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Cutaneous Vascular Lesions

  • Darya BuehlerEmail author
  • Steven D. Billings
Chapter

Abstract

Cutaneous vascular lesions comprise a wide spectrum of entities. Most are benign, but vascular tumors of intermediate malignancy, with a propensity for recurrence but limited metastatic potential, and fully malignant vascular neoplasms are encountered. Many of these vascular proliferations demonstrate significant histologic overlap. However, careful attention to histomorphology and in some cases, the application of ancillary diagnostic tests, usually result in proper classification in all but the most difficult of cases. This chapter will review the wide range of vascular lesions encountered in dermatopathology practice.

Keywords

Hemangioma Vascular malformation Pyogenic granuloma Lymphatic malformation Cutaneous epithelioid angiomatous nodule Hemangioendothelioma Angiosarcoma Atypical vascular lesion Kaposi sarcoma 

Selected References

  1. 1.
    Antonescu CR, Le Loarer F, Mosquera JM, Sboner A, Zhang L, Chen CL, Chen HW, Pathan N, Krausz T, Dickson BC, Weinreb I, Rubin MA, Hameed M, Fletcher CD. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosomes Cancer. 2013;52(8):775–84.PubMedCentralPubMedGoogle Scholar
  2. 2.
    Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML. Somatic activating mutations in GNAQ and GNA11 are associated with congenital hemangioma. Am J Hum Genet. 2016;98(4):789–95.PubMedCentralPubMedGoogle Scholar
  3. 3.
    Billings SD, Folpe AL, Weiss SW. Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol. 2003;27(1):48–57.PubMedGoogle Scholar
  4. 4.
    Billings SD, McKenney JK, Folpe AL, Hardacre MC, Weiss SW. Cutaneous angiosarcoma following breast-conserving surgery and radiation: an analysis of 27 cases. Am J Surg Pathol. 2004;28(6):781–8.PubMedGoogle Scholar
  5. 5.
    Brenn T, Fletcher CD. Cutaneous epithelioid angiomatous nodule: a distinct lesion in the morphologic spectrum of epithelioid vascular tumors. Am J Dermatopathol. 2004;26(1):14–21.PubMedGoogle Scholar
  6. 6.
    Brenn T, Fletcher CD. Radiation-associated cutaneous atypical vascular lesions and angiosarcoma: clinicopathologic analysis of 42 cases. Am J Surg Pathol. 2005;29(8):983–96.PubMedGoogle Scholar
  7. 7.
    Calonje E, Fletcher CD, Wilson-Jones E, Rosai J. Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol. 1994;18(2):115–25.PubMedGoogle Scholar
  8. 8.
    Couto JA, Vivero MP, Kozakewich HP, Taghinia AH, Mulliken JB, Warman ML, Greene AK. A somatic MAP3K3 mutation is associated with verrucous venous malformation. Am J Hum Genet. 2015;96(3):480–6.PubMedCentralPubMedGoogle Scholar
  9. 9.
    Deyrup AT, Tighiouart M, Montag AG, Weiss SW. Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol. 2008;32(6):924–7.PubMedGoogle Scholar
  10. 10.
    Doyle LA, Fletcher CD, Hornick JL. Nuclear expression of CAMTA1 distinguishes epithelioid Hemangioendothelioma from histologic mimics. Am J Surg Pathol. 2016;40(1):94–102.PubMedCentralPubMedGoogle Scholar
  11. 11.
    Duprez R, Lacoste V, Brière J, Couppié P, Frances C, Sainte-Marie D, Kassa-Kelembho E, Lando MJ, Essame Oyono JL, Nkegoum B, Hbid O, Mahé A, Lebbé C, Tortevoye P, Huerre M, Gessain A. Evidence for a multiclonal origin of multicentric advanced lesions of Kaposi sarcoma. J Natl Cancer Inst. 2007;99(14):1086–94.PubMedGoogle Scholar
  12. 12.
    Fanburg-Smith JC, Michal M, Partanen TA, Alitalo K, Miettinen M. Papillary intralymphatic angioendothelioma (PILA): a report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels. Am J Surg Pathol. 1999;23(9):1004–10.PubMedGoogle Scholar
  13. 13.
    Fernandez AP, Sun Y, Tubbs RR, Goldblum JR, Billings SD. FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations. J Cutan Pathol. 2012;39(2):234–42.PubMedGoogle Scholar
  14. 14.
    Fineberg S, Rosen PP. Cutaneous angiosarcoma and atypical vascular lesions of the skin and breast after radiation therapy for breast carcinoma. Am J Clin Pathol. 1994;102(6):757–63.PubMedGoogle Scholar
  15. 15.
    Grayson W, Pantanowitz L. Histological variants of cutaneous Kaposi sarcoma. Diagn Pathol. 2008;3:31.PubMedCentralPubMedGoogle Scholar
  16. 16.
    Gurbuz Y, Muezzinoglu B, Apaydin R, Yumbul AZ. Acral arteriovenous tumor (cirsoid aneurysm): clinical and histopathological analysis of 6 cases. Adv Clin Pathol. 2002;6(1):25–9.Google Scholar
  17. 17.
    Hornick JL, Fletcher CD. Pseudomyogenic hemangioendothelioma: a distinctive, often multicentric tumor with indolent behavior. Am J Surg Pathol. 2011;35(2):190–201.PubMedGoogle Scholar
  18. 18.
    Huang SC, Zhang L, Sung YS, Chen CL, Krausz T, Dickson BC, Kao YC, Agaram NP, Fletcher CD, Antonescu CR. Frequent FOS gene rearrangements in epithelioid hemangioma: a molecular study of 58 cases with morphologic reappraisal. Am J Surg Pathol. 2015;39(10):1313–21.PubMedCentralPubMedGoogle Scholar
  19. 19.
    Hung YP, Fletcher CD, Hornick JL. FOSB is a useful diagnostic marker for pseudomyogenic hemangioendothelioma. Am J Surg Pathol. 2017;41(5):596–606.PubMedCentralPubMedGoogle Scholar
  20. 20.
    ISSVA Classification of Vascular Anomalies ©2014 International Society for the Study of Vascular Anomalies. Available at issva.org/classification. Accessed 1 Nov 2017.
  21. 21.
    Ji Y, Chen S, Xiang B, Li K, Xu Z, Yao W, Lu G, Liu X, Xia C, Wang Q, Li Y, Wang C, Yang K, Yang G, Tang X, Xu T, Wu H. Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: a multicenter retrospective study. Int J Cancer. 2017;141(4):848–55.PubMedGoogle Scholar
  22. 22.
    Joethy J, Al Jajeh I, Tay SC. Intravenous pyogenic granuloma of the hand – a case report. Hand Surg. 2011;16(1):87–9.PubMedGoogle Scholar
  23. 23.
    Ko JS, Billings SD. Diagnostically challenging epithelioid vascular tumors. Surg Pathol Clin. 2015;8(3):331–51.PubMedGoogle Scholar
  24. 24.
    Kurek KC, Pansuriya TC, van Ruler MA, van den Akker B, Luks VL, Verbeke SL, Kozakewich HP, Sciot R, Lev D, Lazar AJ, Fletcher CD, Bovée JV. R132C IDH1 mutations are found in spindle cell hemangiomas and not in other vascular tumors or malformations. Am J Pathol. 2013;182(5):1494–500.PubMedGoogle Scholar
  25. 25.
    Lawley LP, Cerimele F, Weiss SW, North P, Cohen C, Kozakewich HP, Mulliken JB, Arbiser JL. Expression of Wilms tumor 1 gene distinguishes vascular malformations from proliferative endothelial lesions. Arch Dermatol. 2005;141(10):1297–300.PubMedGoogle Scholar
  26. 26.
    Le Huu AR, Jokinen CH, Rubin BP, Mihm MC, Weiss SW, North PE, Dadras SS. Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. Am J Surg Pathol. 2010;34(11):1563–73.PubMedGoogle Scholar
  27. 27.
    Limaye N, Wouters V, Uebelhoer M, Tuominen M, Wirkkala R, Mulliken JB, Eklund L, Boon LM, Vikkula M. Somatic mutations in angiopoietin receptor gene TEK cause solitary and multiple sporadic venous malformations. Nat Genet. 2009;41(1):118–24.PubMedGoogle Scholar
  28. 28.
    Lyons LL, North PE, Mac-Moune Lai F, Stoler MH, Folpe AL, Weiss SW. Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol. 2004;28(5):559–68.PubMedGoogle Scholar
  29. 29.
    Martorell-Calatayud A, Balmer N, Sanmartín O, Díaz-Recuero JL, Sangueza OP. Definition of the features of acquired elastotic hemangioma reporting the clinical and histopathological characteristics of 14 patients. J Cutan Pathol. 2010;37(4):460–4.PubMedGoogle Scholar
  30. 30.
    Marušić Z, Billings SD. Histopathology of spindle cell vascular tumors. Surg Pathol Clin. 2017;10(2):345–66.PubMedGoogle Scholar
  31. 31.
    McMenamin ME, Fletcher CD. Reactive angioendotheliomatosis: a study of 15 cases demonstrating a wide clinicopathologic spectrum. Am J Surg Pathol. 2002;26(6):685–97.PubMedGoogle Scholar
  32. 32.
    Napekoski KM, Fernandez AP, Billings SD. Microvenular hemangioma: a clinicopathologic review of 13 cases. J Cutan Pathol. 2014;41(11):816–22.PubMedGoogle Scholar
  33. 33.
    Nätynki M, Kangas J, Miinalainen I, Sormunen R, Pietilä R, Soblet J, Boon LM, Vikkula M, Limaye N, Eklund L. Common and specific effects of TIE2 mutations causing venous malformations. Hum Mol Genet. 2015;24(22):6374–89.PubMedCentralPubMedGoogle Scholar
  34. 34.
    Nayler SJ, Rubin BP, Calonje E, Chan JK, Fletcher CD. Composite hemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma. Am J Surg Pathol. 2000;24(3):352–61.PubMedGoogle Scholar
  35. 35.
    North PE. Pediatric vascular tumors and malformations. Surg Pathol Clin. 2010;3(3):455–94.PubMedGoogle Scholar
  36. 36.
    North PE, Waner M, Mizeracki A, Mihm MC Jr. GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol. 2000;31(1):11–22.PubMedGoogle Scholar
  37. 37.
    North PE, Waner M, Mizeracki A, Mrak RE, Nicholas R, Kincannon J, Suen JY, Mihm MC Jr. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol. 2001;137(5):559–70.PubMedGoogle Scholar
  38. 38.
    O'Donnell PJ, Pantanowitz L, Grayson W. Unique histologic variants of cutaneous Kaposi sarcoma. Am J Dermatopathol. 2010;32(3):244–50.PubMedGoogle Scholar
  39. 39.
    Parsons A, Sheehan DJ, Sangueza OP. Retiform hemangioendotheliomas usually do not express D2-40 and VEGFR-3. Am J Dermatopathol. 2008;30(1):31–3.PubMedGoogle Scholar
  40. 40.
    Patton KT, Deyrup AT, Weiss SW. Atypical vascular lesions after surgery and radiation of the breast: a clinicopathologic study of 32 cases analyzing histologic heterogeneity and association with angiosarcoma. Am J Surg Pathol. 2008;32(6):943–50.PubMedGoogle Scholar
  41. 41.
    Perez MC, Padhya TA, Messina JL, Jackson RS, Gonzalez RJ, Bui MM, Letson GD, Cruse CW, Lavey RS, Cheong D, Forster MR, Fulp WJ, Sondak VK, Zager JS. Cutaneous angiosarcoma: a single-institution experience. Ann Surg Oncol. 2013;20(11):3391–7.PubMedCentralPubMedGoogle Scholar
  42. 42.
    Perkins P, Weiss SW. Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior. Am J Surg Pathol. 1996;20(10):1196–204.PubMedGoogle Scholar
  43. 43.
    Perry KD, Al-Lbraheemi A, Rubin BP, Jen J, Ren H, Jang JS, Nair A, Davila J, Pambuccian S, Horvai A, Sukov W, Tazelaar HD, Folpe AL. Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant. Mod Pathol. 2017;30(11):1589–602.PubMedGoogle Scholar
  44. 44.
    Requena L, Kutzner H. Hemangioendothelioma. Semin Diagn Pathol. 2013;30(1):29–44.PubMedGoogle Scholar
  45. 45.
    Requena L, Sangueza OP. Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilation of preexisting vessels. J Am Acad Dermatol. 1997a;37(4):523–49. quiz 549–52.PubMedGoogle Scholar
  46. 46.
    Requena L, Sangueza OP. Cutaneous vascular proliferation. Part II. Hyperplasias and benign neoplasms. J Am Acad Dermatol. 1997b;37(6):887–919. quiz 920–2.PubMedGoogle Scholar
  47. 47.
    Requena L, Sangueza OP. Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered as vascular neoplasms. J Am Acad Dermatol. 1998;38(2 Pt 1):143–75. quiz 176–8.PubMedGoogle Scholar
  48. 48.
    Sangüeza OP, Walsh SN, Sheehan DJ, Orland AF, Llombart B, Requena L. Cutaneous epithelioid angiomatous nodule: a case series and proposed classification. Am J Dermatopathol. 2008;30(1):16–20.PubMedGoogle Scholar
  49. 49.
    Shon W, Billings SD. Cutaneous malignant vascular neoplasms. Clin Lab Med. 2017;37(3):633–46.PubMedGoogle Scholar
  50. 50.
    Suurmeijer AJ, Fletcher CD. Papillary haemangioma. A distinctive cutaneous haemangioma of the head and neck area containing eosinophilic hyaline globules. Histopathology. 2007;51(5):638–48.PubMedGoogle Scholar
  51. 51.
    Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP. Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma. Sci Transl Med. 2011;3(98):98ra82.PubMedGoogle Scholar
  52. 52.
    Ten Broek RW, Bekers EM, de Leng WWJ, Strengman E, Tops BBJ, Kutzner H, Leeuwis JW, van Gorp JM, Creytens DH, Mentzel T, van Diest PJ, Eijkelenboom A, Flucke U. Mutational analysis using Sanger and next generation sequencing in sporadic spindle cell hemangiomas: a study of 19 cases. Genes Chromosomes Cancer. 2017;56(12):855–60.PubMedGoogle Scholar
  53. 53.
    Tennant LB, Mulliken JB, Perez-Atayde AR, Kozakewich HP. Verrucous hemangioma revisited. Pediatr Dermatol. 2006;23(3):208–15.PubMedGoogle Scholar
  54. 54.
    Tollefson MM, McEvoy MT, Torgerson RR, Bridges AG. Diffuse dermal angiomatosis of the breast: clinicopathologic study of 5 patients. J Am Acad Dermatol. 2014;71(6):1212–7.PubMedGoogle Scholar
  55. 55.
    Tong PL, Beer TW. Acquired elastotic hemangioma: ten cases with immunohistochemistry refuting a lymphatic origin in most lesions. J Cutan Pathol. 2010;37(12):1259–60.PubMedGoogle Scholar
  56. 56.
    Trindade F, Kutzner H, Tellechea Ó, Requena L, Colmenero I. Hobnail hemangioma reclassified as superficial lymphatic malformation: a study of 52 cases. J Am Acad Dermatol. 2012;66(1):112–5.PubMedGoogle Scholar
  57. 57.
    Tsang WY, Chan JK. Bacillary angiomatosis. A “new” disease with a broadening clinicopathologic spectrum. Histol Histopathol. 1992;7(1):143–52.PubMedGoogle Scholar
  58. 58.
    Verma SB, Vasani R. Stewart-Bluefarb syndrome revisited. Clin Exp Dermatol. 2016;41(5):522–5.PubMedGoogle Scholar
  59. 59.
    Wang L, Gao T, Wang G. Cutaneous lymphatic malformation characterized by swelling of digits: a report of six cases. J Cutan Pathol. 2014;41(5):451–6.PubMedGoogle Scholar
  60. 60.
    Yuri T, Yamazaki F, Takasu K, Shikata N, Tsubura A. Glomeruloid hemangioma. Pathol Int. 2008;58(6):390–5.PubMedGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Pathology and Laboratory MedicineUniversity of Wisconsin School of Medicine and Public HealthMadisonUSA
  2. 2.Department of PathologyCleveland ClinicClevelandUSA

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