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49 Vascular Tumors Abel Sepulveda, MD1 Edward P. Buchanan, MD1 1 Division of Plastic Surgery, Baylor College of Medicine, Address for correspondence Edward P. Buchanan, MD, Division of Houston, Texas Plastic Surgery, Baylor College of Medicine,6701 Fannin St. Suite 610.00, Houston, TX 77030 (e-mail: [email protected]). Semin Plast Surg 2014;28:49–57. Abstract Vascular anomalies are divided into two main groups: tumors and malformations. Vascular tumors are a large and complex group of lesions, especially for clinicians with Keywords none or little experience in this field. In the past, these lesions caused a great deal of ► vascular anomalies confusion because many appear analogous to the naked eye. Thankfully, recent ► vascular tumor advances in diagnostic techniques have helped the medical community to enhance ► hemangioma our comprehension, accurately label, diagnose, and treat these lesions. In this article, we ► vascular will review the most frequent vascular tumors and provide the reader with the tools to malformation properly label, diagnose, and manage these complex lesions. Vascular anomalies can be broken down into two major The word “birthmark” was one of the first ways used to categories: tumors and malformations. These clinical entities described vascular anomalies, based on the folk belief that a are extremely different, but often confused, as evidenced by mother’s emotions can leave an imprint on her unborn fetus.2 the history and medical literature. A thorough medical histo- In the late 1860s, Dugas and Fisher disproved this belief and ry and physical exam is essential when it comes to defining concluded that birthmarks were malformations resulting any vascular anomaly. There are several different vascular from flawed embryologic development.2 Around the mid- tumors, but infantile hemangiomas are the most common. 19th century, Virchow proposed a histopathologic classifica- Others tumors that will be discussed include tuft angiomas, tion based on the size and appearance of the vessels.3 Then in pyogenic granulomas, angiosarcomas, and kaposiform he- 1982, thanks to technologic advances Mulliken and Glowacki mangioendotheliomas. Management of vascular tumors de- classified them based on histology, biology, and clinical pends on their medical history, diagnosis, and most presentation.3 They divided vascular anomalies into two – importantly their location. major categories: tumors and malformations.2 8 In 1996, the International Society for the Study of Vascular Anomalies fi ’ History and Classification (ISSVA) adopted and modi ed Mulliken and Glowacki sorigi- nal classification.2 This classification system is now widely Pathologists, clinicians, and radiologists have traditionally accepted to properly diagnose and manage vascular anoma- confused the diagnosis of vascular anomalies by using medi- lies (►Table 1).2,4 cal terminology that was not specificordefined. Given this In general, the suffix “-oma” is usually reserved for benign inconsistency, the proper diagnosis of many of these tumors endothelial neoplasms that grow by endothelial hyperpla- has been confusing for a great number of care providers. In a sia.4,6 All vascular tumors (except congenital hemangiomas) This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. recent study, PubMed was queried for publications with the are not clinically present at birth, have a period of rapid word “hemangioma” in either the title or abstract. In the 320 growth, and spontaneously involute (except noninvoluting articles found, hemangioma was incorrectly used in 228 congenital hemangiomas [NICH]). A malformation is charac- (71%).1 In the articles that included management recommen- terized as an error in development of vascular embryologic dations, they found that patients were incorrectly treated in tissue. Vascular malformations can be capillary, venous, 13 of 63 (21%) articles that used improper terminology, and lymphatic, and/or arterial in nature. They differentiate from zero of 42 who used the proper terminology.1 vascular tumors by the fact that they are present at birth, do Issue Theme Vascular Anomalies; Copyright © 2014 by Thieme Medical DOI http://dx.doi.org/ Guest Editor, Edward I. Lee, MD Publishers, Inc., 333 Seventh Avenue, 10.1055/s-0034-1376260. New York, NY 10001, USA. ISSN 1535-2188. Tel: +1(212) 584-4662. 50 Vascular Tumors Sepulveda, Buchanan Table 1 International Society for the Study of Vascular Anomalies classification of vascular anomalies Vascular tumors Vascular malformations Infantile hemangioma Slow-flow: Congenital hemangioma Capillary malformation Rapidly involuting congenital hemangioma Venous malformation Noninvoluting congenital hemangioma Lymphatic malformation Tufted angioma (Æ Kasabach-Merritt phenomenon (KMP) Fast-flow: Kaposiform Hemangioendothelioma (Æ KMP) Arterial malformation Spindle cell hemangioendothelioma Arteriovenous malformation Epithelioid hemangioendothelioma Combined vascular malformation Other rare hemangioendotheliomas, i.e., composite, retiform, and others (a mix of any of the above) Dermatologic acquired, i.e., lobular capillary hemangioma (pyogenic granuloma) Angiosarcoma not have increased endothelial cell turnover, and grow pro- rupted placental tissue embedded in fetal soft tissue. This is portionally with the child. An overview of the current clinical, based on the presence of GLUT 1, Lewis Y antigen, and IGF2 in histologic, and immunohistotypical features that distinguish affected patients.6,10 These markers are shared by placental the major types of vascular tumors presenting in infancy and capillaries, rather than normal cutaneous vasculature or childhood is provided. Diagnostic modalities and current other vascular tumors.11 The second theory suggests that management practices will be discussed. they arise from endothelial progenitor and stem cells because these cells are found circulating in patients with IHs.6,10,12 Infantile Hemangiomas Infantile hemangiomas (IHs) are the most common benign Clinical Presentation tumor in infancy, and occur approximately in 5% to 10% of the At birth, the majority of IHs are not visible. A precursor lesion population (►Fig. 1).2,4,7,9 These can appear anywhere in the may be present in some cases, a superficial area of pallor often body, but most commonly they affect the skin, especially of with fine thread-like telangiectasia throughout.7 Infantile the head and neck (60%), trunk (25%), and extremities hemangiomas normally appear weeks after birth during (15%).2,4,9 The majority appear weeks to months after birth, the beginning of the proliferative phase. Superficial IHs but 30% to 50% can present with a precursor lesion at involve the superficial dermis, and grossly appear as lobulat- birth.7,9 Infantile hemangiomas are more prevalent in Cau- ed, bright red lesions with a thin, delicate-surface epitheli- casians, females, premature infants (birth weight < 1kg),and um.6,7,9,10,12 Deep IHs tend to appear later and involve the children whose mothers have had in utero procedures (e.g., deep dermis and subcutaneous tissue. The epidermis retains chorionic villus sampling).2,3,5,7 Infantile hemangiomas un- its normal thickness and the overlying skin often has a bluish dergo three distinctive phases—proliferation, involution, and hue.2,7,13 Infantile hemangiomas can be localized or segmen- involuted phase (►Fig. 1). tal, and present as solitary (85%) or multiple (15%) lesions.2,4,7,9 Large segmental IHs have been associated Pathogenesis with structural anomalies, such as Dandy-Walker malforma- There are two dominant theories regarding the etiology of tion, arterial anomalies, spinal cord tethering, genitourinary IHs. The first suggests IHs’ endothelial cells arise from dis- anomalies, and subglottic hemangiomas.7 Large preauricular segmental IHs usually affect the parotid gland, but rarely affect the facial nerve. Multiple focal IHs have been associated with a higher risk of visceral hemangiomas, most notably affecting the liver, gastrointestinal system, and lungs.7 This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited. Proliferative Phase During the proliferative phase, which usually peaks around the third or fourth months after birth, IHs grow at a very rapid rate. It is during proliferation that ulceration, the most common complication associated with IHs, usually oc- curs.4,13,14 Depending on the location, IHs may present differently. Microscopically, lesions contain mitotically active cellular masses of plumped endothelial cells and pericytes, forming packed sinusoidal capillaries with small rounded lamina. There is marked cellularity with a jigsaw-like appear- ance. The blood vessels are small and difficult to find. Mast cells are prominent, with up to 40 times the normal levels.2,6 Fig. 1 Infantile hemangioma. They are believed to play a major role in proliferation. Seminars in Plastic Surgery Vol. 28 No. 2/2014 Vascular Tumors Sepulveda, Buchanan 51 Infantile hemangiomas continue to grow in the first year of Imaging is useful in questionable lesions either to demon- life, and usually reach a maximum size by the end of the first strate visceral involvement, plan surgical excision, assess or second year of life. On clinical exam, palpation at this stage treatment efficacy, and/or define associated anomalous struc- reveals a tense, rubbery, and noncompressible mass with tures. The best imaging technique to diagnose vascular draining veins at the periphery.2,3 anomalies is magnetic resonance imaging (MRI).2,3,7,9,12
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