Glomus tumor pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]

Overview

Glomus tumor arises from modified smooth muscle cells of the glomus body (or glomus apparatus). The glomus body is a neuromyoarterial plexus in the dermis of skin that is normally involved in thermoregulation.[1] The gene involved in the pathogenesis of familial glomangioma is ''glomulin'' (''GLMN'') gene.[2] On gross pathology, small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary nodules are characteristic findings of glomus tumor.[1] On microscopic histopathological analysis, branching vascular channels and aggregates of specialised glomus cells are characteristic findings of glomus tumor.[3]

Pathophysiology

  • Glomus tumor arises from modified smooth muscle cells of the glomus body (or glomus apparatus).[1]
  • The glomus body is a neuromyoarterial plexus located in the stratum reticulare, that is normally involved in thermoregulation. Although the glomus tumors are mostly concentrated in the digits, they are distributed throughout the body.[4]

Genetics

  • Familial glomangioma is a variant of glomus tumor where the patient presents with multiple glomus tumors.[1] [2]
  • The gene involved in the pathogenesis of familial glomangioma is ''glomulin'' (''GLMN'') gene.[1] [2]
    • Mutations are associated with multiple deletions in the glomulin gene of chromosome 1p21-22.[1] [2]
    • Inheritance is autosomal dominant with incomplete penetrance.[1] [2]

Associated Conditions

  • Glomus tumors of the fingers and toes may be associated with neurofibromatosis type 1.[5][6]

Gross Pathology

  • On gross pathology, small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary nodules are characteristic findings of glomus tumor.[1]
  • Multiple glomus tumors are usually less circumscribed and less solid than the solitary glomus tumors.[2]

Microscopic Findings

  • On microscopic histopathological analysis, characteristic findings of glomus tumor include:[3]
    • Branching vascular channels
    • Aggregates of specialized glomus cells
    • Inconspicuous to well-defined nucleoli

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 7, 2016.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 Gombos Z, Zhang PJ (2008). "Glomus tumor". Arch Pathol Lab Med. 132 (9): 1448–52. doi:10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2. PMID 18788860.
  3. 3.0 3.1 Glomus tumor. Radiopedia. http://radiopaedia.org/articles/glomangioma Accessed on January 7, 2016.
  4. Kim DH (1999). "Glomus tumor of the finger tip and MRI appearance". Iowa Orthop J. 19: 136–8. PMC 1888624. PMID 10847529.
  5. Kumar, Monique G.; Emnett, Ryan J.; Bayliss, Susan J.; Gutmann, David H. (2014). "Glomus tumors in individuals with neurofibromatosis type 1". Journal of the American Academy of Dermatology. 71 (1): 44–48. doi:10.1016/j.jaad.2014.01.913. ISSN 0190-9622.
  6. Stewart, D. R.; Sloan, J. L.; Yao, L.; Mannes, A. J.; Moshyedi, A.; Richard Lee, C.-C.; Sciot, R.; De Smet, L.; Mautner, V.-F.; Legius, E. (2010). "Diagnosis, management, and complications of glomus tumours of the digits in neurofibromatosis type 1". Journal of Medical Genetics. 47 (8): 525–532. doi:10.1136/jmg.2009.073965. ISSN 0022-2593.
  7. 7.0 7.1 7.2 Glomus tumor. Wikimedia commons. https://commons.wikimedia.org/wiki/File:Glomus_tumour_-_very_high_mag.jpg#/media/File:Glomus_tumour_-_intermed_mag.jpg Accessed on January 7, 2016.


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