Glomus tumors
Glomus tumors are benign yet often extremely painful growths that arise from specialized cells in the glomus body, which plays a role in regulating skin temperature. These tumors typically present as small, round, slightly elevated nodules, frequently exhibiting a reddish-blue discoloration on the skin. While glomus tumors can occur in various locations, they are most commonly found in the skin and subcutaneous tissues of the hands and feet, particularly beneath the fingernails. Characteristic symptoms include intense pain, cold sensitivity, and localized tenderness, with pressure on the tumor often causing severe discomfort.
Diagnosing glomus tumors can be challenging due to their rarity and small size, but imaging techniques like MRI, CT scans, and X-rays can help. The definitive diagnosis is confirmed through biopsy. Treatment options range from non-operative measures, such as NSAIDs, to surgical excision for more resistant cases. Other treatments may include laser therapy and sclerotherapy. The prognosis is generally good following complete surgical removal, though there is a possibility of recurrence.
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Subject Terms
Glomus tumors
ALSO KNOWN AS: Glomangiomas, paragangliomas
RELATED CONDITIONS: Benign tumors of blood vessels and lymphatics (hemangiomas, lymphangiomas)
DEFINITION: Glomus tumors are benign but often exquisitely painful tumors that are formed from nests of specialized cells from the glomus body, a specialized arteriovenous structure involved in thermoregulation (temperature control via the skin). They manifest as round, slightly elevated, firm nodules, and when visible, the lesion appears as a small reddish-blue discoloration of the skin. Solitary or multiple in number, they are generally less than 1 centimeter (cm) in diameter. Microscopically, a tumor consists of collections of uniform cells that appear epithelial in nature and lie along the outside of abundant, branching blood vessels. Smooth muscle fibers are seen as part of the connective tissue matrix along with nerve fibers. These nerve fibers are intermixed with thick-walled capillaries and are responsible for the lancinating pain.
Risk factors: There are no known risk factors for glomus tumors.
Etiology and the disease process: Although the initiating event is unknown, the disease process is clearly an abnormal proliferation of glomus cells. These cells are differentiated perivascular cells from the arterial portions of the glomus body (Sucquet-Hoyer canal), a common arteriovenous shunt located in the dermis. Most hereditary glomangiomas are associated with defects in the glomulin, FKBP-associated protein gene (GLMN), located on chromosome 1.
Incidence: Although glomus tumors are more frequent in adults, the exact incidence is unknown. The skin and subcutaneous tissues of the hands and feet are usually affected, but a tumor may develop in any location in which a glomus body is found. These vascular tumors occur more often in the hand (75 percent) than elsewhere, and they are located beneath the fingernail (subungual) in 65 percent of patients. In the head and neck areas, glomus tumors account for 0.6 percent of all neoplasms. Glomus tumors make up between 1 and 2 percent of all soft tissue tumors and up to 5 percent of all hand tumors. Glomus tumors can also be found in the stomach and trachea.
Symptoms: Pain, cold sensitivity, and point tenderness are the characteristic symptoms of a glomus tumor. Direct pressure on the tumor can cause excruciating pain. For glomus tumors in the head and neck area, symptoms may include difficulty swallowing, hearing problems, and paralysis in the face.
Screening and diagnosis: Because of this tumor’s rarity and small size, a diagnosis may be problematic. In subungual tumors, X-rays can demonstrate bony erosion in up to 60 percent of patients. Whereas bone scans and ultrasound scans have been helpful, high-resolution magnetic resonance imaging (MRI) remains the standard for imaging diagnostics. Computed tomography (CT) and angiography are also used. The only way to confirm a diagnosis is through biopsy.
Treatment and therapy: Nonoperative treatment is chosen if local measures such as nonsteroidal anti-inflammatory drugs (NSAIDs) and activity modification adequately control discomfort. For resistant tumors, surgery involves a meticulous and complete excision of the encapsulated lesion. Additional treatment methods include laser therapy, radiation therapy, and sclerotherapy, in which a shrinking agent is injected into the tumor.
Prognosis, prevention, and outcomes: Wide excision is curative, although reoperation rates of between 5 and 50 percent have been reported, varying with the source.
Bibliography
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