Gardner's syndrome associated with periampullary carcinoma, duodenal and gastric adenomatosis. Report of a case

Area of Science:

• Gastroenterology
• Oncology
• Genetics

Background:

• Gardner's syndrome, an autosomal dominant disorder, predisposes individuals to numerous gastrointestinal polyps and extracolonic manifestations.
• Patients with Gardner's syndrome have an increased risk of developing colorectal, duodenal, and gastric malignancies.

Observation:

• A 48-year-old male with a history of rectal carcinoma due to Gardner's syndrome presented with a duodenal tumor.
• Surgical resection revealed a well-differentiated adenocarcinoma with adenomatous remnants, a large duodenal adenoma with focal carcinoma, and numerous duodenal and gastric adenomas.

Findings:

• Histological examination confirmed adenocarcinoma in the duodenum, associated with extensive adenomatous changes.
• A review of 29 periampullary and 12 gastric carcinoma cases complicating familial polyposis coli or Gardner's syndrome was conducted.

Implications:

• This case underscores the critical need for lifelong endoscopic surveillance of the upper and lower gastrointestinal tract in patients diagnosed with Gardner's syndrome.
• Early detection and management of neoplastic lesions are crucial for improving outcomes in individuals with this genetic predisposition to cancer.