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Semin intervent Radiol 2017; 34(03): 225-232
DOI: 10.1055/s-0037-1604295
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Classification of Vascular Anomalies: An Update

Jack E. Steiner
1   Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin
,
Beth A. Drolet
1   Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin
2   Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin
› Author Affiliations
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Publication History

Publication Date:
11 September 2017 (online)

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Abstract

Vascular anomalies present both a diagnostic and therapeutic challenge to physicians. Identification of these lesions is difficult due to their immense phenotypic variability, and naming conventions for vascular anomalies have historically been inconsistent. Terms such as “hemangioma” are informative when used correctly, but can cause confusion and miscommunication if applied indiscriminately to all vascular anomalies. Accuracy in classification is essential, as both disease course and therapeutic options differ greatly depending on the particular vascular anomaly present. In order for clinicians to properly diagnose and treat patients with these diseases, a unified nomenclature must be employed. This section provides an update on the current classification of vascular anomalies, with clinical descriptions of the most commonly encountered lesions, and clarifies the ambiguous nomenclature present in the existing literature.

Keywords

vascular anomalies - malformation - hemangioma - classification - interventional radiology
 

  • References

  • 1 Hassanein AH, Mulliken JB, Fishman SJ, Greene AK. Evaluation of terminology for vascular anomalies in current literature. Plast Reconstr Surg 2011; 127 (01) 347-351
    CrossrefPubMedGoogle Scholar
  • 2 Mulliken JB, Burrows PE, Fishman SJ. , Eds. Mulliken and Young's Vascular Anomalies: Hemangiomas and Malformations. 2nd ed. Oxford, United Kingdom: Oxford University Press; 2013
    Google Scholar
  • 3 Virchow R. Angioma in die Krankhaften Geschwülste. Berlin, Germany: Hirschwald; 1863
    Google Scholar
  • 4 Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982; 69 (03) 412-422
    CrossrefPubMedGoogle Scholar
  • 5 Enjolras O. Classification and management of the various superficial vascular anomalies: hemangiomas and vascular malformations. J Dermatol 1997; 24 (11) 701-710
    CrossrefPubMedGoogle Scholar
  • 6 Wassef M, Blei F, Adams D. , et al; ISSVA Board and Scientific Committee. Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics 2015; 136 (01) e203-e214
    CrossrefPubMedGoogle Scholar
  • 7 ISSVA Classification of Vascular Anomalies ©2014. International Society for the Study of Vascular Anomalies. Available at: issva.org/classification . Accessed on July 20, 2017
    PubMedGoogle Scholar
  • 8 Belov S. Anatomopathological classification of congenital vascular defects. Semin Vasc Surg 1993; 6 (04) 219-224
    PubMedGoogle Scholar
  • 9 Belov S. Classification of congenital vascular defects. Int Angiol 1990; 9 (03) 141-146
    PubMedGoogle Scholar
  • 10 Calonje JE. Hemangiomas. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. , eds. WHO classification of Tumors of Soft Tissue and Bone. Lyon, France: IARC Press; 2013: 138-140
    Google Scholar
  • 11 Kirkorian AY, Grossberg AL, Püttgen KB. Genetic basis for vascular anomalies. Semin Cutan Med Surg 2016; 35 (03) 128-136
    CrossrefPubMedGoogle Scholar
  • 12 Munden A, Butschek R, Tom WL. , et al. Prospective study of infantile haemangiomas: incidence, clinical characteristics and association with placental anomalies. Br J Dermatol 2014; 170 (04) 907-913
    CrossrefPubMedGoogle Scholar
  • 13 Kanada KN, Merin MR, Munden A, Friedlander SF. A prospective study of cutaneous findings in newborns in the United States: correlation with race, ethnicity, and gestational status using updated classification and nomenclature. J Pediatr 2012; 161 (02) 240-245
    CrossrefPubMedGoogle Scholar
  • 14 North PE, Waner M, Mizeracki A, Mihm Jr MC. GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol 2000; 31 (01) 11-22
    CrossrefPubMedGoogle Scholar
  • 15 Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996; 132 (03) 307-311
    CrossrefPubMedGoogle Scholar
  • 16 Iacobas I, Burrows PE, Frieden IJ. , et al. LUMBAR: association between cutaneous infantile hemangiomas of the lower body and regional congenital anomalies. J Pediatr 2010; 157 (05) 795-801.e1 , 7
    CrossrefPubMedGoogle Scholar
  • 17 North PE, Waner M, James CA, Mizeracki A, Frieden IJ, Mihm Jr MC. Congenital nonprogressive hemangioma: a distinct clinicopathologic entity unlike infantile hemangioma. Arch Dermatol 2001; 137 (12) 1607-1620
    PubMedGoogle Scholar
  • 18 Berenguer B, Mulliken JB, Enjolras O. , et al. Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Dev Pathol 2003; 6 (06) 495-510
    CrossrefPubMedGoogle Scholar
  • 19 Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE. Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg 2001; 107 (07) 1647-1654
    CrossrefPubMedGoogle Scholar
  • 20 Nasseri E, Piram M, McCuaig CC, Kokta V, Dubois J, Powell J. Partially involuting congenital hemangiomas: a report of 8 cases and review of the literature. J Am Acad Dermatol 2014; 70 (01) 75-79
    CrossrefPubMedGoogle Scholar
  • 21 Osio A, Fraitag S, Hadj-Rabia S, Bodemer C, de Prost Y, Hamel-Teillac D. Clinical spectrum of tufted angiomas in childhood: a report of 13 cases and a review of the literature. Arch Dermatol 2010; 146 (07) 758-763
    PubMedGoogle Scholar
  • 22 Arai E, Kuramochi A, Tsuchida T. , et al. Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. J Cutan Pathol 2006; 33 (07) 492-497
    CrossrefPubMedGoogle Scholar
  • 23 Le Huu AR, Jokinen CH, Rubin BP. , et al. Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. Am J Surg Pathol 2010; 34 (11) 1563-1573
    PubMedGoogle Scholar
  • 24 Alvarez-Mendoza A, Lourdes TS, Ridaura-Sanz C, Ruiz-Maldonado R. Histopathology of vascular lesions found in Kasabach-Merritt syndrome: review based on 13 cases. Pediatr Dev Pathol 2000; 3 (06) 556-560
    CrossrefPubMedGoogle Scholar
  • 25 Huntington JT, Jones C, Liebner DA, Chen JL, Pollock RE. Angiosarcoma: a rare malignancy with protean clinical presentations. J Surg Oncol 2015; 111 (08) 941-950
    CrossrefPubMedGoogle Scholar
  • 26 Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev 2014; 8 (02) 259
    PubMedGoogle Scholar
  • 27 Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg 1993; 91 (07) 1216-1230
    CrossrefPubMedGoogle Scholar
  • 28 Rozas-Muñoz E, Frieden IJ, Roé E, Puig L, Baselga E. Vascular stains: proposal for a clinical classification to improve diagnosis and management. Pediatr Dermatol 2016; 33 (06) 570-584
    CrossrefPubMedGoogle Scholar
  • 29 Boccardi D, Menni S, Ferraroni M. , et al. Birthmarks and transient skin lesions in newborns and their relationship to maternal factors: a preliminary report from northern Italy. Dermatology 2007; 215 (01) 53-58
    CrossrefPubMedGoogle Scholar
  • 30 Juern AM, Glick ZR, Drolet BA, Frieden IJ. Nevus simplex: a reconsideration of nomenclature, sites of involvement, and disease associations. J Am Acad Dermatol 2010; 63 (05) 805-814
    CrossrefPubMedGoogle Scholar
  • 31 Soblet J, Limaye N, Uebelhoer M, Boon LM, Vikkula M. Variable somatic TIE2 mutations in half of sporadic venous malformations. Mol Syndromol 2013; 4 (04) 179-183
    CrossrefPubMedGoogle Scholar
  • 32 Wouters V, Limaye N, Uebelhoer M. , et al. Hereditary cutaneomucosal venous malformations are caused by TIE2 mutations with widely variable hyper-phosphorylating effects. Eur J Hum Genet 2010; 18 (04) 414-420
    CrossrefPubMedGoogle Scholar
  • 33 Boon LM, Mulliken JB, Enjolras O, Vikkula M. Glomuvenous malformation (glomangioma) and venous malformation: distinct clinicopathologic and genetic entities. Arch Dermatol 2004; 140 (08) 971-976
    PubMedGoogle Scholar
  • 34 Ballieux F, Boon LM, Vikkula M. Blue bleb rubber nevus syndrome. Handb Clin Neurol 2015; 132: 223-230
    PubMedGoogle Scholar
  • 35 de Vos IJ, Vreeburg M, Koek GH, van Steensel MA. Review of familial cerebral cavernous malformations and report of seven additional families. Am J Med Genet A 2017; 173 (02) 338-351
    CrossrefPubMedGoogle Scholar
  • 36 Defnet AM, Bagrodia N, Hernandez SL, Gwilliam N, Kandel JJ. Pediatric lymphatic malformations: evolving understanding and therapeutic options. Pediatr Surg Int 2016; 32 (05) 425-433
    CrossrefPubMedGoogle Scholar
  • 37 Elluru RG, Balakrishnan K, Padua HM. Lymphatic malformations: diagnosis and management. Semin Pediatr Surg 2014; 23 (04) 178-185
    CrossrefPubMedGoogle Scholar
  • 38 Rockson SG. Lymphedema. Am J Med 2001; 110 (04) 288-295
    CrossrefPubMedGoogle Scholar
  • 39 Colletti G, Valassina D, Bertossi D, Melchiorre F, Vercellio G, Brusati R. Contemporary management of vascular malformations. J Oral Maxillofac Surg 2014; 72 (03) 510-528
    CrossrefPubMedGoogle Scholar
  • 40 Couto JA, Vivero MP, Kozakewich HP. , et al. A somatic MAP3K3 mutation is associated with verrucous venous malformation. Am J Hum Genet 2015; 96 (03) 480-486
    CrossrefPubMedGoogle Scholar
  • 41 Kurek KC, Howard E, Tennant LB. , et al. PTEN hamartoma of soft tissue: a distinctive lesion in PTEN syndromes. Am J Surg Pathol 2012; 36 (05) 671-687
    CrossrefPubMedGoogle Scholar