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Special Feature
November 1998

Pathological Case of the Month

Author Affiliations
 

ENIDGILBERT-BARNESSMD

Arch Pediatr Adolesc Med. 1998;152(11):1152. doi:
Diagnosis and Discussion: Juvenile Polyposis Coli

Figure 1. Computed tomographic scan of abdomen showing mass in the cecum with distended proximal bowel.

Figure 2. Gross photograph of the specimen showing multiple pedunculated polyps in the cecum and ascending colon.

Figure 3. Microscopic photograph of a polyp showing cystically dilated crypts filled with mucus and inflammatory cells. There is abundant granulation tissue and inflmamatory exudate composed of plasma cells, polymorphonuclear leukocytes, and histiocytes in the stroma (hematoxylin-eosin, original magnification ×100).

JUVENILE POLYPS are the most common type of gastrointestinal polypoid lesions in childhood.13 Most of them are solitary and confined to the rectosigmoid region. Juvenile polyps have been described as retention polyps or inflammatory polyps, and are routinely grouped with hamartomas in the literature.4 Juvenile polyposis syndrome is characterized by numerous polyps throughout the colon (juvenile polyposis coli)5 or the entire gastrointestinal tract (generalized juvenile polyposis)6 and presents with rectal bleeding, abdominal pain, and intussusception. Jass et al7 have suggested a working diagnostic criteria for juvenile polyposis syndrome that includes one of the following: (1) more than 5 juvenile polyps of the colon or rectum, (2) juvenile polyps throughout the gastrointestinal tract, or (3) any number of juvenile polyps with a family history of juvenile polyposis.

Goodman et al8 have suggested that juvenile polyps represent one step in a spectrum, beginning with hyperplasia, progressing to typical juvenile polyps that then develop adenomatous changes, and progressing eventually to a true adenoma. Familial juvenile polyposis is one of the inherited polyposis syndromes associated with a predisposition to gastrointestinal tract malignant neoplasms.9 It is inherited as an autosomal dominant trait, but no specific genetic defect has been identified. The risk of colon cancer is increased, although the magnitude of the increased risk is controversial.10

Once a diagnosis of juvenile polyposis syndrome is established, the entire gastrointestinal tract must be periodically examined by upper and lower endoscopy because of the increased risk of recurrence and subsequent development of a carcinoma.11 Only prolonged observation will allow accurate assessment of the neoplastic potential of these lesions. Genetic studies may enlighten us further as to the nature of this condition and in particular its relationship to adenomatous polyposis coli.12 No guidelines have been suggested on the indications for prophylactic colectomy.12

References
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Leggettt  BAThomas  LRKnight  NHealey  SChenevix-Trench  GSearle  J Exclusion of APC and MCC as the gene defect in one family with familial juvenile polyposis. Gastrointest Endosc. 1995;42560- 564Article
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