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).[1] The glomus body is a neuromyoarterial plexus in the dermis of skin that is normally involved in thermoregulation.[2] The gene involved in the pathogenesis of familial glomangioma is glomulin (GLMN) gene.[3] On gross pathology, small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary nodules are characteristic findings of glomus tumor.[2] On microscopic histopathological analysis, branching vascular channels and aggregates of specialised glomus cells are characteristic findings of glomus tumor.[4]
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.[2]
- 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.[5]
- Glomus tumors should not be confused with paragangliomas which arise from glomus cells and were previously called glomus tumours.[2]
Genetics
- Familial glomangioma is a variant of glomus tumor where the patient presents with multiple glomus tumors.[2] [3]
- The gene involved in the pathogenesis of familial glomangioma is glomulin (GLMN) gene.[2] [3]
- Mutations are associated with multiple deletions in the glomulin gene of chromosome 1p21-22.[2] [3]
- Inheritance is autosomal dominant with incomplete penetrance.[2] [3]
Associated Conditions
- Glomus tumors of the fingers and toes may be associated with neurofibromatosis type 1.[6][7]
Gross Pathology
- On gross pathology, small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary nodules are characteristic findings of glomus tumor.[2]
- Multiple glomus tumors are usually less circumscribed and less solid than the solitary glomus tumors.[3]
Microscopic Findings
- On microscopic histopathological analysis, characteristic findings of glomus tumor include:[4][8][9]
- 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.[10]](/images/3/39/Glomus_tumour_-_intermed_mag.jpg)
Intermediate magnification micrograph of a glomus tumor. H&E stain.[10]
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![High magnification micrograph of a glomus tumor. H&E stain.[10]](/images/d/d8/1200px-Glomus_tumour_-_high_mag.jpg)
High magnification micrograph of a glomus tumor. H&E stain.[10]
-
![Very high magnification micrograph of a glomus tumor. H&E stain.[10]](/images/4/48/1200px-Glomus_tumour_-_2_-_very_high_mag.jpg)
Very high magnification micrograph of a glomus tumor. H&E stain.[10]
![Intermediate magnification micrograph of a glomus tumor. H&E stain.[10]](/index.php/File:Glomus_tumour_-_intermed_mag.jpg)
![High magnification micrograph of a glomus tumor. H&E stain.[10]](/index.php/File:1200px-Glomus_tumour_-_high_mag.jpg)
![Very high magnification micrograph of a glomus tumor. H&E stain.[10]](/index.php/File:1200px-Glomus_tumour_-_2_-_very_high_mag.jpg)
References
- ↑ Blume-Peytavi U, Adler YD, Geilen CC, Ahmad W, Christiano A, Goerdt S; et al. (2000). "Multiple familial cutaneous glomangioma: a pedigree of 4 generations and critical analysis of histologic and genetic differences of glomus tumors". J Am Acad Dermatol. 42 (4): 633–9. PMID 10727310.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 7, 2016.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 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.
- ↑ 4.0 4.1 Glomus tumor. Radiopedia. http://radiopaedia.org/articles/glomangioma Accessed on January 7, 2016.
- ↑ Kim DH (1999). "Glomus tumor of the finger tip and MRI appearance". Iowa Orthop J. 19: 136–8. PMC 1888624. PMID 10847529.
- ↑ 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.
- ↑ 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.
- ↑ 10.0 10.1 10.2 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.
