What is the Difference Between FAP and Gardner Syndrome?
🆚 Go to Comparative Table 🆚Gardner Syndrome is a variant of Familial Adenomatous Polyposis (FAP), a hereditary condition characterized by the development of multiple adenomatous colon polyps, which can lead to colorectal cancer if not treated. The main differences between FAP and Gardner Syndrome are:
- Extracolonic manifestations: In addition to the colon polyps, people with Gardner Syndrome typically develop other tumors outside the gastrointestinal organs, such as epidermoid cysts, osteomas, and desmoid tumors (aggressive fibromatosis). These extracolonic manifestations are not as prominent in classic FAP.
- Clinical presentation: Patients with Gardner Syndrome often present with multiple gastrointestinal polyps, osteomas of the mandible and skull, epidermal cysts, fibromatosis, and development of extra teeth. These features may not be as prevalent in classic FAP.
- Prevalence: Gardner Syndrome is a rare phenotypic variant of FAP, with a prevalence of 1 in 1,000,000 in the United States. In contrast, classic FAP has a lower prevalence, with a reported incidence ranging from 1 in 7,000 to 1 in 22,000.
- Gene mutations: Both FAP and Gardner Syndrome are caused by mutations in the APC gene. However, the specific gene mutations and their consequences may vary between the two conditions, leading to the distinct clinical presentations and manifestations.
In summary, the main differences between FAP and Gardner Syndrome lie in the presence and severity of extracolonic manifestations, the clinical presentation, and the prevalence of the conditions. Both conditions are characterized by the development of adenomatous colon polyps and are caused by mutations in the APC gene.
Comparative Table: FAP vs Gardner Syndrome
FAP (Familial Adenomatous Polyposis) and Gardner Syndrome are both genetic conditions related to the development of adenomatous colon polyps. They are caused by mutations in the APC gene, but they have distinct features and manifestations. Here is a table comparing the differences between FAP and Gardner Syndrome:
| Feature | FAP | Gardner Syndrome |
|---|---|---|
| Definition | FAP is a genetic condition where an affected person develops more than 100 adenomatous colon polyps. | Gardner Syndrome is a variant of FAP with additional extra-gastrointestinal manifestations. |
| Extra-colonic Manifestations | CHRPEs (congenital hypertrophy of the retinal pigment epithelium) can be identified in both FAP and Gardner Syndrome. | In addition to colonic symptoms, Gardner Syndrome is associated with osteomas, epidermoid cysts, and desmoid-type fibromatosis (particularly mesenteric). |
| Mutation | FAP is caused by a mutation in the APC gene, located on chromosome 5. | Gardner Syndrome arises from a mutation in the APC gene, and the number of colonic polyps is related to the locus of the mutation in the APC gene. |
| Incidence | The incidence of FAP is approximately 1 case per 7500 live births. | The incidence of Gardner Syndrome is not specified in the search results, but it is a rarer condition than FAP. |
| Treatment | Prophylactic removal of the colon can prevent the development of colorectal cancer in FAP patients. | The treatment strategies for Gardner Syndrome may be similar to those for FAP, but the management of extra-colonic manifestations may require additional interventions. |
In summary, FAP is characterized by the development of numerous adenomatous colon polyps, while Gardner Syndrome is a variant of FAP with additional extra-gastrointestinal manifestations. Both conditions are caused by mutations in the APC gene, but their clinical presentations and management may differ due to the presence of extra-colonic manifestations in Gardner Syndrome.
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