Glomus tumor overview
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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 body was first discovered by Hoyer, a polish anatomist, in 1877. Glomus tumor was first described in 1924 by Barre and Masson.[1][2] Glomus tumor arises from modified smooth muscle cells (or pericytes) of the glomus body (previously called as glomus cells).[3] Glomus tumors may be classified into solitary and multiple variants.[4] On gross pathology, small (usually less than 1 cm), bluish or whitish, well circumscribed, solitary nodules are characteristic findings of glomus tumor.[5] On microscopic histopathological analysis, branching vascular channels and aggregates of specialised glomus cells are characteristic findings of glomus tumor.[6] Multiple glomus tumors are caused by a mutation in the GLMN (glomulin) gene.[7] Solitary glomus tumors must be differentiated from other diseases that cause pain such as leiomyoma and eccrine spiradenoma.[8] Multiple glomus tumors must be differentiated from other diseases such as cavernous hemangioma and blue rubber-bleb nevus syndrome.[8][9] Solitary glomus tumors commonly affect young to middle aged individuals.[4][10] Multiple glomus tumors commonly affect children.[4] 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.[4][11] Common risk factors in the development of glomus tumors are age and gender.[12] 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.[13] MRI and ultrasound may be helpful in the diagnosis of glomus tumors. Surgery is the mainstay of treatment for glomus tumor.[14] 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.[15][14][16]
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.
Classification
Glomus tumors may be classified into solitary and multiple variants.[4]
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. 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.
Risk Factors
Common risk factors in the development of glomus tumors are age and gender.
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
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 lab 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.
CT
There are no CT findings associated with glomus tumors.
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.
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 hypervascularity of the tumor.
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
- ↑ Park, Dong Sun; Choe, Woo Jin; Chun, Young Il; Moon, Chang-Taek (2013). "Glomus Tumor in the Femoral Nerve". Journal of Korean Neurosurgical Society. 54 (6): 540. doi:10.3340/jkns.2013.54.6.540. ISSN 2005-3711.
- ↑ Veros, Konstantinos; Markou, Konstantinos; Filitatzi, Chrysa; Kyrmizakis, Dionysios E. (2012). "Glomus Tumor of the Cheek: A Case Report". Case Reports in Medicine. 2012: 1–3. doi:10.1155/2012/307294. ISSN 1687-9627.
- ↑ 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.
- ↑ 4.0 4.1 4.2 4.3 4.4 Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 11, 2016.
- ↑ Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on January 7, 2016.
- ↑ Glomus tumor. Radiopedia. http://radiopaedia.org/articles/glomangioma Accessed on January 7, 2016.
- ↑ Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on February 1, 2016.
- ↑ 8.0 8.1 Lee W, Kwon SB, Cho SH, Eo SR, Kwon C (2015). "Glomus tumor of the hand". Arch Plast Surg. 42 (3): 295–301. doi:10.5999/aps.2015.42.3.295. PMC 4439588. PMID 26015884.
- ↑ Chatterjee JS, Youssef AH, Brown RM, Nishikawa H (2005). "Congenital nodular multiple glomangioma: a case report". J Clin Pathol. 58 (1): 102–3. doi:10.1136/jcp.2003.014324. PMC 1770555. PMID 15623496.
- ↑ Glomangioma. Radiopedia. http://radiopaedia.org/articles/glomangioma. Accessed onJanuary 11, 2016.
- ↑ Glomangioma. Radiopedia. http://radiopaedia.org/articles/glomangioma. Accessed onJanuary 11, 2016.
- ↑ Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on February 1, 2016
- ↑ Glomus tumor. Radiopedia. http://radiopaedia.org/cases/glomus-tumour-index-finger-2 Accessed on January 7, 2016.
- ↑ 14.0 14.1 Grover C, Khurana A, Jain R, Rathi V (2013). "Transungual surgical excision of subungual glomus tumour". J Cutan Aesthet Surg. 6 (4): 196–203. doi:10.4103/0974-2077.123401. PMC 3884883. PMID 24470715.
- ↑ Glomus tumor. Wikipedia. https://en.wikipedia.org/wiki/Glomus_tumor Accessed on February 1, 2016
- ↑ Blanchard AJ (1941). "The Pathology of Glomus Tumours". Can Med Assoc J. 44 (4): 357–60. PMC 1827043. PMID 20322052.