eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa

Oral Granular Cell Tumors

Author: Melinda Jen, MD, Resident Physician, Department of Dermatology, University of Connecticut
Coauthor(s): Steven Brett Sloan, MD, Assistant Professor, Department of Dermatology, University of Connecticut School of Medicine; Director of Nail Disease Clinic and Chief of Dermatology, Newington Veterans Affairs Medical Center
Contributor Information and Disclosures

Updated: Nov 12, 2008

Introduction

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.

Clinical

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.

More on Oral Granular Cell Tumors

Overview: Oral Granular Cell Tumors
Differential Diagnoses & Workup: Oral Granular Cell Tumors
Treatment & Medication: Oral Granular Cell Tumors
Follow-up: Oral Granular Cell Tumors
Multimedia: Oral Granular Cell Tumors
References
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References

  1. Becelli R, Perugini M, Gasparini G, Cassoni A, Fabiani F. Abrikossoff's tumor. J Craniofac Surg. Jan 2001;12(1):78-81. [Medline].

  2. Curtis BV, Calcaterra TC, Coulson WF. Multiple granular cell tumor: a case report and review of the literature. Head Neck. Oct 1997;19(7):634-7. [Medline].

  3. Junquera LM, de Vicente JC, Vega JA, Losa JL, Albertos JM, López-Arranz JS. Granular-cell tumours: an immunohistochemical study. Br J Oral Maxillofac Surg. Jun 1997;35(3):180-4. [Medline].

  4. Lapid O, Shaco-Levy R, Krieger Y, Kachko L, Sagi A. Congenital epulis. Pediatrics. Feb 2001;107(2):E22. [Medline].

  5. Le BH, Boyer PJ, Lewis JE, Kapadia SB. Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. Arch Pathol Lab Med. Jul 2004;128(7):771-5. [Medline].

  6. McGuire TP, Gomes PP, Freilich MM, Sándor GK. Congenital epulis: a surprise in the neonate. J Can Dent Assoc. Oct 2006;72(8):747-50. [Medline].

  7. Nagaraj PB, Ongole R, Bhujanga-Rao BR. Granular cell tumor of the tongue in a 6-year-old girl--a case report. Med Oral Patol Oral Cir Bucal. Mar 2006;11(2):E162-164. [Medline].

  8. Ordóñez NG. Granular cell tumor: a review and update. Adv Anat Pathol. Jul 1999;6(4):186-203. [Medline].

  9. Philipp K, Barnes EL, Carrau RL. Eagle syndrome produced by a granular cell tumor. Arch Otolaryngol Head Neck Surg. Dec 2001;127(12):1499-1501. [Medline].

  10. Stewart CM, Watson RE, Eversole LR, Fischlschweiger W, Leider AS. Oral granular cell tumors: a clinicopathologic and immunocytochemical study. Oral Surg Oral Med Oral Pathol. Apr 1988;65(4):427-35. [Medline].

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Further Reading

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Keywords

granular cell myoblastoma, granular cell schwannoma, Abrikossoff tumor, Abrikossoff’s tumor, tumor of Abrikossoff, granular cell neurofibroma

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Contributor Information and Disclosures

Author

Melinda Jen, MD, Resident Physician, Department of Dermatology, University of Connecticut
Melinda Jen, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Nothing to disclose.

Coauthor(s)

Steven Brett Sloan, MD, Assistant Professor, Department of Dermatology, University of Connecticut School of Medicine; Director of Nail Disease Clinic and Chief of Dermatology, Newington Veterans Affairs Medical Center
Steven Brett Sloan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Connecticut State Medical Society, New England Dermatological Society, and Texas Dermatological Society
Disclosure: Nothing to disclose.

Medical Editor

James W Patterson, MD, Director of Dermatopathology, Professor of Pathology and Dermatology, Departments of Pathology and Dermatology, University of Virginia Medical Center
James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Medical Association, American Society of Dermatopathology, Medical Society of Virginia, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Drore Eisen, MD, DDS, Consulting Staff, Department of Dermatology, Dermatology Research Associates of Cincinnati
Drore Eisen, MD, DDS is a member of the following medical societies: American Academy of Dermatology, American Academy of Oral Medicine, and American Dental Association
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

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
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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