Glomus tumor is a rare benign neoplasm composed of cells derived from the smooth muscle cells that surround blood vessels in the glomus body. It can be multiple – glomangiomatosis. Most cases occur in the fingers, usually in a subungueal location. Feet are also a frequent site. Excruciating pain is the hallmark of this tumor. Radiographs show a lucent, well-defined nodular depression in the extremity of the distal phalanx. Tumor cells are round in glomus tumor but may be elongated, in glomangiomyoma. They are positive for smooth muscle markers. Marginal resection or curettage is curative for benign tumors. Malignant glomus tumors require multimodality cancer treatment.


Glomus Glomangioma Vascular Perivascular Tumor Digits 

Recommended Reading

  1. Blume-Peytavi U, Adler YD, Geilen CC, Ahmad W, Christiano A, Goerdt S, Orfanos CE. Multiple familial cutaneous glomangioma: a pedigree of 4 generations and critical analysis of histologic and genetic differences of glomus tumors. J Am Acad Dermatol. 2000;42(4):633–9.CrossRefPubMedGoogle Scholar
  2. Boon LM, Brouillard P, Irrthum A, Karttunen L, Warman ML, Rudolph R, Mulliken JB, Olsen BR, Vikkula M. A gene for inherited cutaneous venous anomalies (“glomangiomas”) localizes to chromosome 1p21-22. Am J Hum Genet. 1999;65(1):125–33.CrossRefPubMedCentralPubMedGoogle Scholar
  3. Brems H, Park C, Maertens O, Pemov A, Messiaen L, Upadhyaya M, Claes K, Beert E, Peeters K, Mautner V, Sloan JL, Yao L, Lee CC, Sciot R, De Smet L, Legius E, Stewart DR. Glomus tumors in neurofibromatosis type 1: genetic, functional, and clinical evidence of a novel association. Cancer Res. 2009;69(18):7393–401.CrossRefPubMedCentralPubMedGoogle Scholar
  4. Brouillard P, Boon LM, Mulliken JB, Enjolras O, Ghassibé M, Warman ML, Tan OT, Olsen BR, Vikkula M. Mutations in a novel factor, glomulin, are responsible for glomuvenous malformations (“glomangiomas”). Am J Hum Genet. 2002;70(4):866–74.CrossRefPubMedCentralPubMedGoogle Scholar
  5. Choi JJ, Murphey MD. Angiomatous skeletal lesions. Semin Musculoskelet Radiol. 2000;4(1):103–12.CrossRefPubMedGoogle Scholar
  6. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. WHO classification of tumors of soft tissue and bone. Lyon: IARC; 2013. p. 302–4.Google Scholar
  7. Mosquera JM, Sboner A, Zhang L, Chen CL, Sung YS, Chen HW, Agaram NP, Briskin D, Basha BM, Singer S, Rubin MA, Tuschl T, Antonescu CR. Novel MIR143-NOTCH fusions in benign and malignant glomus tumors. Genes Chromosomes Cancer. 2013;52(11):1075–87.CrossRefPubMedCentralPubMedGoogle Scholar
  8. Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 1995;15(4):893–917.CrossRefPubMedGoogle Scholar
  9. Nielsen GP, Rosenberg AE. Diagnostic pathology – bone. Philadelphia: Amirsys; 2013. p. 3.2–3.3.Google Scholar
  10. Stewart DR, Pemov A, Van Loo P, Beert E, Brems H, Sciot R, Claes K, Pak E, Dutra A, Lee CC, Legius E. Mitotic recombination of chromosome arm 17q as a cause of loss of heterozygosity of NF1 in neurofibromatosis type 1-associated glomus tumors. Genes Chromosomes Cancer. 2012;51(5):429–37.CrossRefPubMedCentralPubMedGoogle Scholar
  11. Unni KK, Inwards C. Dahlin’s bone tumors. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2010. p. 179–83.Google Scholar
  12. Wenger DE, Wold LE. Benign vascular lesions of bone: radiologic and pathologic features. Skeletal Radiol. 2000;29(2):63–74.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  1. 1.Laboratory of Orthopaedic PathologyBuenos AiresArgentina
  2. 2.Department of PathologyA.C. Camargo Cancer CenterSão PauloBrazil

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