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Clinicians may be hesitant to prescribe biologics or immunomodulators to individuals with familial adenomatous polyposis (FAP) and comorbid inflammatory disease (CID) because of increased cancer risk. Our aim was to compare the risk of malignancy in FAP individuals with inflammatory bowel (IBD) and/or rheumatic disease that received biologics/immunomodulators to those who did not. Individuals with FAP and CID were included in the study. We compared the incidence of cancer between individuals exposed to biologics/immunomodulators compared to unexposed from the date of diagnosis of comorbid disease till last follow up or death. Hazard ratio (HR) for cancer was computed using Cox regression model and compared by exposure status to biologic/immunomodulators. 25 individuals with FAP and a comorbid inflammatory disease were identified including 9 (36%) with IBD and 16 (64%) with rheumatic disease. 14 (56%) were exposed to a biologic and or immunomodulator. Median duration of biologic/immunomodulator exposure was 48 (2-180) months. 3 (21.4%) in the exposed group compared to 1 (9.1%) in the unexposed group developed cancer with a HR for exposure of 1.92 (CI 0.2-18.5, p = 0.57). Median duration of follow up after the diagnosis of inflammatory disease was 10 (5.5-17.0) years in the exposed and 6 (3.0-15.0) years in the unexposed group. In the exposed group, 1 patient developed gastric and 2 developed colon cancer. One unexposed patient developed medullary thyroid cancer. There is a possible trend of more cancers in the group that received biologics/immunomodulators-but given the small number of patients and p-value, there may be no difference at all. This preliminary finding warrants study in a larger cohort.

Hepatoblastoma is a rare hepatic malignancy that is seen in Familial Adenomatous Polyposis (FAP). The occurrence is four hundred fold higher than the general population [1]. Hepatoblastoma is generally treatable with surgery; in addition it is highly responsive to chemotherapy. While the literature reports on this extracolonic manifestation of FAP, no screening recommendations exist, this may be due to the rarity of the condition and paucity of published literature. Our aim was to examine and assess the number of hepatoblastoma patients, their associated demographics and the outcome of disease.

Purpose The identification of individuals with Hereditary Non Polyposis Colorectal Cancer (HNPCC) in the population is suboptimal. Causes include lack of physician recognition or failure to take an accurate family history. While colorectal cancer (CRC) in HNPCC is preventable by annual colonoscopy it is underutilized in part by lack of physician recommendation or poor understanding of personal risk of disease. We developed an online CRC risk assessment (http://www.clevelandclinic.org/score) incorporating family and personal history of adenomas and CRC which generated a pedigree, risk category and screening recommendations based on ACG guidelines. Modifiable lifestyle factors were also assessed and personalized recommendations were provided to minimize neoplasia due to those factors. We assessed the feasibility and online uptake of this tool and determined the proportion of high risk individuals who meet criteria suspicious for HNPCC. Methods The assessment included questions on demographics, use of previous CRC screening, and family and personal history of adenomas and CRC in 3 generations. Height, weight, age > or < 50, race, smoking exposure, physical activity, and dietary habits assessed. Risk categories included average, low, medium, and high.

Background The APC protein has an important role in maintaining function of microtubules in the ear which playa significant part in the mechanism of hearing. Preliminary data suggests that the APC protein is associated with an increased incidence of abnormal hearing which may affect intellectual function. We sought to assess the hearing among patients with FAP (Familial Adenomatous Polyposis). Methods Patients with FAP were recruited for an IRB (Institutional Review Board) approved study assessing hearing and intelligence. Hearing was tested by pure tone air conduction audiometry, less than or equal to 30 db at 3/4 of the following frequencies (500 Hz, 1000 Hz, 2000 Hz and 4000 Hz). Subjects were then administered the Kaufman Brief Intelligence Test (KBIT-2). We then analyzed the proportion of individuals with an abnormal audiometry as compared to age and gender adjusted normalized hearing standards. Results 44 patients were recruited from 42 families. Subjects included 22 men with a mean age of 42 years. When compared to normalized hearing standards, 19 (43.2%) of the 44 patients failed to meet the standard normal range. Audiologic abnormalities showed unilateral hearing impairment was documented in 6 patients, bilateral impairment in 13. Of these patients 63% were impaired at a single frequency; the other 37% were at multiple frequencies. 59% of patients showed right hearing impairment with the highest deficit (35%) at 2000 Hz. 71% of patients had left sided impairment with the greatest number (32%) at 4000 Hz. Conclusion A large subset of our sample of FAP patients (43.2%) had abnormal audiologic results when compared to the normalized standard. Differences in IQ scores for patients with and without audiologic abnormalities are not statistically significant, suggesting that these results do not reflect an association between hearing and intellectual functioning in our sample.