Smooth Sailing Through The Perfect Storm: A case study in adolescent & young adult (AYA) oncology

Lara E. Davis, MD1, Kellie Nazemi, MD2, Sue Lindemulder, MD2, Brandon Hayes­Lattin, MD3 Oregon Health & Science University, Portland, Oregon, USA

1Divisions of Medical Oncology and Pediatric Hematology/Oncology, 2Division of Pediatric Hematology/Oncology, 3Division of Medical Hematology/Oncology

The Case Discussion

Conclusions

A 29-year-old male presented to a community hospital with a three month history of progressively worsening headache. He sought evaluation when the pain began to interfere with his ability to perform independent activities of daily living. He was found to have a left-sided posterior fossa mass and was referred to our tertiary academic center for resection by the adult neurosurgical service. Histopathology confirmed the diagnosis of medulloblastoma with anaplasia, a predominately pediatric cancer that accounts for <1% of intracranial tumors in adults. The case was reviewed at both the adult and pediatric neuro-oncology tumor boards. His primary oncology care was assigned to the adolescent & young adult (AYA) oncology

This case illustrates many of the factors that have limited progress against cancer in the AYA population as described by the 2006 Progress Review Group of the National Cancer Institute and Lance Armstrong Foundation:

This case, an example of a pediatric cancer occurring in an adult, demonstrates how multidisciplinary coordination can provide excellent cancer care to the AYA population despite multiple challenges. By identifying the most appropriate oncologist to lead treatment decisions and "champions" from other departments to act as liaisons, this patient successfully completed intensive multimodal therapy and remains disease free one year off therapy.

Flair

T2

Challenge Encountered

Relevance to Broader AYA Population Potential Solutions

Limited access to care - Patient had no insurance, no primary care physician and limited financial resources.

No longer covered by parent’s insurance but often without comprehensive benefits through employer

Healthcare reform, including coverage of dependents until age 26 as recently enacted in the United States

Delayed diagnosis - Lack of insurance contributed, as did the patient’s sense that his symptoms weren’t serious.

Sense of invincibility Low degree of suspicion

Raise awareness in AYAs Examples: Websites such as stupidcancer.com; use of social networking sites; popular films like 50/50 Raise awareness in caregivers Example: Nurse Oncology Education Program (NOEP) “At The Crossroads: Cancer in Ages 15-39” videos

Ill-defined treatment setting (“No Man’s Land”) - Referred to adult neurosurgeon initially, then to radiation oncologist, then to pediatric neuro-oncologist.

Treating site often determined by referral pattern rather than expertise Logistical barriers often exist to identifying the most appropriate treatment setting & practice

Educate referral base about unique AYA needs and availability of AYA oncology care Identify institutional & departmental AYA “champions” to break down barriers

Uncertain standard of care - Treated per pediatric standard of care, but limited data exists for this protocol in adults.

Tumor & host biology, drug toxicities, regimen adherence, etc are different from both younger and older patients

Multidisciplinary tumor conferences that incorporate pediatric & adult specialists Increase enrollment on clinical trials to improve understanding of differences seen

Lack of clinical trials - There were no frontline treatment trials available for this patient due to age >22y.

Understudied population Difficult to capture data in a migratory population

Broaden eligibility of current and upcoming pediatric trials to include patients up to age 40 (and adult trials down to age 15) Novel data capture systems (see abstract by Loret de Mola et al) prove that it is feasible to enroll & retain AYA patients

Psychosocial issues - Faced morbidity from disease & treatment, particularly devastating for a young, strong Latino family man who was the head of his household.

Transitioning between child & adult while confronting own mortality Unique financial concerns (supporting young kids, pivotal time in career development, etc) Potential loss of fertility

Ensure clinical care team aware of unique needs and is trained in addressing and/or aware of available resources Establish standard of care policies that incorporate fertility preservation into all diagnostic discussions with AYA cancer patients

Further Information Albritton K, Caligiuri M, Anderson B, Nichols C, Ulman D. Closing the gap: Research and care imperatives for adolescents and young adults with cancer. Report of the adolescent and young adult oncology progress report group. Bethesda, MD: National Cancer Institute; 2006.

Off therapy monitoring MRI brain/spine every 3mo x1y then every 4mo x1y

Chemotherapy Vincristine, cyclophosphamide, cisplatin

given every 28 days x6 (inpatient)

Rest Six weeks

Radiation 31 fractions: 3600 cGy craniospinal with tumor bed boost

to 5580 cGy and concurrent weekly vincristine x8

Surgery Gross total resection

fellow under the co-supervision of a pediatric neuro-oncologist and an adult oncologist. Outpatient care was delivered in the pediatric clinic, while all inpatient admissions were on the adult wards. Radiation was delivered by a radiation oncologist specializing in both adult and pediatric brain tumors. Prior to systemic chemotherapy, fertility preservation was discussed and declined. Following cranio-spinal radiation he was treated per Children’s Oncology Group protocol ACNS 0332. Although the patient’s disease and treatment-related morbidity was mild compared to many with this disease, it had tremendous impact on his life. His fatigue prevented him from working, which impacted the family of four that was financially dependent upon him, and the associated stress contributed to the ultimate failure of his marriage.

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