Polyps

What is the relationship between juvenile polyps and adenomatous polyps?
1. None
2. Juvenile polyps often become adenomatous
3. Juvenile polyps are uniformly premalignant
What is the most common pediatric GI tract polyp?
1. Adenomatous
2. Hamartoma
3. Juvenile
4. Sessile
Which is NOT a component of Denys-Drash syndrome?
1. Female pseudohermaphroditism
2. Early onset glomerulopathy
3. Wilm's' tumor
4. Mutations in the WT1 suppressor gene on 11p13
What is the pathologic appearance of a juvenile polyp?
1. Most are pedunculated
2. Most are sessile
3. They rarely have a cystic appearance
What is the most common site of a juvenile polyp?
1. Cecum
2. Sigmoid
3. Rectum
What causes juvenile polyps?
1. Defect in short arm of chromosome 10
2. Autosomal dominant inheritance
3. Idiopathic
4. Autosomal recessive
What happens to most juvenile polyps?
1. Autoinfarction
2. Gradual enlargement
3. Becomes adenomatous
4. Produces hypokalemia
What is the incidence of juvenile polyps?
1. 1 in 10,000
2. 1 in 5,000
3. 1 in 1,000
4. 1 in 500
What is the most common symptom of juvenile polyps?
1. Obstruction
2. Bleeding
3. Infection
4. Electrolyte abnormalities
T or F: Almost all children with juvenile polyps above the sigmoid colon also have a rectal or lower sigmoid polyp?
1. True
2. False
What is the risk of subsequent bleeding after removal of a juvenile polyp?
1. Under 1%
2. Under 5%
3. Under 10%
4. 15%
What type of intestinal polyps are present in Cowden's syndrome?
1. Hamartomas
2. Adenomas
3. Juvenile polyps
4. Hyerplastic polyps
T or F: There is a slightly increased risk of malignancy in relatives of patients with juvenile polyps.
1. True
2. False
What is the risk of malignancy in the Canada-Cronkhite syndrome?
1. Under 1%
2. Under 5%
3. 10 Â– 20 %
4. 50%
What is the average age at diagnosis for Peutz-Jeghers syndrome?
1. 1 year
2. 5 years
3. Adolescence
4. 20 - 30 years old
Are polyps in Peutz-Jeghers syndrome pigmented?
1. Yes
2. No
What is the most common site of polyps in Peutz-Jeghers syndrome?
1. Duodenum
2. Esophagus
3. Small bowel
4. Colon
Female patients with Peutz-Jeghers syndrome at greatest risk for what tumor?
1. Breast cancer
2. Ovarian tumors
3. Uterine tumors
4. Colon carcinoma
What are the typical symptoms of Peutz-Jeghers syndrome?
1. Massive GI bleeding
2. Intermittent abdominal pain
3. Bowel obstruction
4. Nonbilious emesis
What are the indications for surgery in patients with Peutz-Jeghers syndrome?
1. Intermittent crampy abdominal pain
2. Anemia
3. All patients are operated at diagnosis
4. Prolonged / persistent bleeding
What is the long-term risk for cancer in patients with Peutz-Jeghers syndrome?
1. Normal
2. Mildly increased
3. Markedly increased
T or F: Polyposis coli is 4 times more common in males?
1. True
2. False
In a patient with polyposis coli who develop polyps early in life, what is mean interval until cancer develops?
1. Under 5 years
2. Under 10 years
3. 10 Â– 15 years
4. 20 Â– 25 years
What is the pathology of the polyps in patients with familial polyposis?
1. Adenomatous
2. Hamartomatous
3. Juvenile
What is the metastatic potential for intraepithelial carcinoma or intramucosal carcinoma?
1. High
2. Low
Which is TRUE regarding Cowden's syndrome?
1. Associated with dermatoses
2. Autosomal recessive
3. Adenomatous polyps
What is the most common presenting symptom of familial adenomatous polyposis?
1. Most are asymptomatic
2. Bleeding
3. Obstruction
4. Crampy intermittent abdominal pain
What percent of patients with familial polyposis coli will develop gastric polyps?
1. 5%
2. 10%
3. 25%
4. 50%
Which is more common in familial polyposis coli - gastric or duodenal polyps?
1. Gastric
2. Duodenal
What is the incidence of desmoid tumors of the small intestine, mesentery, or abdominal wall in FPC?
1. 5%
2. 10%
3. 25%
4. 50%
In patients with familial polyposis coli identified at under 13 years, what is the incidence of carcinoma at diagnosis?
1. 5%
2. 10%
3. 25%
4. 50%
At least ___ percent of patients with FPC will develop cancer of the colon or rectum by the age of 40 years?
1. 5%
2. 10%
3. 25%
4. 50%
What is the treatment of familial polyposis coli?
1. Total colectomy and ileostomy
2. Ileal resection
3. Total colectomy, mucosal proctectomy, and pullthrough
4. Yearly colonoscopy
T or F: Complication risk is less for children undergoing endorectal pullthrough for FPC than for ulcerative colitis?
1. True
2. False
Which is NOT part of GardnerÂ’s syndrome?
1. Osteoid osteoma of facial bones
2. Lipomas
3. Supernumerary teeth
4. Leiomyosarcoma
What is the most common serious extracolonic manifestation associated with Gardner's disease?
1. Ependymoma
2. Uveitis
3. Meningioma
4. Ampullary carcinoma
What is the inheritance of GardnerÂ’s syndrome?
1. Autosomal dominant
2. Autosomal recessive
3. X Â– linked recessive
4. Non sex - linked recessive
What percent of patients with GardnerÂ’s syndrome have desmoid tumors?
1. 5%
2. 10%
3. 20%
4. 50%
In what percentage of GardnerÂ’s patients with desmoid tumor is it recognized before the diagnosis of polyposis coli is obtained?
1. 5%
2. 10%
3. 20%
4. 50%
What is the treatment of desmoid tumor?
1. Wide local excision and regional node excision
2. Wide local excision
3. Wide local excision and radiation therapy
What is the inheritance pattern of diffuse juvenile gastrointestinal polyposis?
1. Autosomal dominant
2. Autosomal recessive
3. X Â– linked recessive
4. Non sex linked recessive
Which is NOT seen in TurcotÂ’s syndrome?
1. Ependymoma
2. Autosomal dominant inheritance
3. Thyroid cancer
4. High risk of colon cancer
Which is NOT one of the more common symptoms of diffuse juvenile gastrointestinal polyposis?
1. Rectal prolapse
2. Intussusception
3. Anemia
4. Volvulus
How frequent are gastric polyps in patients with diffuse juvenile gastrointestinal polyposis?
1. Rare
2. Occasional
3. Almost universal
What is the risk of malignancy in patients with diffuse juvenile gastrointestinal polyposis?
1. 5%
2. 10%
3. 25%
4. 50%
True or false: adenomatous polyps are rare in children except in the case of familial polyposis coli?
1. True
2. False
Which statement is TRUE regarding familial juvenile adenomatous polyposis?
1. May be adenomatous polyps or hamartoma
2. Resection unnecessary
3. Familial in 1/4th of cases
4. Usually presents with obstruction
Which statement is FALSE regarding hyperplastic (metaplastic) polyps?
1. Hyperplastic, not neoplastic process
2. Nonmalignant
3. Premalignant
4. Small, sharply marginated
Which statement is FALSE regarding lymphoid nodular hyperplasia (lymphoid polyposis)?
1. Known as benign lymphoma
2. Non-neoplastic
3. Bleeding is uncommon
4. Peak age 1 - 3 yrs
Where are lymphoid nodules located?
1. Mucosa
2. Submucosa
3. Lamina propria
4. Subserosa
What is the surgical management of lymphoid nodular hyperplasia?
1. Colonoscopic resection
2. Total abdominal colectomy
3. Observation, with occasional steroid therapy
Which is NOT seen in Cronkhite-Canada syndrome?
1. Gastric polyps
2. Nail atrophy
3. Hypoproteinemia
4. Malignancy risk of greater than 50%

Polyps

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