Glomus tumor overview: Difference between revisions

Jump to navigation Jump to search
 
(10 intermediate revisions by 2 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Glomus tumor}}
{{Glomus tumor}}
{{CMG}}{{AE}} {{STM}}
{{CMG}}{{AE}} {{STM}} {{RAK}}


==Overview==
==Overview==
[[Glomus body]] was first discovered by Hoyer, a polish anatomist, in 1877. Glomus tumor was first described in 1924 by Barre and Masson.<ref name="ParkChoe2013">{{cite journal|last1=Park|first1=Dong Sun|last2=Choe|first2=Woo Jin|last3=Chun|first3=Young Il|last4=Moon|first4=Chang-Taek|title=Glomus Tumor in the Femoral Nerve|journal=Journal of Korean Neurosurgical Society|volume=54|issue=6|year=2013|pages=540|issn=2005-3711|doi=10.3340/jkns.2013.54.6.540}}</ref><ref name="VerosMarkou2012">{{cite journal|last1=Veros|first1=Konstantinos|last2=Markou|first2=Konstantinos|last3=Filitatzi|first3=Chrysa|last4=Kyrmizakis|first4=Dionysios E.|title=Glomus Tumor of the Cheek: A Case Report|journal=Case Reports in Medicine|volume=2012|year=2012|pages=1–3|issn=1687-9627|doi=10.1155/2012/307294}}</ref> Glomus tumor arises from modified [[smooth muscle cell]]s (or [[pericyte]]s) of the [[glomus body]] (previously called as [[glomus cell]]s).<ref name="pmid10727310">{{cite journal| author=Blume-Peytavi U, Adler YD, Geilen CC, Ahmad W, Christiano A, Goerdt S et al.| title=Multiple familial cutaneous glomangioma: a pedigree of 4 generations and critical analysis of histologic and genetic differences of glomus tumors. | journal=J Am Acad Dermatol | year= 2000 | volume= 42 | issue= 4 | pages= 633-9 | pmid=10727310 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10727310  }} </ref> Glomus tumors may be classified into solitary and multiple variants.<ref name="wd">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 11, 2016.</ref> On gross pathology, small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary nodules are characteristic findings of glomus tumor.<ref name="rs">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 7, 2016.</ref> On microscopic histopathological analysis, branching [[vascular]] channels and aggregates of specialised [[glomus cell]]s are characteristic findings of glomus tumor.<ref name="pm">Glomus tumor. Radiopedia. http://radiopaedia.org/articles/glomangioma Accessed on January 7, 2016.</ref> Multiple glomus tumors are caused by a [[mutation]] in the GLMN (''glomulin'') gene.<ref name="be">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on February 1, 2016.</ref> Solitary glomus tumors must be differentiated from other diseases that cause pain such as [[leiomyoma]] and [[eccrine]] [[spiradenoma]].<ref name="pmid26015884">{{cite journal| author=Lee W, Kwon SB, Cho SH, Eo SR, Kwon C| title=Glomus tumor of the hand. | journal=Arch Plast Surg | year= 2015 | volume= 42 | issue= 3 | pages= 295-301 | pmid=26015884 | doi=10.5999/aps.2015.42.3.295 | pmc=PMC4439588 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26015884  }} </ref> Multiple glomus tumors must be differentiated from other diseases such as [[cavernous hemangioma]] and blue rubber-bleb nevus syndrome.<ref name="pmid26015884">{{cite journal| author=Lee W, Kwon SB, Cho SH, Eo SR, Kwon C| title=Glomus tumor of the hand. | journal=Arch Plast Surg | year= 2015 | volume= 42 | issue= 3 | pages= 295-301 | pmid=26015884 | doi=10.5999/aps.2015.42.3.295 | pmc=PMC4439588 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26015884  }} </ref><ref name="pmid15623496">{{cite journal| author=Chatterjee JS, Youssef AH, Brown RM, Nishikawa H| title=Congenital nodular multiple glomangioma: a case report. | journal=J Clin Pathol | year= 2005 | volume= 58 | issue= 1 | pages= 102-3 | pmid=15623496 | doi=10.1136/jcp.2003.014324 | pmc=PMC1770555 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15623496  }} </ref> Solitary glomus tumors commonly affect young to middle aged individuals.<ref name="wd">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 11, 2016.</ref><ref>Glomangioma. Radiopedia. http://radiopaedia.org/articles/glomangioma. Accessed onJanuary 11, 2016.</ref> Multiple glomus tumors commonly affect children.<ref name="wd">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 11, 2016.</ref> Females are more commonly affected with solitary glomus tumors (particularly subungual lesions) than males. Males are more commonly affected with multiple glomus tumors than females.<ref name="wd">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 11, 2016.</ref><ref>Glomangioma. Radiopedia. http://radiopaedia.org/articles/glomangioma. Accessed onJanuary 11, 2016.</ref> Common risk factors in the development of glomus tumors are age and gender.<ref name="po">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on February 1, 2016</ref> If left untreated, patients with glomus tumors may progress to develop pain and nail discoloration. Common complication of the glomus tumor includes [[malignant]] change in multiple tumors. Common complications of glomus tumors post operatively include nail deformities and recurrence. Findings on x rays suggestive of glomus tumor may include a marginated bone [[erosion]] or thinning of the adjacent cortical bone.<ref name="me">Glomus tumor. Radiopedia. http://radiopaedia.org/cases/glomus-tumour-index-finger-2 Accessed on January 7, 2016.</ref> [[MRI]] and [[ultrasound]] may be helpful in the diagnosis of glomus tumors. Surgery is the mainstay of treatment for glomus tumor.<ref name="pmid24470715">{{cite journal| author=Grover C, Khurana A, Jain R, Rathi V| title=Transungual surgical excision of subungual glomus tumour. | journal=J Cutan Aesthet Surg | year= 2013 | volume= 6 | issue= 4 | pages= 196-203 | pmid=24470715 | doi=10.4103/0974-2077.123401 | pmc=PMC3884883 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24470715  }} </ref> Prognosis is generally excellent for solitary glomus tumors and malignant glomus tumors treated with wide excision. However, the prognosis is poor for malignant glomus tumors with widespread [[metastases]].<ref name="kl">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on February 1, 2016</ref><ref name="pmid24470715">{{cite journal| author=Grover C, Khurana A, Jain R, Rathi V| title=Transungual surgical excision of subungual glomus tumour. | journal=J Cutan Aesthet Surg | year= 2013 | volume= 6 | issue= 4 | pages= 196-203 | pmid=24470715 | doi=10.4103/0974-2077.123401 | pmc=PMC3884883 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24470715  }} </ref><ref name="pmid20322052">{{cite journal| author=Blanchard AJ| title=The Pathology of Glomus Tumours. | journal=Can Med Assoc J | year= 1941 | volume= 44 | issue= 4 | pages= 357-60 | pmid=20322052 | doi= | pmc=PMC1827043 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20322052  }} </ref>
[[Glomus body]] was first discovered by Wood, a Scottish surgeon, in 1812 in the ''Edinburgh Medical Journal''. However, Glomus tumor was first correctly described in 1924 by Barre and Masson. Glomus tumor arises from modified [[smooth muscle cell]]s (or [[pericyte]]s) of the [[glomus body]] (previously called as [[glomus cell]]s). Glomus tumors may be classified into solitary and multiple variants.  On [[gross pathology]], they are small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary [[Nodule (medicine)|nodules]] are characteristic findings of glomus [[tumor]]. On microscopic histopathological analysis, branching [[vascular]] channels and aggregates of specialized [[glomus cell]]s are characteristic findings of glomus tumor. Multiple glomus tumors are caused by a [[mutation]] in the GLMN (''glomulin'') [[gene]]. Solitary glomus [[Tumor|tumors]] must be differentiated from other diseases that cause [[pain]] such as [[leiomyoma]] and [[eccrine]] [[spiradenoma]]. Multiple glomus tumors must be differentiated from other diseases such as [[cavernous hemangioma]] and [[blue rubber bleb nevus syndrome]]. Solitary glomus tumors commonly affect young to middle aged individuals. Multiple glomus tumors commonly affect children. [[Female|Females]] are more commonly affected with [[solitary]] glomus tumors (particularly subungual lesions) than males. [[Male|Males]] are more commonly affected with multiple glomus tumors than [[Female|females]]. Common risk factors in the development of glomus tumors are age and gender. If left untreated, patients with glomus tumors may progress to develop pain and nail discoloration. Common complication of the glomus tumor includes [[malignant]] change in multiple tumors. Common complications of glomus tumors post operatively include nail deformities and recurrence. Findings on [[X-rays|x rays]] suggestive of glomus tumor may include a marginated [[bone]] erosion or thinning of the adjacent [[cortical bone]]. [[MRI]] and [[ultrasound]] may be helpful in the diagnosis of glomus tumors. [[Surgery]] is the mainstay of treatment for glomus tumor. [[Prognosis]] is generally excellent for solitary glomus tumors and [[malignant]] glomus tumors treated with wide [[excision]]. However, the [[prognosis]] is poor for [[malignant]] glomus tumors with widespread [[metastases]].


==Historical Perspective==
==Historical Perspective==
[[Glomus body]] was first discovered by Wood, a scotish surgeon, in 1812 in the ''Edinburgh Medical Journal''. However, Glomus tumor was first correctly described in 1924 by Barre and Masson.
[[Glomus body]] was first discovered by Wood, a Scottish surgeon, in 1812 in the ''Edinburgh Medical Journal''. However, Glomus tumor was first correctly described in 1924 by Barre and Masson.


==Classification==
==Classification==
Glomus tumors may be classified into [[solitary]] and multiple variants.<ref name="wd">Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 11, 2016.</ref>
Glomus tumors may be classified into [[solitary]] and multiple variants.  


==Pathophysiology==
==Pathophysiology==
Glomus tumor arises from modified [[Smooth muscle cell|smooth muscle cells]] (or [[Pericyte|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 cell|glomus cells]] are characteristic findings of glomus tumor.  
Glomus tumor arises from modified [[Smooth muscle cell|smooth muscle cells]] (or [[Pericyte|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]] [[Nodule (medicine)|nodules]] are characteristic findings of glomus tumor. On microscopic histopathological analysis, branching [[vascular]] channels and aggregates of specialised [[Glomus cell|glomus cells]] are characteristic findings of glomus tumor.  


==Causes==
==Causes==
Line 19: Line 19:


==Differentiating Glomus Tumor from other Diseases==
==Differentiating Glomus Tumor from other Diseases==
Solitary glomus tumors must be differentiated from other diseases that cause pain such as [[leiomyoma]] and eccrine [[spiradenoma]]. Multiple glomus tumors must be differentiated from other diseases such as [[cavernous hemangioma]] and blue rubber-bleb nevus syndrome.
Solitary glomus tumors must be differentiated from other diseases that cause pain such as [[leiomyoma]] and eccrine [[spiradenoma]]. Multiple glomus tumors must be differentiated from other diseases such as [[cavernous hemangioma]] and [[blue rubber bleb nevus syndrome]].


==Epidemiology and Demographics==
==Epidemiology and Demographics==
The exact [[incidence]] of glomus tumors is unknown.
The exact [[incidence]] of glomus tumors is unknown. [[Female|Females]] are more commonly affected with solitary glomus tumors (particularly subungual lesions) than males, while multiple lesions are slightly more common in males. Solitary glomus tumors can occur at any age; however, multiple glomus tumors commonly affect children.
 
Females are more commonly affected with solitary glomus tumors (particularly subungual lesions) than males, while multiple lesions are slightly more common in males.
 
Solitary glomus tumors can occur at any age; however, multiple glomus tumors commonly affect children.


==Risk Factors==
==Risk Factors==
There are no established risk factors for glomus tumor; however, an epidemiologic relationship may exist between glomus tumors and neurofibromatosis.
There are no established risk factors for glomus tumor; however, an [[Epidemiology|epidemiologic]] relationship may exist between glomus tumors and [[neurofibromatosis]].


==Screening==
==Screening==
[[Screening]] for multiple glomus tumors by genetic testing is recommended among individuals with a family history of glomangiomas ([[autosomal dominant]] inheritance).
[[Screening]] for multiple glomus tumors by [[genetic testing]] is recommended among individuals with a family history of glomangiomas ([[autosomal dominant]] [[Heredity|inheritance]]).


==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
If left untreated, patients with glomus tumors may progress to develop pain and nail discoloration. Common complication of the glomus tumor includes [[malignant]] change in multiple tumors. Common complications of glomus tumors post operatively include nail deformities and recurrence. Prognosis is generally excellent for solitary glomus tumors and malignant glomus tumors treated with wide [[excision]]. However, the prognosis is poor for malignant glomus tumors with widespread [[metastases]].
If left untreated, [[Patient|patients]] with glomus tumors may progress to develop [[pain]] and nail discoloration. Common [[Complication (medicine)|complication]] of the glomus tumor includes [[malignant]] change in multiple [[Tumor|tumors]]. Common complications of glomus tumors post operatively include [[nail]] deformities and recurrence. [[Prognosis]] is generally excellent for solitary glomus tumors and [[malignant]] glomus tumors treated with wide [[excision]]. However, the [[prognosis]] is poor for [[malignant]] glomus tumors with widespread [[metastases]].


==Diagnosis==
==Diagnosis==


=== Diagnostic Study of Choice ===
=== Diagnostic Study of Choice ===
There is no single diagnostic study of choice for the diagnosis of glomus tumor, but glomus tumors can be diagnosed based on MRI of the finger in addition to history and physical examination.
There is no single [[diagnostic study of choice]] for the diagnosis of glomus [[tumor]], but glomus [[tumors]] can be diagnosed based on [[MRI]] of the finger in addition to history and [[physical examination]].


===History and Symptoms===
===History and Symptoms===
A detailed history from the patient may be helpful. A positive history of trauma may be present. A positive family history may be present in patients with multiple glomus tumors (autosomal dominant).Symptoms of glomus tumor include hypersensitivity to cold and paroxysmal pain at a well defined site.
A detailed history from the [[patient]] may be helpful. A positive history of [[Physical trauma|trauma]] may be present. A positive family history may be present in patients with multiple glomus [[tumors]] ([[autosomal dominant]]). Symptoms of glomus [[tumor]] include [[hypersensitivity]] to cold and paroxysmal [[pain]] at a well defined site.


===Physical examination===
===Physical examination===
Patients with glomus tumor usually appear well. Physical examination of patients with glomus tumor is usually remarkable for small (usually less than 2cm), blue or red palpable [[nodule]]s which are usually distributed in the [[acral]] regions ([[subungual]] most common) and nail deformities.
[[Patient|Patients]] with glomus tumor usually appear well. [[Physical examination|Physical examinatio]]<nowiki/>n of [[Patient|patients]] with glomus tumor is usually remarkable for small (usually less than 2cm), blue or red palpable [[nodule]]s which are usually distributed in the acral regions ([[subungual]] most common) and [[nail]] deformities.


===Laboratory Findings===
===Laboratory Findings===
There are no diagnostic lab findings associated with glomus tumor.
There are no diagnostic [[Medical laboratory|laboratory]] findings associated with glomus tumor.
 
=== Electrocardiogram ===
There are no [[ECG]] findings associated with glomus tumor.


===X Ray===
===X Ray===
X rays may be helpful in the diagnosis of glomus tumor. Findings on x rays suggestive of glomus tumor may include a marginated bone erosion or thinning of the adjacent cortical bone.
[[X-rays|X rays]] may be helpful in the diagnosis of glomus tumor. Findings on [[X-rays|x rays]] suggestive of glomus tumor may include a marginated [[bone]] erosion or thinning of the adjacent [[cortical bone]].


===CT===
=== Echocardiography and Ultrasound ===
There are no CT findings associated with glomus tumors.
[[Ultrasound]] may be helpful in the preoperative diagnosis of glomus tumor; it provides the localization, size, and shape of [[Tumor|tumors]] as small as 3 mm. Findings on an [[ultrasound]] suggestive of glomus [[tumor]] include a well-circumscribed hypoechoic mass.
 
===CT Scan===
There are no [[CT|CT findings]] associated with glomus [[tumors]].


===MRI===
===MRI===
MRI is helpful in the diagnosis of glomus tumor. Findings on MRI suggestive of glomus tumor includes hyperintense signal on T2 and PD (proton-density) weighted imaging.
An [[MRI]] may be helpful in the [[diagnosis]] of glomus [[tumor]]. Findings on [[MRI]] suggestive of glomus tumor include slightly hypointense or hyperintense [[T1]] images and hyperintense [[MRI|T2]] images.
 
===Ultrasound===
[[Ultrasound]] may be helpful in the diagnosis of glomus tumor. Findings on finger ultrasound suggestive of subungual glomus tumor includes [[subungual]] [[hypoechoic]] lesion at the region of maximum tenderness. [[Doppler ultrasound]] may be helpful in the diagnosis of glomus tumor. Findings on doppler ultrasound suggestive of glomangioma include [[hypervascular]]ity of the tumor.


===Other Imaging Findings===
===Other Imaging Findings===
There are no other imaging findings associated with glomus tumor.
There are no other [[imaging]] findings associated with glomus [[tumor]].


===Other Diagnostic Studies===
===Other Diagnostic Studies===
Other diagnostic studies for glomus tumor include [[immunohistochemistry]] staining, which demonstrates [[glomus cells]] positive for [[vimentin]] and alpha-smooth muscle actin and negative for [[desmin]].
Other diagnostic studies for glomus [[tumor]] include [[immunohistochemistry]] staining, which demonstrates [[glomus cells]] positive for [[vimentin]] and alpha-[[smooth muscle]] [[actin]] and negative for [[desmin]].
==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
The predominant therapy for solitary glomus tumor is surgical resection. Patients with multiple glomus tumors are treated with sclerotherapy or laser therapy.
The predominant therapy for solitary glomus tumor is [[Resection|surgical resection]]. Patients with multiple glomus [[tumors]] are treated with [[sclerotherapy]] or [[laser therapy]].


===Surgery===
===Surgery===
[[Surgery]] is the mainstay of treatment for glomus tumor.
[[Surgery]] is the mainstay of [[Treatment Planning|treatment]] for glomus tumor.


==References==
==References==

Latest revision as of 16:40, 21 June 2019

Glomus tumor Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Glomus tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Glomus tumor overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Glomus tumor overview

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Glomus tumor overview

CDC on Glomus tumor overview

Glomus tumor overview in the news

Blogs on Glomus tumor overview

Directions to Hospitals Treating Glomus tumor

Risk calculators and risk factors for Glomus tumor overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2] Roukoz A. Karam, M.D.[3]

Overview

Glomus body was first discovered by Wood, a Scottish surgeon, in 1812 in the Edinburgh Medical Journal. However, Glomus tumor was first correctly described in 1924 by Barre and Masson. Glomus tumor arises from modified smooth muscle cells (or pericytes) of the glomus body (previously called as glomus cells). Glomus tumors may be classified into solitary and multiple variants. On gross pathology, they are 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. Multiple glomus tumors are caused by a mutation in the GLMN (glomulin) gene. Solitary glomus tumors must be differentiated from other diseases that cause pain such as leiomyoma and eccrine spiradenoma. Multiple glomus tumors must be differentiated from other diseases such as cavernous hemangioma and blue rubber bleb nevus syndrome. Solitary glomus tumors commonly affect young to middle aged individuals. Multiple glomus tumors commonly affect children. Females are more commonly affected with solitary glomus tumors (particularly subungual lesions) than males. Males are more commonly affected with multiple glomus tumors than females. Common risk factors in the development of glomus tumors are age and gender. If left untreated, patients with glomus tumors may progress to develop pain and nail discoloration. Common complication of the glomus tumor includes malignant change in multiple tumors. Common complications of glomus tumors post operatively include nail deformities and recurrence. Findings on x rays suggestive of glomus tumor may include a marginated bone erosion or thinning of the adjacent cortical bone. MRI and ultrasound may be helpful in the diagnosis of glomus tumors. Surgery is the mainstay of treatment for glomus tumor. Prognosis is generally excellent for solitary glomus tumors and malignant glomus tumors treated with wide excision. However, the prognosis is poor for malignant glomus tumors with widespread metastases.

Historical Perspective

Glomus body was first discovered by Wood, a Scottish surgeon, in 1812 in the Edinburgh Medical Journal. However, Glomus tumor was first correctly described in 1924 by Barre and Masson.

Classification

Glomus tumors may be classified into solitary and multiple variants.

Pathophysiology

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.

Causes

The cause of solitary glomus tumors has not been identified. Multiple glomus tumors are caused by a mutation in the GLMN (glomulin) gene.

Differentiating Glomus Tumor from other Diseases

Solitary glomus tumors must be differentiated from other diseases that cause pain such as leiomyoma and eccrine spiradenoma. Multiple glomus tumors must be differentiated from other diseases such as cavernous hemangioma and blue rubber bleb nevus syndrome.

Epidemiology and Demographics

The exact incidence of glomus tumors is unknown. Females are more commonly affected with solitary glomus tumors (particularly subungual lesions) than males, while multiple lesions are slightly more common in males. Solitary glomus tumors can occur at any age; however, multiple glomus tumors commonly affect children.

Risk Factors

There are no established risk factors for glomus tumor; however, an epidemiologic relationship may exist between glomus tumors and neurofibromatosis.

Screening

Screening for multiple glomus tumors by genetic testing is recommended among individuals with a family history of glomangiomas (autosomal dominant inheritance).

Natural History, Complications and Prognosis

If left untreated, patients with glomus tumors may progress to develop pain and nail discoloration. Common complication of the glomus tumor includes malignant change in multiple tumors. Common complications of glomus tumors post operatively include nail deformities and recurrence. Prognosis is generally excellent for solitary glomus tumors and malignant glomus tumors treated with wide excision. However, the prognosis is poor for malignant glomus tumors with widespread metastases.

Diagnosis

Diagnostic Study of Choice

There is no single diagnostic study of choice for the diagnosis of glomus tumor, but glomus tumors can be diagnosed based on MRI of the finger in addition to history and physical examination.

History and Symptoms

A detailed history from the patient may be helpful. A positive history of trauma may be present. A positive family history may be present in patients with multiple glomus tumors (autosomal dominant). Symptoms of glomus tumor include hypersensitivity to cold and paroxysmal pain at a well defined site.

Physical examination

Patients with glomus tumor usually appear well. Physical examination of patients with glomus tumor is usually remarkable for small (usually less than 2cm), blue or red palpable nodules which are usually distributed in the acral regions (subungual most common) and nail deformities.

Laboratory Findings

There are no diagnostic laboratory findings associated with glomus tumor.

Electrocardiogram

There are no ECG findings associated with glomus tumor.

X Ray

X rays may be helpful in the diagnosis of glomus tumor. Findings on x rays suggestive of glomus tumor may include a marginated bone erosion or thinning of the adjacent cortical bone.

Echocardiography and Ultrasound

Ultrasound may be helpful in the preoperative diagnosis of glomus tumor; it provides the localization, size, and shape of tumors as small as 3 mm. Findings on an ultrasound suggestive of glomus tumor include a well-circumscribed hypoechoic mass.

CT Scan

There are no CT findings associated with glomus tumors.

MRI

An MRI may be helpful in the diagnosis of glomus tumor. Findings on MRI suggestive of glomus tumor include slightly hypointense or hyperintense T1 images and hyperintense T2 images.

Other Imaging Findings

There are no other imaging findings associated with glomus tumor.

Other Diagnostic Studies

Other diagnostic studies for glomus tumor include immunohistochemistry staining, which demonstrates glomus cells positive for vimentin and alpha-smooth muscle actin and negative for desmin.

Treatment

Medical Therapy

The predominant therapy for solitary glomus tumor is surgical resection. Patients with multiple glomus tumors are treated with sclerotherapy or laser therapy.

Surgery

Surgery is the mainstay of treatment for glomus tumor.

References


Template:WikiDoc Sources