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Malignant potential in intestinal juvenile polyposis syndromes
M C Coburn1, V E Pricolo, F G DeLuca
1Department of Surgery, Brown University School of Medicine, Providence, Rhode Island 02902, USA.
Insights
Juvenile polyposis syndromes have a higher cancer risk than previously thought. Increased endoscopic surveillance and surgical options are recommended for managing this condition.
Area of Science:
- Gastroenterology
- Oncology
- Genetics
Background:
- Juvenile polyposis (JP) is a rare gastrointestinal disorder.
- Unlike familial polyposis coli, JP has historically been considered to have a low cancer risk.
Purpose of the Study:
- To assess the occurrence and prognosis of gastrointestinal carcinoma in patients with juvenile polyposis.
- To evaluate the cancer risk associated with juvenile polyposis syndromes.
Main Methods:
- Systematic review of all reported English-language cases of juvenile polyposis.
- Analysis of patient demographics, symptoms, treatments, and cancer development.
Main Results:
- 218 patients met inclusion criteria; mean age at diagnosis 18.5 years.
- Gastrointestinal carcinoma developed in 17% of patients, with a mean age of 35.5 years at cancer diagnosis.
- Most cancers were advanced, located in the distal colon and rectum, with poor survival.
Conclusions:
- Juvenile polyposis syndromes present a significant risk of gastrointestinal carcinoma.
- Enhanced endoscopic surveillance and consideration of surgical interventions are warranted for JP patients.
Background:
Unlike familial polyposis coli, where the premalignant nature of adenomatous polyps is well established, the cancer risk in juvenile polyposis has generally been considered not increased.
Methods:
This study reviews all cases of juvenile polyposis reported in the English language to date to assess the occurrence and prognosis of carcinoma in the gastrointestinal tract.
Results:
A total of 218 patients met the inclusion criteria. Mean age at diagnosis was 18.5 years (range: 9 months to 67 years). No gender preference was identified. The most common presenting symptom was chronic anemia, followed by acute gastrointestinal bleeding, rectal prolapse of polyp, protein-losing enteropathy, and intussusception. A family history of juvenile polyposis could be established in approximately 50% of patients, and associated congenital malformations were detected in 15%. Ninety-nine patients underwent 138 gastrointestinal operations: 121 colorectal, 12 gastric, and 5 small intestinal procedures. The development of a gastrointestinal carcinoma was reported in 36 cases (17%). Mean age at diagnosis of carcinoma was 35.5 years (range: 4-60 years). Most malignancies were located in the distal colon and rectum, with only one case of gastric and one case of duodenal carcinoma. Tumor stage at diagnosis was usually advanced, with poor survival figures.
Conclusions:
This study shows that juvenile polyposis syndromes carry a more significant risk of carcinoma than generally appreciated. Therefore, more intense endoscopic surveillance may be warranted, and definitive surgical options should often be considered in these syndromes.