$Unique_ID{BRK03449} $Pretitle{} $Title{Ameloblastoma} $Subject{Ameloblastoma Adamantinoma Odontogenic Tumor Mandibular Ameloblastoma Maxillary Ameloblastoma Hard Odontoma Osteosarcoma Globulomaxiallary Cysts} $Volume{} $Log{} Copyright (C) 1992 National Organization for Rare Disorders, Inc. 908 Ameloblastoma ** IMPORTANT ** It is possible that the main title of the article (Ameloblastoma) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Adamantinoma Odontogenic Tumor Mandibular Ameloblastoma Maxillary Ameloblastoma Information on the following diseases can be found in the Related Disorders section of this report: Hard Odontoma Osteosarcoma Globulomaxiallary Cysts General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Ameloblastoma is a very rare disorder of the jaw and sinuses. Major symptoms may include cysts or tumors in the dental area. They may also include the sinus, nose and/or eye socket. In some cases the Ameloblastoma can become malignant and spread to other areas of the body. 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 that Ameloblastoma is diagnosed and treated early in order to stop growth of the tumors and progression to cancer. Ameloblastomas have been known to become malignant and spread to other parts of the body including the lungs. However, if the initial treatment removes the entire tumor and a wide margin of tissue around the tumor, reoccurrence is rare. If the Ameloblastoma does become malignant it usually spreads through the lymphatic or blood systems. In these cases, although they are rare, the site it spreads to most often is the lung area. The Ameloblastoma does not usually become malignant unless it has reoccurred after surgery that was unsuccessful. Wide margins of healthy tissue around the tumor must be removed. Causes The cause of Ameloblastoma is not understood. It has been suggested that it may be caused by dental irritation during the growth of teeth, the pulling of teeth or in some cases by cavities in the teeth. Other 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 Population 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. There are more than 200 different forms of cancer, and Malignant Ameloblastoma is a very rare form of cancer. 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 can 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. Therapies: Standard Ameloblastoma can show up either in a regular x-ray or in an MRI imaging study. Treatment of Ameloblastoma consists of surgical removal of the cysts or tumors. 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. Therapies: Investigational This disease entry is based upon medical information available through May 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Ameloblastoma, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Dental Research 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4261 The National Cancer Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5583 or 5717 (800) 4-CANCER American Cancer Society 1599 Clifton RD, NE Atlanta, GA 30329 (404) 320-3333 References DISEASES OF THE NOSE, THROAT, EAR, HEAD, & NECK., 14th Ed., John Jacob Ballenger, Lea & Febiger, Philadelphia, 1991. Pp. 213, 328. METASTASIZING AMELOBLASTOMA., E H Laughlin, Cancer, August 1, 1991 (issue 64 (3)). Pp. 776-780. AMELOBLASTOMA IN YOUNG PERSONS: A CLINICOPATHOLOGIC ANALYSIS AND ETIOLOGIC INVESTIGATION. MA Kahn, Oral Surg Oral Med Oral Pathol, June, 1998, (issue 67 (6)). Pp. 706-715. AMELOBLASTOMA METASTATIC TO THE LUNG. RP Clay, et al.; Ann Plast Surg, February, 1989, (issue 22 (2)). Pp. 160-162. MAXILLARY AMELOBLASTOMA: CASE REPORT, FJ Sacaccia, et al.; Am J Otolaryngol, January-February, 1991, (issue 12 (1)). Pp. 20-25. AMELOBLASTIC CARCINOMA. REPORT OF AN AGGRESSIVE CASE AND REVIEW OF THE LITERATURE, RA Bruce, et al.; J Craniomaxillofac Surg, August, 1991, (issue 19 (6)). Pp. 267-271. DIAGNOSIS AND TREATMENT OF METASTATIC AMELOBLASTOMA. AH Eliasson, et al.; South Med J, September, 1989, (issue 82 (9)). Pp. 1165-1168.