| Glomus tumor | |
|---|---|
| Classification and external resources | |
| ICD-O: | 8711/0 |
| OMIM | 138000 |
| DiseasesDB | 30732 |
| eMedicine | derm/167 |
| MeSH | D005918 |
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]
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]
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.
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.
Solitary glomus tumors, particularly subungual lesions, are more common in females than in males. Multiple lesions are slightly more common in males.
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, histologically and/or clinically are exceedingly rare. Criteria for the diagnosis of malignancy in glomus tumors are:[8]
Radiation therapy and surgery can be involved in the treatment of these tumors.[9]
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