You are here: Home / Rare Disease Information / Rare Disease Database

Search Rare Diseases

Enter a disease name or synonym to search NORD's database of reports.

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

Ameloblastoma

NORD is very grateful to Barry Steinberg, MD, DDS, PhD, FACS, Chief, Pediatric Craniomaxillofacial Surgery, Director, Craniofacial Disorders Clinic, for assistance in the preparation of this report.

Synonyms of Ameloblastoma

  • Adamantinoma
  • Mandibular Ameloblastoma
  • Maxillary Ameloblastoma
  • Odontogenic Tumor

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Ameloblastoma is a rare disorder of the jaw involving abnormal tissue growth. The resulting tumors or cysts are usually not malignant (benign) but the tissue growth may be aggressive in the involved area. On occasion, tissue near the jaws, such as around the sinuses and eye sockets, may become involved as well. The tissues involved are most often those that give rise to the teeth so that ameloblastoma may cause facial distortion. Malignancy is uncommon as are metastases, but they do occur.

Symptoms

Ameloblastoma is characterized by an abnormal growth in the sinus area or jaw, often at the site of the third molar. The tumors or cysts may be aggressive and may spread to the nose, eye socket and skull. It is important for ameloblastoma to be diagnosed and treated early in order to stop growth of the tumors and possible progression to cancer. Although it is uncommon, ameloblastomas have been known to become malignant and spread to other parts of the body, especially to the lungs. The initial surgical treatment must be carefully and scrupulously done to avoid recurrence.

Ameloblastomas do not usually become malignant. There is evidence that tissue is more likely to become malignant if the condition reoccurs after surgery.

Causes

The cause of ameloblastoma is not understood. Causes may include injury to the mouth or jaw, infections of the teeth or gums, or inflammation of these same areas. Infections by viruses or lack of protein or minerals in the persons diet are also suspected of causing the growth or development of these tumors. In general, however, scientists do not understand the cause of cysts and tumors, nor the reasons why they can become malignant.

Affected Populations

Ameloblastoma is a rare disorder that affects males and females in equal numbers. It affects persons of all ethnic backgrounds and of all age groups.

Related Disorders

Symptoms of the following disorders can be similar to those of ameloblastoma. Comparisons may be useful for a differential diagnosis:

Hard Odontoma is a tumor of dental origin. It is composed of several characteristics of teeth such as enamel, dentin and cement. The Hard Odontoma grows by spreading directly; the ameloblastoma grows by infiltrating other spaces.

Osteosarcoma may often be confused with ameloblastoma. This cancer of the bone differs from the dental tumor by arising from the bone forming cells of the long bones.

Globulomaxillary Cysts are located between the teeth and may cause the teeth to spread apart. The cysts are either oval or heart shaped and may be removed or drained. Sometimes the treatment of the cysts can cause loss of teeth.

Standard Therapies

Diagnosis
Ameloblastoma can show up either in a regular x-ray or in an MRI imaging study.

Treatment
Surgical removal of the affected tissue is the preferred treatment. A wide margin of healthy tissue should be removed from the treated area to keep the chance of tumor regrowth to a minimum. If the tumor does reoccur, surgery is performed again.

If there is malignant spread of the tumor, radiation is the treatment choice. Chemotherapy is usually not as effective in these cases.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.

For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Ameloblastoma

References

TEXTBOOKS
Ballenger JJ, ed. Diseases of the Nose, Throat, Ear, Head and Neck. 14th ed. Lea & Febiger, Malvern, PA; 1991:213, 328.

Hicks WL Jr, Myers LL, Winston JS. Rare Pediatric Malignancies of the Head and Neck. In: Raghaven D, Brecher ML, Johnson DH. Textbook of Uncommon Cancer. 2nd ed. John Wiley & Sons. New York, NY; 1999:557-61.

REVIEW ARTICLES
Avon SL, McComb J, Clokie C. Ameloblastic carcinoma: case report and literature review. J Can Dent Assoc. 2003;69:573-76.

Dhir K, Sciubba J, Tufano RP. Ameloblastic carcinoma of the maxilla. Oral Oncol. 2003;39:736-41.

Mosqueda-Taylor A, Carlos-Bregni R, Ramirez-Amador V, et al. Odontoameloblastoma. Clinico-pathologic study of three cases and critical review of the literature. Oral Oncol. 2002;38:800-05.

Zwahlen RA, Gratz KW. Maxillary ameloblastomas: a review of the literature and of a 15-year database. J Craniomaxillofac Surg. 2002;30:273-79.

Ord RA, Blanchaert RH Jr, Nikitakis NG, et al. Ameloblastoma in children. J Oral Maxillofac Surg. 2002;60:762-70, 770-1.

JOURNAL ARTICLES
Pillai RS, Ongole R, Ahsan A, et al. Recurrent desmoplastic ameloblastoma of the maxilla: a case report. J Can Dent Assoc. 2004;70:100-104.

Junquera L, Ascani G, Vincente JC, et al. Ameloblastoma revisited. Ann Otol Rhinol Laryngol. 2003;112:1034-39.

Levin MP, Kratochvil FJ, Nolan J. Ameloblastoma of the mandible: a case report. J Periodontal. 2003;74:883-86.

Campbell D, Jeffrey RR, Wallis F, et al. Metastatic pulmonary ameloblastoma. An unusual case. Br J Oral Maxillofac Surg. 2003;41:194-96.

Datta R, Winston JS, Diaz-Reyes G, et al. Ameloblastic carcinoma: report of an aggressive case with multiple bony metastases. Am J Otolaryngol. 2003;24:64-69.

Becelli R, Carboni A, Carulli G, et al. Mandibular ameloblastoma: analysis of surgical treatment carried out in 60 patients between 1977 and 1998. J Craniofac Surg. 2002;13:395-400; 400.

Ciment LM, Ciment AJ. Malignant ameloblastoma metastatic to the lungs 29 years after primary resection: a case report. Chest. 2002;121:1359-61.

Kumamoto H, Ohki K, Ooya K. Association between vascular endothelial growth factor (VEGF) expression and tumor angiogenesis in ameloblastomas. J Oral Pathol Med. 2002;31:28-34.

FROM THE INTERNET
Ameloblastoma. Marquette University School of Dentistry - Oral & Maxillofacial Pathology. nd. 4pp.
www.dental.mu.edu/oralpath/lesions/ameloblastoma/ameloblastoma.htm

Zane RS. Maxillary Ameloblastoma. August 10,1991.
www.bcm.tmc.edu/oto/grand/81091.html
www.icom.ca/geneinfo/amelob.htm

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2009/04/06 00:00:00 GMT+0

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

NORD's Rare Disease Information Database is copyrighted and may not be published without the written consent of NORD.

 
Copyright ©2014 NORD - National Organization for Rare Disorders, Inc. All rights reserved.
The following trademarks/registered service marks are owned by NORD: NORD, National Organization for Rare Disorders, the NORD logo, RareConnect. .