Glomus tumor pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
Glomus tumor arises from modified [[smooth muscle cell]]s (or [[pericyte]]s) of the [[glomus body]] (previously called as glomus cells). | Glomus tumor arises from modified [[smooth muscle cell]]s (or [[pericyte]]s) 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 [[Histopathology|histopathological]] analysis, branching [[vascular]] channels and aggregates of specialized [[glomus cell]]s are characteristic findings of glomus tumor. | ||
==Pathophysiology== | ==Pathophysiology== | ||
*Glomus tumor arises from modified [[smooth muscle cell]]s of the [[glomus body]] (or glomus apparatus). The glomus body consists of an arterio-venous shunt surrounded by a capsule of connective tissue. | *Glomus tumor arises from modified [[smooth muscle cell]]s of the [[glomus body]] (or glomus apparatus). The [[glomus body]] consists of an [[Arteriovenous shunts|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.<ref name="pmid10847529">{{cite journal| author=Kim DH| title=Glomus tumor of the finger tip and MRI appearance. | journal=Iowa Orthop J | year= 1999 | volume= 19 | issue= | pages= 136-8 | pmid=10847529 | doi= | pmc=PMC1888624 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10847529 }} </ref> | *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.<ref name="pmid10847529">{{cite journal| author=Kim DH| title=Glomus tumor of the finger tip and MRI appearance. | journal=Iowa Orthop J | year= 1999 | volume= 19 | issue= | pages= 136-8 | pmid=10847529 | doi= | pmc=PMC1888624 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10847529 }} </ref> | ||
*Glomus tumors should not be confused with [[paraganglioma]]s which arise from [[glomus cell]]s and were previously called glomus | *Glomus [[tumors]] should not be confused with [[paraganglioma]]s which arise from [[glomus cell]]s and were previously called glomus [[tumors]]. | ||
==Genetics== | ==Genetics== | ||
*Familial glomangioma is a variant of glomus tumor where the patient presents with multiple glomus tumors. | *Familial [[glomangioma]] is a variant of glomus [[tumor]] where the [[patient]] presents with multiple glomus [[tumors]].<ref name="pmid18788860">{{cite journal| author=Gombos Z, Zhang PJ| title=Glomus tumor. | journal=Arch Pathol Lab Med | year= 2008 | volume= 132 | issue= 9 | pages= 1448-52 | pmid=18788860 | doi=10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18788860 }} </ref> | ||
*The gene involved in the pathogenesis of familial glomangioma is ''glomulin'' (''GLMN'') gene. | *The [[gene]] involved in the [[pathogenesis]] of familial [[glomangioma]] is ''glomulin'' (''GLMN'') [[gene]].<ref name="pmid18788860">{{cite journal| author=Gombos Z, Zhang PJ| title=Glomus tumor. | journal=Arch Pathol Lab Med | year= 2008 | volume= 132 | issue= 9 | pages= 1448-52 | pmid=18788860 | doi=10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18788860 }} </ref> | ||
**Mutations are associated with multiple deletions in the ''glomulin'' gene of chromosome 1p21-22. | **[[Mutation|Mutations]] are associated with multiple [[Deletion (genetics)|deletions]] in the ''glomulin'' [[gene]] of [[chromosome]] 1p21-22.<ref name="pmid18788860">{{cite journal| author=Gombos Z, Zhang PJ| title=Glomus tumor. | journal=Arch Pathol Lab Med | year= 2008 | volume= 132 | issue= 9 | pages= 1448-52 | pmid=18788860 | doi=10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18788860 }} </ref> | ||
**Inheritance is [[autosomal dominant]] with incomplete penetrance. | **[[Inheritance]] is [[autosomal dominant]] with incomplete [[penetrance]].<ref name="pmid18788860">{{cite journal| author=Gombos Z, Zhang PJ| title=Glomus tumor. | journal=Arch Pathol Lab Med | year= 2008 | volume= 132 | issue= 9 | pages= 1448-52 | pmid=18788860 | doi=10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18788860 }} </ref> | ||
==Associated Conditions== | ==Associated Conditions== | ||
*Glomus tumors of the fingers and toes may be associated with [[neurofibromatosis type 1]].<ref name="KumarEmnett2014">{{cite journal|last1=Kumar|first1=Monique G.|last2=Emnett|first2=Ryan J.|last3=Bayliss|first3=Susan J.|last4=Gutmann|first4=David H.|title=Glomus tumors in individuals with neurofibromatosis type 1|journal=Journal of the American Academy of Dermatology|volume=71|issue=1|year=2014|pages=44–48|issn=01909622|doi=10.1016/j.jaad.2014.01.913}}</ref><ref name="StewartSloan2010">{{cite journal|last1=Stewart|first1=D. R.|last2=Sloan|first2=J. L.|last3=Yao|first3=L.|last4=Mannes|first4=A. J.|last5=Moshyedi|first5=A.|last6=Richard Lee|first6=C.-C.|last7=Sciot|first7=R.|last8=De Smet|first8=L.|last9=Mautner|first9=V.-F.|last10=Legius|first10=E.|title=Diagnosis, management, and complications of glomus tumours of the digits in neurofibromatosis type 1|journal=Journal of Medical Genetics|volume=47|issue=8|year=2010|pages=525–532|issn=0022-2593|doi=10.1136/jmg.2009.073965}}</ref> | *Glomus [[tumors]] of the [[fingers]] and [[toes]] may be associated with [[neurofibromatosis type 1]].<ref name="KumarEmnett2014">{{cite journal|last1=Kumar|first1=Monique G.|last2=Emnett|first2=Ryan J.|last3=Bayliss|first3=Susan J.|last4=Gutmann|first4=David H.|title=Glomus tumors in individuals with neurofibromatosis type 1|journal=Journal of the American Academy of Dermatology|volume=71|issue=1|year=2014|pages=44–48|issn=01909622|doi=10.1016/j.jaad.2014.01.913}}</ref><ref name="StewartSloan2010">{{cite journal|last1=Stewart|first1=D. R.|last2=Sloan|first2=J. L.|last3=Yao|first3=L.|last4=Mannes|first4=A. J.|last5=Moshyedi|first5=A.|last6=Richard Lee|first6=C.-C.|last7=Sciot|first7=R.|last8=De Smet|first8=L.|last9=Mautner|first9=V.-F.|last10=Legius|first10=E.|title=Diagnosis, management, and complications of glomus tumours of the digits in neurofibromatosis type 1|journal=Journal of Medical Genetics|volume=47|issue=8|year=2010|pages=525–532|issn=0022-2593|doi=10.1136/jmg.2009.073965}}</ref> | ||
==Gross Pathology== | ==Gross Pathology== | ||
* | * | ||
*Multiple glomus tumors are usually less circumscribed and less solid than the solitary glomus tumors.<ref name="pmid18788860">{{cite journal| author=Gombos Z, Zhang PJ| title=Glomus tumor. | journal=Arch Pathol Lab Med | year= 2008 | volume= 132 | issue= 9 | pages= 1448-52 | pmid=18788860 | doi=10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18788860 }} </ref> | *[[Solitary]] glomus [[tumors]] appear as small reddish-gray [[Capsule|encapsulated]] masses 0.1 to 0.3 cm in diameter.<ref name="pmid15215032">{{cite journal| author=Pater TJ, Marks RM| title=Glomus tumor of the hallux: case presentation and review of the literature. | journal=Foot Ankle Int | year= 2004 | volume= 25 | issue= 6 | pages= 434-7 | pmid=15215032 | doi=10.1177/107110070402500614 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15215032 }}</ref> | ||
**May reach 3 cm | |||
*Multiple glomus [[tumors]] are usually less circumscribed and less solid than the [[solitary]] glomus [[tumors]].<ref name="pmid18788860">{{cite journal| author=Gombos Z, Zhang PJ| title=Glomus tumor. | journal=Arch Pathol Lab Med | year= 2008 | volume= 132 | issue= 9 | pages= 1448-52 | pmid=18788860 | doi=10.1043/1543-2165(2008)132[1448:GT]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18788860 }} </ref> | |||
==Microscopic Findings== | ==Microscopic Findings== | ||
*On microscopic histopathological analysis, characteristic findings of glomus tumor include: | *On microscopic [[Histopathology|histopathological]] analysis, characteristic findings of glomus [[tumor]] include:<ref name="pmid25279028">{{cite journal| author=Fazwi R, Chandran PA, Ahmad TS| title=Glomus tumour: a retrospective review of 15 years experience in a single institution. | journal=Malays Orthop J | year= 2011 | volume= 5 | issue= 3 | pages= 8-12 | pmid=25279028 | doi=10.5704/MOJ.1111.007 | pmc=PMC4093623 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25279028 }} </ref><ref>Glomus tumor. Libre pathology. http://librepathology.org/wiki/index.php/Glomus_tumour Accessed on February 1, 2016.</ref> | ||
**Polygonal cells with moderately distinct cellular borders | **Polygonal cells with moderately distinct cellular borders | ||
**Round to oval [[nuclei]] | **Round to oval [[nuclei]] | ||
| Line 32: | Line 34: | ||
**Aggregates of specialized [[glomus cell]]s | **Aggregates of specialized [[glomus cell]]s | ||
**Thin-walled, branching vascular channels with bland [[endothelial cell]]s | **Thin-walled, branching vascular channels with bland [[endothelial cell]]s | ||
**Tumor cells identified immediately adjacent to the endothelial | **[[Tumor]] cells identified immediately adjacent to the [[endothelial cell]]s | ||
**No apparent [[mitotic]] activity | **No apparent [[mitotic]] activity | ||
**Focal nuclear enlargement is present; however, no significant [[nuclear atypia]] is identified. | **Focal nuclear enlargement is present; however, no significant [[nuclear atypia]] is identified. | ||
<gallery> | <gallery> | ||
Image:Glomus_tumour_-_intermed_mag.jpg|Intermediate magnification micrograph of a glomus tumor. H&E stain.<ref name=op>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.</ref> | Image:Glomus_tumour_-_intermed_mag.jpg|Intermediate magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons<ref name=op>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.</ref> | ||
Image:1200px-Glomus_tumour_-_high_mag.jpg|High magnification micrograph of a glomus tumor. H&E stain.<ref name=op>Glomus tumor. Wikimedia commons. https://commons.wikimedia.org/wiki/File:Glomus_tumour_-_very_high_mag.jpg#/media/File:Glomus_tumour_-_high_mag.jpg Accessed on January 7, 2016.</ref> | Image:1200px-Glomus_tumour_-_high_mag.jpg|High magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons<ref name=op>Glomus tumor. Wikimedia commons. https://commons.wikimedia.org/wiki/File:Glomus_tumour_-_very_high_mag.jpg#/media/File:Glomus_tumour_-_high_mag.jpg Accessed on January 7, 2016.</ref> | ||
Image:1200px-Glomus_tumour_-_2_-_very_high_mag.jpg|Very high magnification micrograph of a glomus tumor. H&E stain.<ref name=op>Glomus tumor. Wikimedia commons. https://commons.wikimedia.org/wiki/File:Glomus_tumour_-_very_high_mag.jpg#/media/File:Glomus_tumour_-_2_-_very_high_mag.jpg Accessed on January 7, 2016.</ref> | Image:1200px-Glomus_tumour_-_2_-_very_high_mag.jpg|Very high magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons<ref name=op>Glomus tumor. Wikimedia commons. https://commons.wikimedia.org/wiki/File:Glomus_tumour_-_very_high_mag.jpg#/media/File:Glomus_tumour_-_2_-_very_high_mag.jpg Accessed on January 7, 2016.</ref> | ||
</gallery> | </gallery> | ||
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Latest revision as of 15:11, 24 June 2019
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Glomus tumor Microchapters |
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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 specialized 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
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]
- 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.
-
![Intermediate magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]](/images/3/39/Glomus_tumour_-_intermed_mag.jpg)
Intermediate magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]
-
![High magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]](/images/d/d8/1200px-Glomus_tumour_-_high_mag.jpg)
High magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]
-
![Very high magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]](/images/4/48/1200px-Glomus_tumour_-_2_-_very_high_mag.jpg)
Very high magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]
![Intermediate magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]](/index.php/File:Glomus_tumour_-_intermed_mag.jpg)
![High magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]](/index.php/File:1200px-Glomus_tumour_-_high_mag.jpg)
![Very high magnification micrograph of a glomus tumor. H&E stain.Source: wikimedia commons[8]](/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.
- ↑ 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.
- ↑ 8.0 8.1 8.2 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.
