This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Granular cell tumors (GCTs) are soft tissue tumors that can occur anywhere in the body. They are thought to arise from the cells that surround and insulate the nerve cells in our body (Schwann cells). Most granular cell tumors are benign (non-cancerous), although some may be locally aggressive. Less than 2% are malignant (cancerous), but these are aggressive and often are associated with a poor prognosis. GCTs affect females more often than males and usually present as a solitary painless mass. A few individuals have multiple GCTs. In most cases, these tumors are located in the skin of the head and neck, especially inside the mouth (70%), the tongue being the most common site. They may also occur in the breast, heart, pituitary gland, stomach, esophagus, genitalia, and upper respiratory tract. GCTs may be part of some genetic syndromes, such as LEOPARD syndrome and neurofibromatosis. Complete surgical removal of the tumor is usually curative, but in some cases the tumors may recur.
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