Perivascular Tumors

  • Steven D. BillingsEmail author
  • Rajiv M. Patel


This chapter focuses on a group of tumors that arise from perivascular “myoid” modified smooth muscle cells that invest blood vessels. This family of tumors includes angioleiomyoma, glomus tumor, glomuvenous malformation (glomangioma), glomangiomyoma, myopericytoma, myofibroma, glomus tumor of uncertain malignant potential, and malignant glomus tumor. This group of tumors, consisting of round to spindled cells that invest a proliferation of bland blood vessels, shows significant histologic and immunophenotypic overlap. Indeed, perivascular myoid cell tumors are often hybrid lesions that demonstrate a combination of histopathologic features of the aforementioned entities.


Angioleiomyoma Glomus tumor Glomuvenous malformation (glomangioma) Glomangiomyoma Myopericytoma Myofibroma Glomus tumor of uncertain malignant potential and malignant glomus tumor 

Selected References

  1. 1.
    Agaimy A, Bieg M, Michal M, et al. Recurrent somatic PDGFRB mutations in sporadic infantile/solitary adult Myofibromas but not in Angioleiomyomas and Myopericytomas. Am J Surg Pathol. 2017;41(2):195–203.CrossRefGoogle Scholar
  2. 2.
    Antonescu CR, Sung YS, Zhang L, Agaram NP, Fletcher CD. Recurrent SRF-RELA fusions define a novel subset of cellular Myofibroma/Myopericytoma: a potential diagnostic pitfall with sarcomas with myogenic differentiation. Am J Surg Pathol. 2017;41(5):677–84.CrossRefGoogle Scholar
  3. 3.
    Boon LM, Mulliken JB, Enjolras O, Vikkula M. Glomuvenous malformation (glomangioma) and venous malformation: distinct clinicopathologic and genetic entities. Arch Dermatol. 2004;140(8):971–6.CrossRefGoogle Scholar
  4. 4.
    Borroni RG, Grassi S, Concardi M, et al. Glomuvenous malformations with smooth muscle and eccrine glands: unusual histopathologic features in a familial setting. J Cutan Pathol. 2014;41(3):308–15.CrossRefGoogle Scholar
  5. 5.
    Brouillard P, Ghassibé M, Penington A, et al. Four common glomulin mutations cause two thirds of glomuvenous malformations ("familial glomangiomas"): evidence for a founder effect. J Med Genet. 2005;42(2):e13.CrossRefGoogle Scholar
  6. 6.
    Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol. 2001;25(1):1–12.CrossRefGoogle Scholar
  7. 7.
    Granter SR, Badizadegan K, Fletcher CD. Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation. Am J Surg Pathol. 1998;22(5):513–25.CrossRefGoogle Scholar
  8. 8.
    Hung YP, Fletcher CDM. Myopericytomatosis: Clinicopathologic analysis of 11 cases with molecular identification of recurrent PDGFRB alterations in Myopericytomatosis and Myopericytoma. Am J Surg Pathol. 2017;41(8):1034–44.CrossRefGoogle Scholar
  9. 9.
    LeBlanc RE, Taube J. Myofibroma, Myopericytoma, Myoepithelioma, and Myofibroblastoma of skin and soft tissue. Surg Pathol Clin. 2011;4(3):745–59.CrossRefGoogle Scholar
  10. 10.
    Linos K, Carter JM, Gardner JM, Folpe AL, Weiss SW, Edgar MA. Myofibromas with atypical features: expanding the morphologic spectrum of a benign entity. Am J Surg Pathol. 2014;38(12):1649–54.CrossRefGoogle Scholar
  11. 11.
    McMenamin ME, Fletcher CD. Malignant myopericytoma: expanding the spectrum of tumours with myopericytic differentiation. Histopathology. 2002;41(5):450–60.CrossRefGoogle Scholar
  12. 12.
    Monteagudo C, Carda C, Llombart-Bosch A, Calduch L, Jordá E. Multiple glomangiomyoma versus glomangioma: conceptual and ultrastructural observations. Am J Dermatopathol. 2000;22(4):371–3.CrossRefGoogle Scholar
  13. 13.
    Mravic M, LaChaud G, Nguyen A, Scott MA, Dry SM, James AW. Clinical and histopathological diagnosis of glomus tumor: an institutional experience of 138 cases. Int J Surg Pathol. 2015;23(3):181–8.CrossRefGoogle Scholar
  14. 14.
    Oudijk L, den Bakker MA, Hop WC, et al. Solitary, multifocal and generalized myofibromas: clinicopathological and immunohistochemical features of 114 cases. Histopathology. 2012;60(6B):E1–11.CrossRefGoogle Scholar
  15. 15.
    Yang JS, Ko JW, Suh KS, Kim ST. Congenital multiple plaque-like glomangiomyoma. Am J Dermatopathol. 1999;21(5):454–7.CrossRefGoogle Scholar

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

Authors and Affiliations

  1. 1.Department of PathologyCleveland ClinicClevelandUSA
  2. 2.Department of PathologyUniversity of Michigan Medical SchoolAnn ArborUSA

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