A retrospective study: Clinicopathological and immunohistochemical analysis of 54 cases of tufted angioma.

BACKGROUND: Tufted angioma is a rare benign lesion with vascular proliferation. AIM: To retrospectively analyze the clinicopathological manifestations and immunohistochemical features of tufted angioma. METHODS: Clinical and histopathological features of tufted angioma (n = 54) were evaluated and analyzed retrospectively in the Department of Dermatology, Xijing Hospital from 2003 to 2014. RESULTS: Clinically, tufted angioma usually presented as erythematous plaques and papules on the head and neck (n = 11), trunk (n = 21) and extremities (n = 22), mainly in children (n = 48), without gender difference (24 males and 30 females). A total of 45 cases showed solitary lesions and nine cases showed multiple lesions. Common symptoms included pain (n = 11), tenderness (n = 7), itching (n = 1), hypertrichosis (n = 7), hyperhidrosis (n = 6) and Kasabach-Merritt phenomenon (n = 1). Histopathologically, typical tufted angioma (n = 37) showed proliferation of endothelial cells in a so-called cannonball pattern, while in the early (n = 4) and regressed (n = 13) stages the tufted appearance was not prominent. The proliferated endothelial cells were diffusely positive for CD31 and Wilms tumor 1, focally positive for D2-40 and Prox1, and negative for Glut-1. LIMITATIONS: Our research was confined to patients of Chinese origin and our sample size was limited. CONCLUSIONS: Tufted angioma is a rare vascular neoplasm with diverse clinical manifestations and unique pathological features. It should be recognized as a vascular tumor with lymphatic differentiation. We emphasize the importance of considering tufted angioma in the differential diagnoses of any congenital or acquired vascular tumor.

Characteristic patterns of segmental infantile hemangiomas in Indian children: A descriptive study.

BACKGROUND: Approximately 18% of infantile hemangiomas are segmental. These are larger than other infantile hemangiomas, associated with higher rate of complications and developmental anomalies, and often require treatment. They follow nonrandom patterns on the head and neck as well as extremities which are probably related to embryologic development. AIMS: Our study aimed to describe segmental patterns of infantile hemangiomas in Indian children, with associated anatomical abnormalities if any. METHODS: Over a 9-year period, 59 infants presenting with lesions classified as segmental infantile hemangiomas were evaluated and analyzed. Associated developmental anomalies were assessed and recorded. In addition, patterns of "indeterminate" infantile hemangiomas in another 43 patients were analyzed. RESULTS: There were 14 male and 45 female infants with an average birth weight of 2.7 ± 0.726 kg in our study; the average age at onset was 1 ± 1.25 months with most (50.8%) lesions localized to the head and neck area. Mapping of lesions showed that the most common facial segments involved were mandibular (33%) and maxillary (30%). However, additional repetitive patterns not previously described (such as an "inverted comma" pattern on the chest, bilateral neck involvement and unilateral labium involvement) were seen in our patients. Common local complications were ulceration (27%), amblyopia and nasal obstruction (3% each). Mapping of the additional 43 patients with indeterminate infantile hemangiomas also showed repetitive though incomplete patterns. LIMITATIONS: Relatively small number of patients. CONCLUSION: Segmental infantile hemangiomas present as large, distinctively patterned lesions, even on the trunk and genitalia. These patterns are probably based on embryologic developmental patterns. In addition, indeterminate lesions also showed distinctive repetitive patterns. Our study suggests that additional segments may need to be defined, particularly on the trunk and genital area.

Tufted angioma in ear auricle: importance of the differential diagnosis.

Tufted angioma is an acquired vascular proliferation with specific histological characteristics. The most common clinical features are erythematous macules. It occurs predominantly in children and young adults, especially in the chest and neck. We report the case of a male patient, 12 years old, with an increased right ear auricle associated with erythematous macules and increased local temperature, referred with a diagnosis of lepromatous leprosy; however, this diagnosis was questioned. Histopathology was consistent with tufted angioma.

Tufted angioma in ear auricle: importance of the differential diagnosis / Hemangioma em penacho no pavilhao auricular: importancia do diagnostico diferencial

Tufted angioma is an acquired vascular proliferation with specific histological characteristics. The most common clinical features are erythematous macules. It occurs predominantly in children and young adults, especially in the chest and neck. We report the case of a male patient, 12 years old, with an increased right ear auricle associated with erythematous macules and increased local temperature, referred with a diagnosis of lepromatous leprosy; however, this diagnosis was questioned. Histopathology was consistent with tufted angioma.

O Hemangioma em penacho é uma proliferação vascular adquirida de características histológicas peculiares. Sua característica clínica mais comum são máculas eritematosas. Ocorre predominantemente em crianças e adultos jovens, principalmente no tórax e pescoço. Relata-se o caso de paciente do sexo masculino, 12 anos, com aumento do pavilhão auricular direito associado a máculas eritematosas e aumento da temperatura local, encaminhado com diagnóstico de Hanseníase Virchowiana, entretanto, tal diagnóstico foi questionado. Histopatologia compatível com Hemangioma em penacho.

Infantile hemangioma: an update.

Infantile hemangiomas (IH) are neoplastic proliferations of endothelial cells, characterized by a period of growth after birth, and eventual spontaneous involution. The course can be uneventful, culminating in spontaneous resolution; or it may be marked by complications such as bleeding; ulceration; infection; visual, feeding and breathing compromise; cosmetic and life-threatening complications such as congestive heart failure. Recognition of associated syndromes and impending complications of hemangiomas is of utmost importance. Great advances have taken place in the nomenclature, pathogenesis, immunohistochemistry, diagnostic workup and management of hemangiomas in the recent years. This article reviews current advances in the understanding of the pathogenesis, diagnostic tools, medical and surgical modalities of treatment for infantile hemangiomas.

Poland syndrome (anomaly) with congenital hemangioma: a new association.

Unilateral defect of pectoral muscle and ipsilateral syndactyly constitute Poland syndrome. Absence or hypoplasia of the breast and nipple, axillary hair loss and dermatoglyphic abnormalities have also been reported in this syndrome. The primary defect could be in the development of the proximal subclavian artery with early deficit of blood flow to the distal limb and the pectoral region, resulting in partial loss of tissue in those regions. However, the association of congenital hemangioma with Poland sequence has not been observed so far. Such an association is being reported here in a 1-year-old infant, second-born of nonconsanguineous parents, who also had polydactyly instead of the documented syndactyly.

Vascular nevi in children.

BACKGROUND: Vascular nevi are cutaneous anomalies of angiogenesis and vasculogenesis resulting in various different clinical presentations. AIM: The purpose of our descriptive study was to observe the various types of vascular nevi in children and their features. METHODS: A total of 4256 pediatric cases attending the dermatology OPD during the study period from August 2002 to August 2004 were screened for vascular nevi. RESULTS: Out of these, 19 children (0.44%) had vascular nevi-17 hemangiomas of infancy (HOIs) and 2 port-wine stains. The mean age of the affected children was 1.3 years (ranging from 2.5 months to 8 years). There were 13 girls and 6 boys. Seventeen (89.5%) patients had progressing lesions and two (10.5%) had non-involuting ones. A solitary lesion was seen in ten (52.6%) cases and two to five lesions were present in five (26.3%) cases. The nevi were distributed over multiple sites in seven (36.8%) cases, the head and neck in six (31.6%) cases, the chest and lower limb in two (10.5%) cases each, and the upper limbs and genitalia in one (5.3%) case each. Among the hemangiomas of infancy, 15 (88.2%) cases of superficial type and 2 (11.8%) cases of deep type were seen. The cutaneous complications included ulceration in four cases and infection in one. CONCLUSIONS: HOIs were the most common vascular nevi of childhood.