imageOrlando, Fla.—In recent years, a niche of medicine has sprung up to address the needs of adolescent and young adults (AYA) with cancer.

AYA oncology now has its own society and its own journal, and in March, the National Comprehensive Cancer Network (NCCN) issued its first set of AYA guidelines.

The attention to this group of cancer patients is needed. In the past 40 years of the battle against cancer, survival rates of children and older adults have improved significantly, whereas survival rates among AYAs with cancer have barely budged.

“Improvements in cancer survival among AYA patients definitely have lagged behind other sectors of the population,” said Kerry Parsons, PharmD, pediatric oncology pharmacist at Children’s of Alabama, in Birmingham, who discussed AYA oncology at the annual meeting of the Hematology/Oncology Pharmacy Association.

According to the American Cancer Society, 70,000 AYAs, defined as patients aged 15 to 39 years, are diagnosed with invasive cancer each year in the United States. (For a breakdown of cancer types, see Table 1.) Some of those malignancies are associated with a poorer prognosis due to either more aggressive or unique biology features, including breast cancer, colorectal cancer, soft tissue sarcoma, non-Hodgkin’s lymphoma and acute leukemia, according to Leonard Sender, MD, the founder of the Society for Adolescent and Young Adult Oncology (SAYAO), and director of the combined adolescent and young adult cancer program at Children’s Hospital of Orange County and University of California, Irvine. Dr. Sender added that AYAs often do better on more rigorous pediatric protocols than adult protocols, but many doctors are not aware of this.

Table 1. The Most Common AYA Cancers per 100,000 People in the United States
Cancer Type Ages 15-39 Years
Breast cancer 20.4
Thyroid cancer 14.6
Melanoma 9.5
Cervix and uterine cancer 9.1
Hodgkin’s lymphoma 3.7
Colorectal cancer 3.4
Gonadal germ cell tumors 10.1
Melanoma 5.5
Non-Hodgkin’s lymphoma 4.7
Colorectal cancer 3.6
Thyroid cancer 2.9
Source: SEER Cancer Statistics Review, 1975-2008, National Cancer Institute. Bethesda, MD., based on November 2010 SEER

A 2007 study on AYA cancer trends offers some sobering statistics on just how wide the survival gap has become in this vulnerable patient population. Fourteen cancer types in AYA patients have five-year survival rates that are significantly lower than rates in younger patients, the authors reported (CA Cancer J Clin 57;242-255). Of those, six cancer types have survival rates that lag behind both younger and older patients.

Moreover, “cancer kills more 20- to 30-year-olds than any other disease except depression-induced suicide,” the authors noted. “Yet cancer in young adults has been under-recognized and frequently not considered” by clinicians who often come into contact with these patients—“including oncologists.”

Major Culprit: Clinical Trials Access

According to Dr. Parsons, the factor most responsible for this lack of progress in AYA oncology is inadequate access to clinical trials. “During the period of 1997 and 2003, approximately 70% of children were enrolled in an NCI-sponsored clinical trial,” she said. Unfortunately, “this level of clinical trial participation drops dramatically as you span into the AYA age range and falls to less than 2% [for patients] between 20 and 39 years of age.” The new AYA NCCN guidelines strongly advise that AYA patients be placed on clinical trials and referred to cancer centers with experience in treating cancer patients in this age group.

Dr. Parsons said that a 20-year-old Marine with osteosarcoma was recently referred to her hospital because it has been gaining a reputation as a center with AYA expertise. Her hospital was able to place him in a clinical trial. “We don’t need a fancy facility to care for these patients,” she said. “In my practice at the University of Alabama, we don’t necessarily have a formal program, although we are moving that way, but over the past few years, we have had a lot more phone calls and discussions between our physicians and our pharmacy colleagues about managing AYA patients.” Dr. Parsons pointed out that strides are being made in terms of getting AYAs into clinical trials for sarcoma. The percentage of AYA patients enrolled in NCI clinical trials for sarcoma was 5% during 1998 and 1999 and 19% during 2004 and 2005, she said.

In addition to inadequate research opportunities, poor access to health care, delayed diagnosis and treatment and poor adherence to treatment regimens also play a role in the survival rates of AYAs not improving over the years. She noted that a 2010 Gallup poll identified AYAs as the most underinsured age among all age ranges.

Signs of Progress

All is not dire, however, in the field of AYA cancer. Since 2006, the NCI has collaborated with the Lance Armstrong Foundation and published a series of reports on AYAs with cancer, in an effort to bolster awareness. In 2010, Dr. Sender helped found the SAYAO. In 2011, the quarterly Journal of Adolescent and Young Adult Oncology was launched. The NCCN AYA oncology guidelines released in March are the latest big development in this growing field (Table 2). The guidelines aim to help improve the diagnosis, treatment and survivorship care of AYAs. (Access to the NCCN Guidelines for AYA Oncology or any of the NCCN guidelines is available free of charge at

Table 2. Highlights From the NCCN Adolescent and Young Adult Oncology Guidelines
AYA patients usually are more tolerant of intensive therapies than older patients.
Monitoring of cumulative dosing for certain medications (anthracylines, epipodophyllotoxins, cisplatin, ifosfamide) associated with irreversible organ damage may be essential when certain lifetime exposure is encountered.
Fertility preservation should be an essential part in the management of AYAs with cancer.
AYA patients with cognitive dysfunction or other psychiatric symptoms (e.g., depression or anxiety) should be referred to a mental health provider and community-based resources serving AYA patients.
All AYAs should have a survivorship care plan.
AYAs with cancer need long-term follow-up care for monitoring and treatment of late effects long after completion of treatment. This can include screening for pulmonary, thyroid, cardiovascular and audiologic issues; breast, colorectal and bladder cancer; acute myeloid leukemia; myelo-dysplasia; and kidney disease.

According to Dr. Parsons, physicians can do their part by paying particular attention to toxicity and dosing considerations in this population because of different pharmacokinetics. The oral clearance of dexamethasone and methotrexate, for example, is twofold greater for young children than for adolescents, and there is an inverse relationship in AYAs between age and clearance of etoposide and busulfan. Pharmacists should be aware that when using dactinomycin, the area under the curve is lower in adolescents than in young children, she said.

Through acute lymphoblastic leukemia (ALL) clinical trials run by the Children’s Oncology Group, researchers have found that patients between the ages of 16 and 30 years experience higher rates of toxicities than children aged 1 to 15 when it comes to hyperglycemia (22% vs. 15.4%), hyperbilirubinemia (6.7% vs. 3.7%), oral mucositis during interim maintenance therapy (18.5% vs. 11.3%) and peripheral neuropathy (11.5% vs. 7.4%; Blood, ASH Annual Meeting abstracts, 2011;118: Abstract 1510). Osteonecrosis is far more prevalent in ALL patients 10 years or older than in patients younger than age 10 (19.6% vs. 3.1%; J Clin Oncol 2011;29: Abstract 9504).

Survivorship Issues Need To Be Addressed

AYAs have specific survivorship issues that need attention, including financial pressures, psychosocial issues and medical concerns such as fertility. According to Dr. Sender, psychological distress is significantly greater in AYAs than in younger children and older patients with cancer. Thus, health care workers need to focus on managing the psychosocial issues of this particular group of patients. The AYA NCCN guidelines echo the need for specific attention to psychosocial issues.

Dr. Parsons stressed that there still is a need to increase the overall awareness among the medical community as well as the lay community about the specific needs of AYA patients. “The AYAs represent a unique enough cancer diagnosis that argues for the emergence of AYA oncology as a specialty practice in medicine,” she said.

Dr. Sender agreed and said practitioners can dive into the subject matter using online resources. “With Google and PubMed, you can bring yourself up to speed pretty quickly, if you know [how] to look for it. You need to know that the term ‘young adult’ needs to be put into a search when you have a leukemia or a sarcoma,” he said. He hopes the growing awareness of AYA oncology will spur health care providers to specialize in the niche. “There is a really rewarding career in doing young adult medicine,” Dr. Sender said.

—Kate O’Rourke