by Karen Albritton MD
Source: Spring 2005 CCCF Newsletter

Cancer is the leading cause of death from disease in adolescents aged 15-19 in the U.S. It affects over four thousand older teens each year, 1.9 times the number in 5- to 9-year olds, and 1.7 times that in 10- to 14-year-olds. However, you may be surprised to learn that only one-third of these adolescent cancer patients receive their care at pediatric oncology centers. And when they do, they often feel out of place, as over 85% of patients seen at pediatric oncology programs are under age 15, and these programs focus their research, clinical care and psychosocial support on the needs of these younger patients. Alternatively when they are seen by adult medical oncologists in an adult cancer facility, they are an even greater anomaly, as they account for 1% of the population seen by this specialty. What determines which specialist they see? Mostly, random referral patterns. Upper age limits at pediatric oncology centers range from 16 to 21 years, while adult oncology programs accept patients as young as 15. Therefore, some patients in this age group are being treated as adults, while some are being treated as children, with no systematic method for discerning what is most appropriate for the patient. This leaves this population without a home, stranded between two cultures that do not own  it. Without any focus on them, they have suffered.

Studies show that statistically there has been much less improvement in the survival rates for cancer patients within this adolescent age group compared with the steady progress that has increased survival rates by 30% both for children and older adults over the last twenty years (the lack of improvement is even worse for those 20-30 years of age!). As shown in figure 1 below, the average annual improvement in the five year survival rates of teens and young adults with cancer is dismally low in comparison to all other age groups.

Figure 1 (Data from SEER, NCI and U.S. Census analyzed by A. Bleyer.)

This stagnation is partly due to the lack of associated clinical research for this patient population. As seen below in figure 2, an average of nine percent of cancer patients ages 15 to 24 years are enrolled in clinical trials, in contrast to approximately 40% of children ages birth to 14 years.

Figure 2 (Data from SEER, NCI and U.S. Census analyzed by A. Bleyer.)

In part, this is because few trials are written for the types of tumor these young adults have. Also, until very recently, trials were written with an age cut-off of 18, meaning the 19 year old seen at a pediatric center was not able to be offered a trial, and the 17 year old seen at a medical center was not eligible for studies open there.

Other issues compound the problem. A combination of low clinical suspicion by medical professionals and a young person's feeling of invincibility often delay diagnosis. In a study looking at the time between symptom onset and diagnosis in 2,665 children participating in Pediatric Oncology Group therapeutic protocols between 1982 and 1988, for all solid tumors except Hodgkin disease, as age increased, lag time to diagnosis increased.

Furthermore, 18-24 year olds have the lowest rate of insurance coverage of any age group in the US. Upon reaching a legal age of independence, this age group often finds themselves no longer covered under their parent's health coverage or State Child Health Insurance Plans "SCHIP" and not yet established in careers that provide full health benefits. 49.6% of 18-24 year olds were uninsured for at least one month over a 24-month period (2001-2002), with half of these uninsured for more than 12 months. This lack of insurance further discourages access to regular medical care.

As illustrated in Figure 3 below, the five year survival rate of adolescent patients treated for rhabdomyosarcoma is approximately 42%. This compares to a five year survival rage of 65% for children treated for the same cancer between the ages of birth to 14 years. These decreased survival rates can be seen for acute myeloid leukemia, Ewing's sarcoma, osteosarcoma, softtissue sarcomas, non-hodgkin's lymphoma and renal tumors.

Figure 3 (Albritton KH and Bleyer WA.The management of cancer in the older adolescent. Eur J Cancer. 2003 Dec;39(18):2584-99).

We do not know if this is due to a difference in the biology of the tumor at different ages or differences in treatment protocols. A recent German study of Ewing s sarcoma patients age 15 to 30 treated on a single therapeutic protocol showed a marked increase in survival rates for patients treated at pediatric institutions. This is possibly because pediatric oncologists have greater experience with this tumor that peaks in incidence in late adolescence. Similarly, in the U.S., Holland, and France, 25% more 15 to 21 year olds with acute lymphoblastic leukemia were alive five years after diagnosis when treated on pediatric leukemia protocols than when treated on a concurrent protocol in the adult oncology group. Although there may indeed be biologic differences between a given cancer at age 10 and 30, this data suggests that for a narrow age group where tumor biology is likely to be the same, lives are being lost because some patients are not getting to the most appropriate place for treatment of their disease. Further research must now be done to determine the relative contributions of tumor biology, patient characteristics and treatment specifics to outcome in these patients.

Another important factor in successfully treating adolescents and young adults (AYA) is addressing their unique psychosocial needs. When cancer occurs in young people, their lives, previously active and hopeful, are put on hold. There are concerns about education, career development, parental dynamics, social interactions, and insecurity about body image and infertility. These concerns can affect decisions about treatment and ultimately survival. Few support staff (psychologists, social workers, nurse educators) are trained in looking for these needs and meeting them. Again, the binary systems of adult medical and pediatric oncology are likely impeding progress because such a provider's job would likely cross administrative and financial departmental lines. AYA are difficult to support anyway; they don't easily admit to needing help and almost never seek it on their own. They shun traditional support groups, especially while on therapy, both because of discomfort with the format and the toll on their precious time (meeting social calendar needs come far before meeting emotional needs).

Lastly, an issue that affects young adult survivors of both childhood and adolescent cancers is the issue of transition of care from a pediatric oncology system to adult health services. This transition is often erratic, uncoordinated, and lacking the necessary transfer of medical information. Individuals at this age are transient, and continued follow-up, currently recommended for all survivors of childhood and adolescent cancers, gets left behind.

In order to create appropriate interventions and treatment methodologies that would increase the likelihood of cure and enhance the quality of life for this patient population, we need to create a focused effort to understand the biology and clinical needs of these adolescents and young adults with cancer. The following are concrete recommendations that have been suggested by interested providers and patient/parent advocates:

• Develop programs or divisions of adolescent and young adult oncology at academic and community cancer centers that bridge pediatric and medical oncology
• Train and credential physicians, nurses, psychologists and social workers in care of adolescent and young adult oncology
• Carry out research on biologic variation of tumors over age spectrum
• Carry out research on effect of treatment variation on survival outcome for more AYA cancers (Hodgkin's disease, Non- Hodgkin's lymphoma, testicular cancer, brain tumors)
• Increase support services geared specifically at AYA patients
• Increase awareness of needs of AYA patients, especially regarding timely diagnosis, appropriate referral, fertility preservation, and psychosocial support (perhaps through a mechanism such as Institute of Medicine White Paper, or NCI Progress Review Group, and via grassroots education)
• Find and remove systematic roadblocks to enrollment on clinical trials
• Encourage universal catastrophic insurance (at least for young adults)
• Carry out research, then implement interventions to aid in physician- patient communication, parent-patient communication, fertility preservation, quality of life including symptom management and depression therapy
• Study transition of care of AYA cancer survivors from pediatric oncology to adult health services

Continued and increased efforts by parents and advocates will encourage the National Cancer Institute, the Children's Oncology Group (COG), and the academic medical community to realize this agenda and the goal of best care, outcomes, and future for adolescent and young adult cancer patients.

Karen Albritton, MD Director, Adolescent and Young Adult Oncology
Dana Farber Cancer Institute
44 Binney St. Boston, MA 02115
617-632-2545
karen_albrittton@dfci.harvard.edu