Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex.

BACKGROUND: Hemangiomas of infancy vary widely in appearance, size, and depth of cutaneous involvement. There is currently no standard classification system for these lesions. While they occur in any race, an increased incidence occurs in girls, light-skinned whites, and premature infants, especially those weighing less than 1500 g. Other epidemiologic and demographic factors have not been well characterized. OBJECTIVE: To determine any correlations between hemangioma subtype and anatomic location with demographic factors, complications, and other associated anomalies. DESIGN: Retrospective chart review of 327 patients with hemangioma of infancy seen between 1997 and 2000 in an ambulatory referral center. MAIN OUTCOMES MEASURES: Demographic and gestational information, lesion size, associated anomalies, complications, treatments, and outcomes were analyzed together with classification of hemangiomas into 4 groups: localized, segmental, indeterminate, and multifocal. Subtypes were correlated with race and ethnicity, the incidence of complications, and overall outcome. RESULTS: Of 472 hemangiomas (327 patients), 339 (72%) were localized, 84 (18%) were segmental, 37 (8%) were indeterminate, and 12 (3%) were multifocal (8 or more noncontiguous lesions). Segmental lesions were larger and were more frequently associated with developmental abnormalities. They also required more intensive and prolonged therapy and were associated with more complications and a poorer overall outcome (P<.001). Lesions on Hispanic patients were more likely to involve mucous membranes, to be segmental (P<.004), to be associated with abnormalities (P =.05), especially PHACE syndrome (P =.05), and to have more complications (P =.01). Increased incidence of segmental hemangiomas was the only factor in Hispanic infants associated with complications, more extensive treatment, or associated anomalies. CONCLUSIONS: Hemangiomas of infancy can usually be classified as localized, segmental, indeterminate, and multifocal, based on clinical features. Segmental lesions have a higher frequency of complications and associated abnormalities, and this type of hemangioma seems to present with increased frequency in Hispanic infants.

Molecular pathogenesis of vascular anomalies: classification into three categories based upon clinical and biochemical characteristics.

Vascular tumors and malformations can be challenging to diagnose. Although they can resemble one another, their classification into tumors, such as hemangiomas of infancy, and malformations, such as venous or arteriovenous malformations, is based not only on their divergent biological behavior, but also on their pathogenesis. This review examines the molecular pathobiology of the processes involved in the development of these vascular birthmarks as they are currently understood. The terms hemangioma, hemangiosarcoma, and vascular proliferation are often used interchangeably, even though these entities are clinically and biochemically distinct. A more precise classification is necessary to facilitate communication between basic scientists and clinicians. Vasculogenesis, the in situ differentiation of blood vessels, occurs very early in the developing embryo. In vivo and in vitro studies, as well as knockout models, seem to indicate that this mechanism is unlikely to be involved in the development of either vascular malformations or hemangiomas of infancy. Recent advances in embryonic angiogenesis, especially explorations of mechanisms of vascular remodeling, have brought new understanding of the pathogenesis of vascular malformations. Vascular remodeling, an integral part of angiogenesis that centers upon the interactions between pericytes and endothelial cells, has been shown to be defective in certain experimental models and in some familial cases of vascular malformation. The occurrences of arteriovenous malformations in territories susceptible to increased remodeling also point towards epigenetic events in the development of vascular malformations.

Myofibrosarcoma treated with Mohs micrographic surgery.

BACKGROUND: Myofibrosarcoma is a rare malignant mesenchymal tumor composed predominantly of differentiated myofibroblasts. These tumors occur in both children and adults alike and are most commonly located on the head and neck. Recurrences rates range from 44% to 75%, and metastatic disease has been reported in up to 44% of cases. OBJECTIVE: The objective was to present a case of a myofibrosarcoma treated with Mohs micrographic surgery and discuss the use of ultrastructural evaluation in the diagnosis of this rare tumor. METHODS: A 31-year-old African American woman who presented to the Emory University Dermatologic Surgery Clinic with a 4-month history of a 2.5 x 2.5-cm indurated firm painful right upper lateral thigh nodule. A prior biopsy revealed a proliferation of somewhat bland spindled cells with large zones of necrosis with prominent mitotic figures, changes compatible with a cellular dermatofibroma. Because the lesion exhibited clinically suspicious characteristics such as rapid growth and deep infiltration, the patient was subsequently referred to Emory for further evaluation. There was no evidence of lymphadenopathy and a chest X-ray was unremarkable. RESULTS: A two-staged (five and four sections, respectively) uneventful Mohs micrographic surgery procedure was performed resulting in a defect measuring 3.5 x 3.5 x 1.0 cm. Primary closure was achieved with no complication, and the final scar measured 10 cm. Because of the suspicious clinical behavior of this tumor debulking specimen was sent for permanent section. Histopathologic interpretation of these sections was consistent with a fibrosarcoma with myofibroblastic differentiation. No clinical recurrence noted after 14-month follow-up. CONCLUSION: Mohs micrographic surgery is a technique that has been shown to provide superior cure rates in the treatment of many mesenchymal tumors. Here, we report the first case of myofibrosarcoma treated with Mohs micrographic surgery. Myofibrosarcoma is a rare but aggressive tumor that can be difficult to distinguish from other somewhat less aggressive malignancies such as dermatofibrosarcoma protuberans or malignant fibrous histiocytoma. Specific histopathologic criteria are reviewed. We recommend including Mohs micrographic surgery in the armamentarium for the treatment of this rare tumor.