Gardner syndrome is associated with all of the following except: March 2010
**Core Concept**
Gardner syndrome is a rare genetic disorder characterized by the association of colorectal adenomatous polyps with various extracolonic manifestations, including osteomas, skin and soft tissue tumors, and dental anomalies. This syndrome is caused by mutations in the APC gene, which is a tumor suppressor gene.
**Why the Correct Answer is Right**
Gardner syndrome typically presents with multiple osteomas, which are benign bone tumors, often affecting the jaw, skull, and long bones. The presence of osteomas in Gardner syndrome is due to the inactivating mutations in the APC gene, leading to the uncontrolled growth of osteoblasts, the cells responsible for bone formation. This results in the formation of multiple osteomas.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because osteomas are a hallmark feature of Gardner syndrome. Osteomas are benign bone tumors that can occur in various parts of the body, including the jaw, skull, and long bones.
**Option B:** This option is incorrect because gastrointestinal polyps are a characteristic feature of Gardner syndrome. The APC gene mutations lead to the formation of adenomatous polyps in the colon, which can progress to colorectal cancer if left untreated.
**Option C:** This option is incorrect because skin and soft tissue tumors are a common feature of Gardner syndrome. The APC gene mutations can lead to the formation of various skin and soft tissue tumors, including desmoid tumors, which are benign but locally aggressive.
**Option D:** This option is incorrect because dental anomalies are a characteristic feature of Gardner syndrome. The APC gene mutations can lead to various dental anomalies, including supernumerary teeth, impacted teeth, and odontomas.
**Clinical Pearl / High-Yield Fact**
Gardner syndrome is a rare genetic disorder that highlights the importance of genetic testing in patients with multiple osteomas or gastrointestinal polyps. Early diagnosis and management can prevent the progression of adenomatous polyps to colorectal cancer.
**Correct Answer:** D. Dental anomalies are not typically associated with Gardner syndrome.