Glomus tumor pathophysiology
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Glomus tumor pathophysiology On the Web |
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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 (or pericytes) of the glomus body (previously called as glomus cells). The glomus body is a neuromyoarterial plexus in the dermis of skin that is normally involved in thermoregulation. The gene involved in the pathogenesis of familial glomangioma is the glomulin (GLMN) gene. On gross pathology, small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary nodules are characteristic findings of glomus tumor. On microscopic histopathological analysis, branching vascular channels and aggregates of specialised glomus cells are characteristic findings of glomus tumor.
Pathophysiology
- Glomus tumor arises from modified smooth muscle cells of the glomus body (or glomus apparatus). The glomus body consists of an arterio-venous shunt surrounded by a capsule of connective tissue.
- 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.[1]
- Glomus tumors should not be confused with paragangliomas which arise from glomus cells and were previously called glomus tumors.
Genetics
- Familial glomangioma is a variant of glomus tumor where the patient presents with multiple glomus tumors.[2]
- The gene involved in the pathogenesis of familial glomangioma is glomulin (GLMN) gene.[2]
- Mutations are associated with multiple deletions in the glomulin gene of chromosome 1p21-22.[2]
- Inheritance is autosomal dominant with incomplete penetrance.[2]
Associated Conditions
- Glomus tumors of the fingers and toes may be associated with neurofibromatosis type 1.[3][4]
Gross Pathology
- Solitary glomus tumors appear as small reddish-gray encapsulated masses 0.1 to 0.3 cm in diameter.[5]
- May reach 3 cm
- 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:[6][7][8]
- Polygonal cells with moderately distinct cellular borders
- Round to oval nuclei
- Striking eosinophilic cytoplasm
- Inconspicuous to well-defined nucleoli
- Aggregates of specialized glomus cells
- Thin-walled, branching vascular channels with bland endothelial cells
- Tumor cells identified immediately adjacent to the endothelial cells
- No apparent mitotic activity
- Focal nuclear enlargement is present; however, no significant nuclear atypia is identified.
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![Intermediate magnification micrograph of a glomus tumor. H&E stain.[9]](/images/3/39/Glomus_tumour_-_intermed_mag.jpg)
Intermediate magnification micrograph of a glomus tumor. H&E stain.[9]
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![High magnification micrograph of a glomus tumor. H&E stain.[9]](/images/d/d8/1200px-Glomus_tumour_-_high_mag.jpg)
High magnification micrograph of a glomus tumor. H&E stain.[9]
-
![Very high magnification micrograph of a glomus tumor. H&E stain.[9]](/images/4/48/1200px-Glomus_tumour_-_2_-_very_high_mag.jpg)
Very high magnification micrograph of a glomus tumor. H&E stain.[9]
![Intermediate magnification micrograph of a glomus tumor. H&E stain.[9]](/index.php/File:Glomus_tumour_-_intermed_mag.jpg)
![High magnification micrograph of a glomus tumor. H&E stain.[9]](/index.php/File:1200px-Glomus_tumour_-_high_mag.jpg)
![Very high magnification micrograph of a glomus tumor. H&E stain.[9]](/index.php/File:1200px-Glomus_tumour_-_2_-_very_high_mag.jpg)
References
- ↑ Kim DH (1999). "Glomus tumor of the finger tip and MRI appearance". Iowa Orthop J. 19: 136–8. PMC 1888624. PMID 10847529.
- ↑ 2.0 2.1 2.2 2.3 2.4 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.
- ↑ 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.
- ↑ 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.
- ↑ Pater TJ, Marks RM (2004). "Glomus tumor of the hallux: case presentation and review of the literature". Foot Ankle Int. 25 (6): 434–7. doi:10.1177/107110070402500614. PMID 15215032.
- ↑ Glomus tumor. Radiopedia. http://radiopaedia.org/articles/glomangioma Accessed on January 7, 2016.
- ↑ Fazwi R, Chandran PA, Ahmad TS (2011). "Glomus tumour: a retrospective review of 15 years experience in a single institution". Malays Orthop J. 5 (3): 8–12. doi:10.5704/MOJ.1111.007. PMC 4093623. PMID 25279028.
- ↑ Glomus tumor. Libre pathology. http://librepathology.org/wiki/index.php/Glomus_tumour Accessed on February 1, 2016.
- ↑ 9.0 9.1 9.2 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.
