcoma. The congenital presentation of a TA makes it particularly challenging to differentiate clinically from rapidly involuting congenital hemangioma.11 Comparative clinical and histopathologic features of TAs and rapidly involuting congenital hemangiomas are listed in Table 2. Biopsy of tissue specimens is often necessary for definitive diagnosis of TA.
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Table 2. Comparision of Tufted Angioma and Rapidly Involuting Congenital Hemangioma
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Potential indications for treatment of TA, similar to those for other benign vascular tumors, include vital organ or functional compromise, significant symptoms, or need to improve appearance. We did not observe KMP in any of our cases or those reviewed; it seems to be a rare complication. Surgical excision is generally the treatment of choice for TA. For those lesions not amenable to surgery, particularly when complicated by KMP, medical therapy (generally systemic corticosteroids or vincristine sulfate) may be used.4
In conclusion, spontaneous regression of TA may occur and seems to be a phenomenon possibly more common to congenital or early infantile presentations. Whether such lesions represent the same entity as acquired, persistent cases or is a variant is unknown. We suggest that physicians consider observation for otherwise uncomplicated congenital or early infantile cases of TA before considering intervention, particularly surgical intervention.
AUTHOR INFORMATION
Correspondence: Denise Metry, MD, Department of Dermatology, One Baylor Plaza, Houston, TX 77030 (dwmetry{at}texaschildrenshospital.org).
Author Contributions: Study concept and design: Browning and Metry. Acquisition of data: Browning, Frieden, Baselga, Wagner, and Metry. Analysis and interpretation of data: Browning, Frieden, Wagner, and Metry. Drafting of the manuscript: Browning and Metry. Critical revision of the manuscript for important intellectual content: Browning, Frieden, Baselga, Wagner, and Metry. Study supervision: Browning, Wagner, and Metry.
Financial Disclosure: None.
Author Affiliations: Departments of Dermatology, Baylor College of Medicine, Houston, Tex (Drs Browning and Metry), University of California, San Francisco (Dr Frieden), Hospital de la Santa Creu I, Sant Pau, Barcelona, Spain (Dr Baselga), and Northwestern University, Evanston, Ill (Dr Wagner).
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