oral pathology
Gardner Syndrome A Case Report and Review Babak Bina, D.M.D.; Boris Zats, D.D.S.; Myrodati Lyristis, D.M.D.; Arin Abrahamian, D.D.S.
ABSTRACT With a projected 145,600 cases of colorectal cancers and 51,020 deaths in 2019, colorectal cancer is the third most common cancer and second leading cause of death in the U.S.[1] Gardner syndrome is associated with widespread development of polyps of the colon and rectum. These polyps have a 100% chance of malignancy in the fourth decade of life[2-5] Most affected individuals have dental abnormalities that may help to diagnose otherwise asymptomatic patients. The following is an overview of Gardner syndrome and
Osteomas most often occur in the angle of the mandible, but they may occur in the skull, long bones and paranasal sinus cavities.[7] Multiple osteomas are the main skeletal indication of Gardner syndrome. They can be mistaken for tori, exostosis, odontomas, osteoid osteoma and cemento-ossifying fibroma. Other manifestations of Gardner syndrome include abnormality of the retina of the eye; desmoid tumors; skin manifestations, such as epidermoid and sebaceous cysts; fibromas; neurofibroma; lipomas; leiomyomas; pigmented skin; papillary thyroid cancer osteosarcoma; chondrosarcoma; and hepatoblastoma.[4,8,9,10] Today Gardner syndrome is considered to be a variant form of familial adenomatous polyposis (FAP). Gardner syndrome is caused by a mutation of a tumor suppressor gene “APC” (adeno-
a patient who was diagnosed based on dental abnormalities, and the course of the disease over 15 years. After studying a family whose members had nine deaths from colon cancer within three generations (average age 34 years old), Dr. Eldon J. Gardner introduced “Gardner syndrome.”[2] Gardner syndrome is associated with intestinal polyposis, multiple osteomas, and skin and soft tissue tumors.[3,4,5] First reported by Fader, dental abnormalities include supernumerary teeth, unerupted teeth, fused molar roots and long, tapered roots of posterior teeth, and osteomas of both the maxilla and mandible.[6]
28 NOVEMBER 2020 The New York State Dental Journal ●
As per the NYU School of Medicine IRB, a case report is the external reporting (e.g., publication or poster/verbal presentation) of an interesting clinical situation or medical condition of a single patient. Case reports normally contain detailed information about an individual patient and may include demographic information and information on diagnosis, treatment, response to treatment, follow-up after treatment, as well as a discussion of existing relevant literature. The patient information used in this paper was originally collected solely for non-research purposes as the result of a clinical experience. Kindly see the completed self-certification form uploaded as a separate file indicating that this case report was not deemed to be human subject research, as per the NYU School of Medicine IRB. Hence, no informed consent was required. All procedures conducted were in accordance with the Declaration of Helsinki.