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Glomus tumor
Classification and external resources
ICD-O: 8711/0
OMIM 138000
DiseasesDB 30732
eMedicine derm/167
MeSH D005918
Glomus tumor was also the name formerly used for a tumor now called a paraganglioma.

A glomus tumor (also known as glomangioma, or nonchromaffin paraganglioma[1]) is a rare benign neoplasm, is a specialized arteriovenous anastomosis usually found in the skin of the extremities.[2]:670 It arises from the glomus body, and was first described at Mount Sinai Hospital in New York City.[3]

Research has indicated that there are at least four genetic mutations that lead to a glomus tumor. If there is no underlying inherited condition, then the tumor is considered "sporadic" or random. Mutations are masked when inherited through mothers, which allows mutations to pass through a generation without making themselves plainly obvious. [4]

Presentation

They are usually solitary and small, and can be found under the fingernails.[5] They can also be found on the tympanic membrane.[6]

They are often painful, and the pain is reproduced when the lesion is placed in cold water.

These tumors tend to have a bluish discoloration, although a white appearance is also noted. Elevation of the nail bed can occur

In rare cases, the tumors may present in other body areas, such as the glans penis. Treatment is essentially the same. [7]

Frequency

The exact incidence of glomus tumors is unknown. The multiple variant is rare, accounting for less than 10% of all cases. The probable misdiagnosis of many of these lesions as hemangiomas or venous malformations also makes an accurate assessment of incidence difficult.

Mortality/Morbidity

The most common adverse effect is pain, which is usually associated with solitary lesions. Multiple tumors are less likely to be painful. In one report, a patient with more than 400 glomus tumors had thrombocytopenia as a result of platelet sequestration (ie, Kasabach-Merritt syndrome). Malignant glomus tumors, or glomangiosarcomas, are extremely rare and usually represent a locally infiltrative malignancy. However, metastases do occur and are usually fatal.

Sex

Solitary glomus tumors, particularly subungual lesions, are more common in females than in males. Multiple lesions are slightly more common in males.

Age

Solitary glomus tumors are more frequent in adults than in others. Multiple glomus tumors develop 10–15 years earlier than single lesions; about one third of the cases of multiple tumors occur in those younger than 20 years. Congenital glomus tumors are rare; they are plaquelike in appearance and are considered a variant of multiple glomus tumors.

Malignant glomus tumors

Malignant glomus tumors, histologically and/or clinically are exceedingly rare. Criteria for the diagnosis of malignancy in glomus tumors are:[8]

  1. Tumor size of more than 2 centimeters and subfascial or visceral location.
  2. Atypical mitotic figures.
  3. Marked nuclear atypia and any level of mitotic activity.

Treatment

Radiation therapy and surgery can be involved in the treatment of these tumors.[9]

References

  1. ^ Ruben RJ (2007). "The history of the glomus tumors - nonchromaffim chemodectoma: a glimpse of biomedical Camelot". Acta Otolaryngol. 127 (4): 411–6. doi:10.1080/00016480601002088. PMID 17453463.  
  2. ^ Freedberg, et. al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0071380760.
  3. ^ Mount Sinai - Timeline of Accomplishments retrieved September 15, 2008
  4. ^ Glomus Tumors at the American Hearing Research Foundation Chicago, Illinois
  5. ^ Koç O, Kivrak AS, Paksoy Y (2007). "Subungual glomus tumour: magnetic resonance imaging findings". Australasian radiology 51 Spec No.: B107–9. doi:10.1111/j.1440-1673.2007.01797.x (inactive 2008-06-22). PMID 17875128.  
  6. ^ De Candia A, Como G, Passon P, Pedace E, Bazzocchi M (2002). "Sonographic findings in glomus tympanicum tumor". Journal of clinical ultrasound : JCU 30 (4): 236–40. doi:10.1002/jcu.10058. PMID 11981934.  
  7. ^ Macaluso JN, Sullivan JW, Tomberlin S: Glomus tumor of the glans penis. Urology. 25:409-410, 1985
  8. ^ Folpe AL, Fanburg-Smith JC, Mietinen M, Weiss SW (2001): Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 25: 1-12.
  9. ^ Kaylie DM, O'Malley M, Aulino JM, Jackson CG (2007). "Neurotologic surgery for glomus tumors". Otolaryngol. Clin. North Am. 40 (3): 625–49, x. doi:10.1016/j.otc.2007.03.009. PMID 17544699.  

See also

References

External links








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