Granular cell tumors are rare neoplasms most commonly found on the tongue, with a predilection for the dermis or subcutaneous tissues. They are usually benign and can be cured through surgical removal, but malignant granular cell tumors can recur and cause death.
A granular cell tumor is a neoplasm or new growth most commonly found on the tongue. According to its location, it is also called Abrikossoff’s tumor and granular cell myoblastoma. The terms “granular cell nerve sheath tumor” and “granular cell schwannoma” are used to refer to the fact that it is a tumor that arises from neural cells, the same cells from which the nerve sheath and Schwann cells arise . It appears granular due to the presence of secondary lysosomes in the cytoplasm of the tumor cells. In general, these tumors rarely grow beyond 1.18 inches (3 cm), and most of them are benign or noncancerous.
It is not known why a person develops granular cell tumor. This cancer is very rare, so there is no data on how many people are affected. The observations have led medical professionals to believe that it is slightly more common among women and middle-aged individuals. Most cases of this condition have been found among people between the ages of 30 and 50.
While this cancer can affect any site on the body, it is most often an oral disease. In 45-65% of granular cell tumors in the head and neck area, 70% are found within the mouth, particularly in the tongue, buccal mucosa and hard palate. The larynx can also be involved in 10% of cases. An influence of the gastrointestinal tract also occurs. In fact, about 5% of granular cell tumors are found in the esophagus, stomach, intestines, biliary system, and gallbladder.
Microscopic studies have revealed that this tumor has a predilection for the dermis or subcutaneous tissues; therefore, it often manifests itself as dermal growths or skin conditions. Less frequently, it occurs in the muscles or submucosa. When viewed with the naked eye, the tumor will appear pale white or yellowish, with no bleeding or necrosis. The skin or mucous membrane covering the tumor may appear normal. Sometimes a thickening and cobbled appearance can be observed.
Diagnosis is usually made by biopsy. When the pathologist says the lesion is benign, a cure can be achieved through surgical removal of the tumor. A benign granular cell tumor, however, has a recurrence rate ranging from 2 to 8%. On the other hand, when the lesion is considered malignant, surgical removal may not provide a cure, and chemotherapy and radiation options are ineffective. A malignant granular cell tumor can recur at a rate of 30% and can cause the death of 60% of affected individuals within 3 years of initial detection.
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