Glomus tumor overview: Difference between revisions
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==Overview== | ==Overview== | ||
[[Glomus body]] was first discovered by | [[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 | [[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. | 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== | ||
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==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 | 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 | [[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 | There are no diagnostic [[Medical laboratory|laboratory]] findings associated with glomus tumor. | ||
=== Electrocardiogram === | === Electrocardiogram === | ||
There are no ECG findings associated with glomus tumor. | 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]]. | ||
=== Echocardiography and Ultrasound === | === 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. | [[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=== | ===CT Scan=== | ||
There are no CT findings associated with glomus tumors. | There are no [[CT|CT findings]] associated with glomus [[tumors]]. | ||
===MRI=== | ===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 | 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. | ||
[[ | |||
===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
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American Roentgen Ray Society Images of 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.