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Dermatology > DISEASES OF THE ORAL MUCOSA
Oral Granular Cell Tumors
Article Last Updated: Aug 30, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Melinda Jen, MD, Resident Physician, Department of Dermatology, University of Connecticut
Melinda Jen is a member of the following medical societies: Alpha Omega Alpha
Coauthor(s):
Steven Brett Sloan, MD, Assistant Professor, Department of Dermatology, University of Connecticut Health Center
Editors: James W Patterson, MD, Director of Dermatopathology, Professor of Pathology and Dermatology, Departments of Pathology and Dermatology, University of Virginia Medical Center; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Drore Eisen, MD, DDS, Consulting Staff, Department of Dermatology, Dermatology Research Associates of Cincinnati; Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
granular cell myoblastoma, granular cell schwannoma, Abrikossoff tumor, Abrikossoff’s tumor, tumor of Abrikossoff, granular cell neurofibroma
Background
Granular cell tumors are uncommon lesions, although the head and neck region accounts for approximately 50% of all lesions. A somewhat similar lesion, congenital epulis, occurs less frequently than the granular cell tumor and occurs on the alveolar ridge of newborns.
Pathophysiology
In 1926, Abrikossoff first described granular cell tumors. The origin of these lesions was believed to be from skeletal muscle because they were found insinuated within muscle. For many years, these lesions were referred to as granular cell myoblastoma. It was not until the advent of electron microscopy that the origin from muscle came into question. Granular cell tumors now generally are accepted as being of neural origin. Immunocytochemical markers for S-100, neuron specific enolase, and myelin basic protein support a neural origin. Markers such as CD68 and protein gene product 9.5 (PGP9.5), however, suggest an origin other than neural. Malignant granular cell tumors are extremely uncommon.
Congenital epulis is a lesion found on the alveolar ridge of newborns. Histologically, these lesions appear similar to the granular cell tumor; however, congenital epulis is negative for S-100 and other markers found in the granular cell tumor. The congenital epulis, however, is vimentin positive, whereas the granular cell tumor is not. Pseudoepitheliomatous hyperplasia, a frequent finding in granular cell tumors, generally is not present over the congenital epulis. Malignant congenital epulis has not been reported.
Frequency
United States
Oral granular cell tumor is too uncommon to accurately assess an incidence rate.
Mortality/Morbidity
Morbidity is related only to risks associated with routine excisional biopsy.
Race
Granular cell tumors occur more commonly in blacks than in whites. Congenital epulis does not appear to have a racial predilection.
Sex
Granular cell tumors occur twice as frequently in females than in males. The occurrence rate for congenital epulis is approximately 8-10 times more common in females than in males.
Age
The age range for occurrence of granular cell tumors is from the second to the eighth decade, most frequently in the fourth and fifth decades.
History
- Granular cell tumors are slow-growing lesions (0.5-1 mm per year) that are neither painful nor tender.
- Congenital epulis apparently is neither painful nor tender. The rate of growth is difficult to determine because of its presence at birth and the need for its removal to allow feeding.
- No etiologic factors appear to be associated with either lesion.
Physical
- Granular cell tumors usually are painless and appear as slightly yellowish swelling just beneath the surface epithelium.
- These lesions are firm and nontender on palpation and generally less than 2 cm in diameter.
- Ulceration of the surface epithelium is uncommon.
- Salivary gland obstruction by lesions on the floor of the mouth can cause pain.
- Displacement of the glossopharyngeal nerve and subsequent irritation from impingement against the styloid process can lead to pain with swallowing, turning the head, or extending the tongue (Eagle syndrome).
- The head and neck region accounts for approximately 45-65% of all sites for granular cell tumors.
- Approximately 70% of oral granular cell tumors occur in the tongue.
- Buccal mucosa accounts for about 10-15% of oral lesions.
- Approximately 10% of lesions have been reported bilaterally.
- Congenital epulis occurs more frequently in the maxilla. These lesions generally are less than 2 cm in diameter. Large lesions can interfere with breathing and feeding.
Causes
The etiology of the granular cell tumor and congenital epulis is unknown.
Melanotic Neuroectodermal Tumor of Infancy
Other Problems to be Considered
Verruciform xanthoma
Paraganglioma
Rhabdomyoma (extremely rare in the oral cavity)
Nerve sheath neoplasms
Peripheral odontogenic tumors (peripheral granular cell odontogenic tumor, peripheral odontogenic ghost cell tumor)
Alveolar soft part sarcoma
Developmental epithelial cysts (dermoid cysts, oral lymphoepithelial cysts)
Squamous cell carcinoma
Fibroma
Lipoma
Neurofibroma
Schwannoma
Neuroma
Lab Studies
- No clinical laboratory tests are used for these lesions. A definitive diagnosis requires biopsy.
Procedures
- Conservative excisional biopsy is indicated because both lesions are rarely larger than 2 cm in diameter.
- As a general rule, the depth of biopsy for granular cell tumors approximates the diameter of the lesion. Margins do not need to be extensive; generally, a few millimeters is adequate.
- The depth of biopsy for congenital epulis is the periosteum. Removal of bone is not indicated. Since these are exophytic lesions, surgical margins do not need to be much greater than the clinical margins.
Histologic Findings
The granular cell tumor is characterized by the presence of sheets or clusters of plump, polygonal cells with a granular cytoplasm. These lesions are not circumscribed and often come into intimate contact with the overlying surface squamous epithelium. A frequent finding is pseudoepitheliomatous hyperplasia of the overlying epithelial layer. These cells generally are uniform and do not exhibit significant atypical features. Malignant granular cell tumors, which fortunately are rare, may have a very benign histologic appearance. Tumor cells more or less stain uniformly positive for S-100 protein, neuron-specific enolase, CD68, and PGP9.5. Congenital epulis also contains sheets and/or clusters of plump cells with a granular cytoplasm. Pseudoepitheliomatous hyperplasia generally is not present. Congenital epulis lacks specificity for S-100 protein is vimentin positive.
Medical Care
Treatment for granular cell tumors and congenital epulis is surgery. While extensive surgery is not indicated, recurrence of granular cell tumors has been reported several years after removal. Recurrence of congenital epulis has not been reported; however, the number of total cases is small.
Surgical Care
Excision with a scalpel under local anesthesia is the preferred method; however, surgical margins are difficult to determine intraoperatively in the case of the granular cell tumor. Excision down to the periosteum usually is the goal for treating congenital epulis; however, recurrence does not seem to occur even if this lesion is not completely removed.
Activity
Advise the patient with routine postoperative precautions.
No medications are efficacious.
Further Outpatient Care
- Granular cell tumors have been reported to recur several years after removal. Long-term follow-up care is warranted.
Complications
- Complications may occur as a result of risks associated with routine excisional biopsy.
Prognosis
- The prognosis is excellent. Recurrence is uncommon.
Medical/Legal Pitfalls
- Misinterpretation of pseudoepitheliomatous hyperplasia as squamous cell carcinoma
- Banal histologic pattern seen in some malignant granular cell tumors
- General anesthetic risks associated with removing congenital epulis in a newborn
Special Concerns
- Reports of recurrence years following removal of the primary lesion
The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Robert Howell, DDS, MSD, to the development and writing of this article
| Media file 1:
Clinical photograph of a granular cell tumor on the tongue of a 38-year-old man. |
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Media type: Photo
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Oral Granular Cell Tumors excerpt Article Last Updated: Aug 30, 2007
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