DOCSLIB.ORG

Hemangiomas: Update on Classification, Clinical Presentation, and Associated Anomalies Maria Garzon, MD, New York, New York

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Hemangiomas: Update on Classification, Clinical Presentation, and Associated Anomalies Maria Garzon, MD, New York, New York columbia contributions Hemangiomas: Update on Classification, Clinical Presentation, and Associated Anomalies Maria Garzon, MD, New York, New York Infantile hemangiomas occur in 10% of children and are 3 times more common in female infants.1,2 The majority of hemangiomas are small, superficial tumors that require little, if any, treatment. During the last several years, new information regarding the classification, presentation, associations, and differential diagnosis of hemangioma has emerged and altered the management of these tumors. The purpose of this article is to briefly review some of these clinically relevant findings. A discussion of the pathogenesis and management of these poten- tially problematic tumors is beyond the scope of this article, but these topics have been addressed FIGURE 1. 2-5 Macular telangiectatic hemangioma at in several excellent reviews. 2 months. Update on Classification—Hemangiomas basic fibroblast growth factor. Tissue inhibitor of Versus Vascular Malformations metalloproteinase, which acts to inhibit blood vessel In 1982, Mulliken and Glowacki6 published a bio- formation, is expressed during the involuting phase. logic classification for vascular birthmarks that Vascular malformations are structural anomalies distinguished hemangiomas from vascular malforma- believed to represent errors in the normal vascular tions based on clinical characteristics, histopatholo- morphogenesis that persist throughout a patient’s gy, and biologic behavior. Takahashi et al7 further lifetime. Vascular malformations are composed of investigated the cellular differences in 1994 and anomalous vascular channels demonstrating normal confirmed the distinct biologic characteristics of levels of endothelial cell turnover.7 Prior to the these 2 types of lesions. acceptance of this classification, many types of vascu- Hemangiomas are proliferating tumors composed lar anomalies, including those that were clearly mal- of endothelial cells that grow rapidly in the first year formations, were referred to as hemangiomas. By of life and spontaneously involute as childhood adopting this new classification, clinicians have been ensues. They are classified as superficial, deep, or able to more accurately address prognosis and ther- combined hemangiomas based on their clinical apy. Moreover, recognition of the differences appearance. Hemangiomas demonstrate increased between these 2 types of vascular birthmarks has cell turnover and express markers of proliferation, enabled researchers to investigate their pathogenesis including proliferating cell nuclear antigen, type IV more precisely. collagenase, vascular endothelial growth factor, and The biologic classification was updated in 1996 by Mulliken and other members of the International Society for the Study of Vascular Anomalies Dr. Garzon is from the Department of Dermatology, Columbia Uni- (ISSVA) to reflect the results of their studies.4 Inves- versity College of Physicians and Surgeons, New York, New York. REPRINT REQUESTS to Department of Dermatology, Columbia tigations indicated that vascular tumors resembling University, 161 Fort Washington Avenue, 14th Floor, New York, NY common hemangiomas could arise during infancy, 10032. but these lesions were distinct from hemangiomas in VOLUME 66, NOVEMBER 2000 325 HEMANGIOMA UPDATE FIGURE 2. Characteristic proliferation in portions of the FIGURE 3. Kasabach-Merritt syndrome associated with hemangioma at 4 months. tufted angioma (photo courtesy of I.J. Frieden, MD). their clinical characteristics, histopathologies, and genital hemangiomas from other vascular tumors or biologic behaviors. Consequently, the ISSVA or sarcomas that may arise in the neonatal period. The modified biological classification now defines both diagnosis of congenital hemangioma is usually made vascular tumors and vascular malformations. Vascular on physical examination, but may require the assis- tumors include common hemangioma of infancy, tance of magnetic resonance imaging (MRI) and kaposiform hemangioendothelioma (KHE), tufted Doppler ultrasonography. angioma hemangiopericytoma, spindle-cell heman- Other uncommon presentations of the common gioendothelioma, and pyogenic granuloma. Vascular hemangioma include superficial hemangiomas, malformations are divided into simple or combined which resemble capillary malformations, and deep lesions and are further categorized based on their pre- subcutaneous masses with normal overlying skin. The dominant anomalous channels: capillary, lymphatic, latter contrasts with the majority of deep heman- venous, or arterial. giomas that are associated with bluish discoloration, dilated veins, or superficial telangiectasia. MRI eval- Update on Clinical Presentation uation and Doppler ultrasonography may help to Classic descriptions of hemangiomas often state that confirm a diagnosis.12 they are rarely present at birth, but usually become apparent within the first few weeks of life. Precursor Associated Anomalies lesions frequently are unrecognized within the first The majority of syndromes reported with vascular few days of life. Early authors described these precur- birthmarks occurs in association with vascular sors as telangiectatic macules, areas of pallor, or malformations. Familiar eponyms assigned to these ecchymoses.8,9 The telangiectatic macules may be associations include Sturge-Weber and Klippel- mistaken for capillary malformations on an initial Trenaunay syndromes. It is uncommon to see heman- assessment, but subsequent proliferation confirms the giomas associated with malformations, but it is essential diagnosis of hemangioma (Figures 1 and 2). Superfi- to recognize the situations in which this may occur. cial ulceration in the absence of clinically apparent Large cervicofacial hemangiomas can be associ- hemangioma is an unusual precursor that may pre- ated with a variety of intracranial anomalies, includ- cede the development of a common hemangioma.10 ing structural malformations of the posterior fossa. An uncommon subset of fully developed heman- These malformations include the Dandy-Walker mal- giomas that may be recognized on routine prenatal formation, arachnoid cyst, enlarged cisterna magna, ultrasonography but that are rarely present at birth and atrophy or hypoplasia of the cerebellum.13 Anom- are called congenital hemangiomas. Congenital heman- alies of the internal carotid artery, posterior or anteri- giomas undergo proliferation during fetal life. At or cerebral artery, persistent trigeminal arteries, and birth, they are fully developed vascular masses that coarctation of the aorta also occur in this setting.14 present as violaceous tumors with ectatic veins, gray- Patients with large facial hemangiomas and violaceous tumors with overlying telangiectasia, or intracranial structural vascular anomalies may pale halo or flat infiltrative lesions with an overlying demonstrate progressive occlusive arterial changes violaceous discoloration of the skin. Accelerated, and cerebral infarction.15 spontaneous involution is a frequent occurrence in The acronym PHACE, which refers to Posterior these tumors.4,11 It is necessary to differentiate con- fossa malformations, Hemangiomas, Arterial anom- 326 CUTIS® HEMANGIOMA UPDATE alies, Coarctation of the aorta and cardiac defects, line a slitlike lumen. Often, KHE extends beyond the and Eye abnormalities,16 has been proposed to deep dermis into the subcutaneous fat and is accom- describe the setting in which numerous malforma- panied by lymphangiomatosis.24 tions coexist with large facial hemangioma. Sternal Enjolras et al22 reviewed 22 cases of patients with agenesis, hypoplasia, or supraumbilical raphe may Kasabach-Merritt syndrome. Histopathologic speci- also occur concomitantly with large facial heman- mens were available for 15 of the cases and none of giomas.17 Hemangiomas arising on the lower face in the patients demonstrated features of common what has been described as a “beard” distribution may hemangioma in infancy. Moreover, histopathology herald subglottic involvement and potential airway revealed KHE or tufted angioma in all of the cases.22 compromise. Large problematic facial hemangiomas Sarkar et al23 reviewed 21 cases of patients with appear to arise more commonly in female patients, Kasabach-Merritt syndrome for which histopathol- which shows an even greater female predilection ogic specimens were available for 16 patients. than hemangiomas in general.18,19 Hemangiomas aris- Although all of the patients were reported to have ing over the lumbo-sacral spine may also be associ- KHE, none demonstrated the clinical or histologic ated with congenital abnormalities of the features of common hemangioma. KHE may also dis- gastrointestinal and genitourinary systems. In addi- play a distinctive pattern on MRI.23 The first case of tion, they may herald underlying spinal dys- Kasabach-Merritt syndrome25 was reported to have raphism.20,21 similar clinical features and the histopathology described “spindled-cells,” which is not a feature of Kasabach-Merritt Syndrome and common hemangioma. The natural history of this Vascular Tumors tumor is unpredictable. Cases that are associated with Two recent reports22,23 support the finding that infants Kasabach-Merritt syndrome may respond poorly to with Kasabach-Merritt syndrome do not have ordi- treatment, and mortality rates between 20% and 30% nary infantile hemangiomas. This syndrome, which is are often cited.2 Improvement may occur after suc- characterized by a rapid enlargement of “heman- cessful treatment or
Load more

Recommended publications
  • Kaposiform Hemangioendothelioma with Kasabach-Merritt Syndrome Mistaken for Child Abuse in a Newborn
    Kaposiform Hemangioendothelioma With Kasabach-Merritt Syndrome Mistaken for Child Abuse in a Newborn Amanda A. Cyrulnik, MD; Manju C. Dawkins, MD; Gert J. Smalberger, MD; Scott Young, MD; Ranon E. Mann, MD; Mark I. Jacobson, MD; Adam J. Friedman, MD Practice Points Vascular tumors in dermatology may mimic child abuse. Even when all signs favor abuse, a nonresolving lesion should alarm clinicians to consider an alter- nate diagnosis. Kasabach-Merritt syndrome is a serious and potentially life-threatening condition that requires prompt evaluation. Kaposiform hemangioendotheliomaCUTIS is a rare vas- elevated D-dimer levels, confirming a diagnosis of cular neoplasm of childhood that may have an Kasabach-Merritt syndrome (KMS). alarming and potentially misleading clinical pre- Cutis. 2014;93:E17-E20. sentation. Awareness of this entity is important to provide appropriate and immediate medical care. Case Report We report the case of a 24-day-old female new- A 24-day-old female newborn presented to a hospi- born who presented with a large bruiselike lesion tal with a large bruiselike lesion on the left leg. A on theDo left leg. A diagnosis ofNot cellulitis suspected diagnosis Copy of cellulitis suspected to be secondary to to be secondary to child abuse was made and the child abuse was made and the patient subsequently patient subsequently was placed in foster care; was placed in foster care; however, the lesion did not however, the lesion did not resolve after treatment resolve after treatment and relocation. At 69 days of and relocation. On reevaluation at our institution, age, the patient was readmitted, now to our hospi- physical examination revealed a round, 34-cm, tal, after the lesion persisted and had progressively violaceous, indurated, fixed, nonblanching, non- expanded.
    [Show full text]
  • Benign Hemangiomas
    TUMORS OF BLOOD VESSELS CHARLES F. GESCHICKTER, M.D. (From tke Surgical Palkological Laboratory, Department of Surgery, Johns Hopkins Hospital and University) AND LOUISA E. KEASBEY, M.D. (Lancaster Gcaeral Hospital, Lancuster, Pennsylvania) Tumors of the blood vessels are perhaps as common as any form of neoplasm occurring in the human body. The greatest number of these lesions are benign angiomas of the body surfaces, small elevated red areas which remain without symptoms throughout life and are not subjected to treatment. Larger tumors of this type which undergb active growth after birth or which are situated about the face or oral cavity, where they constitute cosmetic defects, are more often the object of surgical removal. The majority of the vascular tumors clinically or pathologically studied fall into this latter group. Benign angiomas of similar pathologic nature occur in all of the internal viscera but are most common in the liver, where they are disclosed usually at autopsy. Angiomas of the bone, muscle, and the central nervous system are of less common occurrence, but, because of the symptoms produced, a higher percentage are available for study. Malignant lesions of the blood vessels are far more rare than was formerly supposed. An occasional angioma may metastasize following trauma or after repeated recurrences, but less than 1per cent of benign angiomas subjected to treatment fall into this group. I Primarily ma- lignant tumors of the vascular system-angiosarcomas-are equally rare. The pathological criteria for these growths have never been ade- quately established, and there is no general agreement as to this par- ticular form of tumor.
    [Show full text]
  • 8 Liver Hemangioma
    Liver Hemangioma 101 8 Liver Hemangioma Valérie Vilgrain and Giuseppe Brancatelli CONTENTS 8.1 Introduction 8.1 Introduction 101 8.2 Sonography 101 8.3 Computed Tomography 102 Hemangioma is the most common benign hepatic 8.4 Magnetic Resonance 104 tumor. The prevalence of hemangioma in the general 8.5 Scintigraphy 107 population ranges from 1%–2% to 20% (Semelka and 8.6 Percutaneous Biopsy 107 Sofka 1997). The female-to-male ratio varies from 8.7 Atypical Patterns 108 2:1 to 5:1. They occur at all ages. The vast majority of 8.7.1 Hemangioma with Echoic Border 108 8.7.2 Large, Heterogeneous Hemangioma 108 hemangiomas remain clinically silent. Few patients 8.7.3 Rapidly Filling Hemangioma 108 are symptomatic due to a mass lesion, complications 8.7.4 Very Slow Filling Hemangioma 110 or compression of adjacent structures. Most of these 8.7.5 Calcifi ed Hemangioma 110 symptoms are observed in large hemangiomas. The 8.7.6 Hyalinized Hemangioma 110 natural history of hemangiomas is variable: most of 8.7.7 Cystic or Multilocular Hemangioma 111 8.7.8 Hemangioma with Fluid–Fluid Level 111 them remain stable, some may grow or involute. The 8.7.9 Pedunculated Hemangioma 111 role of sex hormones in causing enlargement during 8.7.10 Hemangioma with pregnancy or recurrence is disputed. Arterial-Portal Venous Shunt 111 Hemangiomas are usually solitary, less than 5 cm 8.7.11 Hemangioma with Capsular Retraction 111 in size and appear as well-delineated lesions of red 8.8 Hemangioma Developing in Abnormal Liver 112 color that partially collapse on sectioning.
    [Show full text]
  • Vascular Tumors
    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.
    [Show full text]
  • Ill-Defined Macule on the Abdomen
    DERMATOPATHOLOGY DIAGNOSIS Ill-Defined Macule on the Abdomen Claire O. Dorfman, DO; Nektarios Lountzis, MD; Tanya Ermolovich, DO Eligible for 1 MOC SA Credit From the ABD This Dermatology Diagnosis in our print edition is eligible for 1 self-assessment credit for Maintenance of Certification from the American Board of Dermatology (ABD). After completing this activity, diplomates can visit the ABD website (http://www.abderm.org) to self-report the credits under the activity title “Cutis Dermatology Diagnosis.” You may report the credit after each activity is completed or after accumulating multiple credits. A 38-year-old woman presented with an asymp- tomatic lesion on the abdomen. On physical examination, there was a 5×2-mm, solitary, ill- defined pink macule on the right side of the abdomen. The patient denied recent change in size or color of the lesion, prior trauma, or a per- sonal or family history of similar lesions. Due to the uncertain diagnostic appearance, a punch biopsy was performed. THE BEST DIAGNOSIS IS: a. cutaneous angiosarcoma b. Kaposi sarcoma H&E, original magnification ×200 (inset, original magnification ×40). c. microvenular hemangioma d. targetoid hemosiderotic hemangioma e. tufted angioma PLEASE TURN TO PAGE 68 FOR THE DIAGNOSIS Dr. Dorfman is from Lehigh Valley Health Network, Allentown, Pennsylvania. Drs. Lountzis and Ermolovich are from Advanced Dermatology Associates, LTD, Allentown. The authors report no conflict of interest. Correspondence: Claire O. Dorfman, DO, 1259 S Cedar Crest Blvd, Ste 100, Allentown, PA 18103 ([email protected]). WWW.MDEDGE.COM/DERMATOLOGY VOL. 104 NO. 1 I JULY 2019 53 DERMATOPATHOLOGY DIAGNOSIS DISCUSSION THE DIAGNOSIS: Microvenular Hemangioma icrovenular hemangioma is an acquired benign vas- from pink patches to dark purple plaques or nodules that cular neoplasm that was described by Hunt et al1 in commonly occur on the lower legs10; however, the clinical M1991, though Bantel et al2 reported a similar entity appearance of KS varies depending on the clinical variant termed micropapillary angioma in 1989.
    [Show full text]
  • Vascular Lesions to Look out For
    10/25/19 Vascular Lesions to Look Out For Adnan Mir, MD, PhD Dermpath Diagnostics Port Chester, New York Assistant Professor Pediatric Dermatology and Dermatopathology Dermpath New York Medical College Diagnostics Disclosures I have no relevant financial conflicts of interest I will not mention off-label uses of FDA-approved medications 62 year old man Site: Left cheek Clinical: Facial fullness for seven months Asympatomatic R/o Morbihan, rosacea, seborrheic dermatitis 1 10/25/19 61 year old woman Site: Left cheek Clinical: Bleeding ulcer on abdomen Obesity, Pulmonary HTN Harker et al, J Cut Pathol 2017 Jan;44(1) 2 10/25/19 Cutaneous Angiosarcoma Angiosarcoma of the face/scalp first described in 1964 as malignant angioendothelioma of the skin 1% of sarcomas, 1/100,000 incidence Distinct behavior and presentation among angiosarcomas: Face, scalp – Wilson-Jones Lymphedema – Stewart-Treves Radiation-induced Epithelioid 3 10/25/19 Cutaneous Angiosarcoma Clinical presentation Typically asymptomatic Bruise-like Violaceous papules, plaques, nodules Clinical impressions often: Hematoma, rosacea, eczema, seborrheic dermatitis, hemangioma, xanthelasma, cellulitis, angioedema Cutaneous Angiosarcoma Epidemiology and predisposing factors Average age 70 M:F = 2:1 Chronic sun exposure? Overall, unknown etiology Cutaneous Angiosarcoma Pathophysiology Mutations detected in P53 (>50%) KRAS PTPRB Angiogenesis PLCG1 Upregulated: NTSR1, ANKRD1, CDKN2C, KDR 4 10/25/19 Cutaneous Angiosarcoma Histologic features Infiltrative growth pattern Well-differentiated
    [Show full text]
  • Diagnosis and Management of Infantile Hemangioma D
    CORE Metadata, citation and similar papers at core.ac.uk Provided by Hofstra Northwell Academic Works (Hofstra Northwell School of Medicine) Donald and Barbara Zucker School of Medicine Journal Articles Academic Works 2015 Diagnosis and Management of Infantile Hemangioma D. H. Darrow A. K. Greene A. J. Mancini A. J. Nopper T. M. O Northwell Health Follow this and additional works at: https://academicworks.medicine.hofstra.edu/articles Part of the Otolaryngology Commons Recommended Citation Darrow D, Greene A, Mancini A, Nopper A, O T. Diagnosis and Management of Infantile Hemangioma. 2015 Jan 01; 136(4):Article 575 [ p.]. Available from: https://academicworks.medicine.hofstra.edu/articles/575. Free full text article. This Article is brought to you for free and open access by Donald and Barbara Zucker School of Medicine Academic Works. It has been accepted for inclusion in Journal Articles by an authorized administrator of Donald and Barbara Zucker School of Medicine Academic Works. CLINICAL REPORT Guidance for the Clinician in Rendering Pediatric Care Diagnosis and Management of Infantile Hemangioma: Executive Summary David H. Darrow, MD, DDS, Arin K. Greene, MD, Anthony J. Mancini, MD, Amy J. Nopper, MD, the SECTION ON DERMATOLOGY, SECTION ON OTOLARYNGOLOGY–HEAD & NECK SURGERY, AND SECTION ON PLASTIC SURGERY INTRODUCTION Infantile hemangiomas (IHs) are the most common tumors of childhood. Unlike other tumors, they have the capacity to involute after proliferation, often leading primary care providers to assume they will resolve without intervention or consequence. However, a subset of IHs may be associated with complications, resulting in pain, functional impairment, or permanent disfigurement.
    [Show full text]
  • Diagnosis of Bacillary Angiomatosis and Kaposi´S Sarcoma in the Same Skin Lesion in a Patient with Recent HIV/AIDS Diagnosis
    Clinical Dermatology: Research and Therapy Open Access Case Report Diagnosis of Bacillary Angiomatosis and Kaposi´s Sarcoma in the same Skin Lesion in a Patient with Recent HIV/AIDS Diagnosis Correa J1,2*, Villafane MF1,2, De Carolis L1,2, Corti M1,2, Sforza R1, Maronna E3, Giamperetti S4 and Bare P5 1HIV/AIDS Division, Infectious Diseases F. J. Muniz Hospital, Argentina 2Department of Medicine, University of Buenos Aires, Argentina 3Histopathology Laboratory, Infectious Diseases F. J. Muniz Hospital, Argentina 4Zoopathology Laboratory, Infectious Diseases F. J. Muniz Hospital, Argentina 5Virology Laboratory, National Academy of Medicine, Argentina A R T I C L E I N F O A B S T R A C T Article history: Received: 21 September 2017 Kaposi´s Sarcoma (KS) is polyclonal multifocal disease of endothelial origin, Accepted: 27 November 2017 Published: 05 December 2017 with four clinical and epidemiological well described variants. AIDS-related Keywords: KS has a variable clinical course, ranging from minimal disease located to the Bacillary angiomatosis; Kaposi´s sarcoma; skin and presenting as an incidental finding to a rapidly progressing neoplasm HIV/AIDS with skin, mucosal, and visceral compromise that can result in significant Copyright: © 2017 Correa J et al., morbidity and mortality, depending upon the specific sites of involvement. Clin Dermatol Res Ther This is an open access article distributed Bacillary Angiomatosis (BA) is a rare manifestation of infection caused by under the Creative Commons Attribution License, which permits unrestricted use, Bartonella species, which leads to vasoproliferative lesions of skin and other distribution, and reproduction in any medium, provided the original work is organs.
    [Show full text]
  • Vascular Anomalies Classification: Recommendations from The
    Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies Michel Wassef, MDa, Francine Blei, MDb, Denise Adams, MDc, Ahmad Alomari, MDd, Eulalia Baselga, MDe, Alejandro Berenstein, MDf, Patricia Burrows, MDg, Ilona J. Frieden, MDh, Maria C. Garzon, MDi, Juan-Carlos Lopez-Gutierrez, MD, PhDj, David J.E. Lord, MDk, Sally Mitchel, MDl, Julie Powell, MDm, Julie Prendiville, MDn, Miikka Vikkula, MD, PhDo, on behalf of the ISSVA Board and Scientific Committee Vascular anomalies represent a spectrum of disorders from a simple “birthmark” abstract to life- threatening entities. Incorrect nomenclature and misdiagnoses are commonly experienced by patients with these anomalies. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring aAssistance Publique–Hopitaux de Paris, Lariboisière multidisciplinary specialists. Classification schemes provide a consistent Hospital, Department of Pathology, Paris Diderot University, Paris, France; bVascular Birthmark Program, Lenox Hill terminology and serve as a guide for pathologists, clinicians, and researchers. Hospital of North Shore Long Island Jewish Healthcare OneofthegoalsoftheInternationalSociety for the Study of Vascular Anomalies System, New York, New York; cCincinnati Children’s Hospital fi fi Medical Center, Cancer and Blood Disease Institute, University (ISSVA) is to achieve a uniform classi cation. The last classi cation (1997) of Cincinnati, Cincinnati, Ohio; dDepartment of Radiology, stratified vascular lesions
    [Show full text]
  • Vascular Malformations and Tumors Continues to Grow Overview Table Vascular Anomalies
    ISSVA classification for vascular anomalies © (Approved at the 20th ISSVA Workshop, Melbourne, April 2014, last revision May 2018) This classification is intended to evolve as our understanding of the biology and genetics of vascular malformations and tumors continues to grow Overview table Vascular anomalies Vascular tumors Vascular malformations of major named associated with Simple Combined ° vessels other anomalies Benign Capillary malformations CVM, CLM See details See list Lymphatic malformations LVM, CLVM Locally aggressive or borderline Venous malformations CAVM* Arteriovenous malformations* CLAVM* Malignant Arteriovenous fistula* others °defined as two or more vascular malformations found in one lesion * high-flow lesions A list of causal genes and related vascular anomalies is available in Appendix 2 The tumor or malformation nature or precise classification of some lesions is still unclear. These lesions appear in a separate provisional list. For more details, click1 on Abbreviations used the underlined links Back to ISSVA classification of vascular tumors 1a Type Alt overview for previous view Benign vascular tumors 1 Infantile hemangioma / Hemangioma of infancy see details Congenital hemangioma GNAQ / GNA11 Rapidly involuting (RICH) * Non-involuting (NICH) Partially involuting (PICH) Tufted angioma * ° GNA14 Spindle-cell hemangioma IDH1 / IDH2 Epithelioid hemangioma FOS Pyogenic granuloma (also known as lobular capillary hemangioma) BRAF / RAS / GNA14 Others see details * some lesions may be associated with thrombocytopenia
    [Show full text]
  • Diagnosis and Management of Infantile Hemangioma David H
    CLINICAL REPORT Guidance for the Clinician in Rendering Pediatric Care Diagnosis and Management of Infantile Hemangioma David H. Darrow, MD, DDS, Arin K. Greene, MD, Anthony J. Mancini, MD, Amy J. Nopper, MD, the SECTION ON DERMATOLOGY, SECTION ON OTOLARYNGOLOGY–HEAD AND NECK SURGERY, and SECTION ON PLASTIC SURGERY abstract Infantile hemangiomas (IHs) are the most common tumors of childhood. Unlike other tumors, they have the unique ability to involute after proliferation, often leading primary care providers to assume they will resolve without intervention or consequence. Unfortunately, a subset of IHs rapidly develop complications, resulting in pain, functional impairment, or permanent disfigurement. As a result, the primary clinician has the task of determining which lesions require early consultation with a specialist. Although several recent reviews have been published, this clinical report is the first based on input from individuals representing the many specialties involved in the treatment of IH. Its purpose is to update the pediatric community regarding recent discoveries in IH pathogenesis, treatment, and clinical associations and This document is copyrighted and is property of the American to provide a basis for clinical decision-making in the management of IH. Academy of Pediatrics and its Board of Directors. All authors have filed conflict of interest statements with the American Academy of Pediatrics. Any conflicts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any commercial involvement in the development of the content of this publication. NOMENCLATURE Clinical reports from the American Academy of Pediatrics benefit from The nomenclature and classification of vascular tumors and expertise and resources of liaisons and internal (American Academy malformations have evolved from clinical descriptions (“strawberry of Pediatrics) and external reviewers.
    [Show full text]
  • Infantile Hemangiomas: Our Current Understanding and Treatment Options
    Volume 24 Number 9| September 2018| Dermatology Online Journal || Review 24(9): 1 Infantile hemangiomas: our current understanding and treatment options Yssra S Soliman1 BA, Amor Khachemoune2,3 MD Affiliations: 1Albert Einstein College of Medicine, Bronx, New York, USA, 2State University of New York Downstate, Department of Dermatology, Brooklyn, New York, USA 3Veterans Health Administration, Department of Dermatology, Brooklyn, New York, USA Corresponding Author: Amor Khachemoune MD, FAAD, FACMS, Veterans Affairs Hospital & SUNY Downstate, Dermatology Service, 800 Poly Place, Brooklyn, NY 11209, Tel: 718-630-3725, Fax: 718-630-2881, Email: [email protected] or in visceral organs. Classically, the hemangiomas Abstract display rapid growth during the third month of life, Infantile hemangioma (IH) is the most common followed by slow involution [4]. By 48 months, vascular tumor of infancy, affecting up to 10% of all approximately 90% of cases will have regressed [5]. infants. Our understanding of IH and its management has greatly evolved. The etiology of IH is unclear but This article aims to review the epidemiology, clinical hypoxia is thought to play a key role. Furthermore, presentation, genetics, histopathology, associated GLUT1, IGF2, and HIF-1- syndromes, evolution without treatment, and important mediators. Current management options treatment options of infantile hemangioma. The include active observation, medical treatment, and primary database used was PubMed. We searched surgical intervention. The goals of treatment are , preventing cosmetic disfiguration, psychosocial , distress, and life-threatening complications. Infantile hemangioma should be managed with an individual, f patient-centered approach. Generally, uncompli- -1- cated IH can be observed up to 18 months. However, IH should be treated in the setting of bleeding, Historical note and evolving classification ulceration, functional compromise, or eventual Infantile hemangioma has been reported in the failure to regress.
    [Show full text]