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Breast and colorectal cancers rarely occur in children, but when they do, these conditions are more precarious, according to two National Cancer Data Base (NCDB) studies presented at the American College of Surgeons Clinical Congress.

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Public emphasis on screening tests has improved detection rates and outcomes for both cancer types in the last 10 years for people age 50 and older, and outcomes in breast cancer patients have improved steadily as well, noted the researchers, from Seattle Children's Hospital and Maine Medical Center, who decided to investigate what happens when patients under age 21 are diagnosed with breast or colorectal cancer.


"The thought that kids even face these diseases is surprising," Morgan K. Richards, MD, a research fellow in the Division of General Surgery at Seattle Children's Hospital, said in a news release. "But that's why it's important to study such diseases. Although rare, they are not impossible to find in children. We need to better understand how these cancers present and progress so we know how to recognize them clinically and so we can counsel patients and their families."


Richards and her colleagues looked at patient medical records for the years 1998 to 2011 entered in the NCDB of the American College of Surgeons and the American Cancer Society. Of the 2,636,722 breast cancer patients, only 574 were in children or young adults age 21 or younger.


There were two striking sets of differences between the adults and those under age 21, the researchers said: First, the younger patients had a totally different demographic profile than the adult population of patients; more than 25 percent of the younger patients were African-American, compared with only 10 percent of the adult patients. The younger patients were also more likely to be male and uninsured. Richards noted that these demographic differences are consistent with previous, smaller studies.


Second, the results also showed that the younger breast cancer patients fared worse: Their cancers were more aggressive and were often discovered at a later stage.


The younger breast cancer patients also had longer intervals between definitive surgical treatment and radiation or chemotherapy compared with the situation for adults. It took the younger patients a week longer than the adult patients to get surgical treatment. Because breast cancer in young people is so rare, the longer time between diagnosis and an operation makes sense, Richards said. "It may take an extra biopsy or secondary pathologic confirmation to make the diagnosis."


However, after surgical treatment, it took younger patients approximately 100 additional days to receive radiation therapy. "That delay is a red flag for further investigation," she said. "Once the operation happens, you typically know what the diagnosis is. We don't know what's causing the extended time period between the operation and radiation therapy, but clinically, it's potentially significant.


"There may be good reasons for the delay, but even rare diseases benefit from standardized care. Getting this information out among providers may help to raise awareness of this discrepancy between pediatric and adult care."


Also as noted in the news release, the second team of investigators, from Maine Medical Center, reviewed the NCDB data to assess how younger patients fared after a diagnosis of colorectal cancer. Between 1998 and 2011, about 920 of the colorectal cancer patients recorded in the NCDB were age 21 or younger (compared with 157,779 patients between ages 22 and 50; and 1,304,085 over age 50.


The youngest group of patients, age 21 and under, were more often diagnosed with stages III or IV cancer compared with older patients, and had more aggressive tumors even when comparing cancers of similar stage.


This finding could explain why they were also more likely to have more aggressive treatments, such as total removal of the colon and rectum, the team noted. Older patients were more likely to have only the malignant sections of the colon removed.


"There must be an underlying reason or predisposition leading to these patients developing colorectal cancer so young," said Monica Langer, MD, pediatric surgeon at Maine Medical Center. "If you don't perform a total resection, they will need a lot more surveillance postoperatively."


Despite more aggressive surgical treatment, children still had lower five-year survival rates than young adults. Previous studies have drawn similar conclusions, but this study used the largest sample size to date and is the first to directly compare pediatric with adult patients for this type of cancer, Langer noted.


Both researchers noted that although the studies are too early to have direct implications for treatment, the results do serve as a call to raise awareness among pediatric clinicians about the findings and highlight the different behavior of these cancers in children for reasons as yet not understood. Studying these patients may improve our understanding of colorectal and breast cancer for all ages.