Download “AYA” Cancers?

Serving Adolescents
and Young Adults:
What We’re Doing at USC and Other
Institutions in the U.S.
Your speaker
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Melanie Goldfarb, M.D.
Endocrine Surgeon
USC / Norris Comprehensive Cancer Center
AYA “Thyroid champion”
for the USC AYA program
What were going to talk about
• AYA cancers
– Introduction – What is AYA ?
– Challenges, uniqueness of AYA cancers & patients
– AYA nationwide; AYA@USC
• AYA Thyroid cancers
– What little we know
– Historical philosophy for diagnosis and
treatment
– Areas for research
What is AYA ?
What is AYA ?
• Describes cancers among Adolescents and
Young Adult population ages 15 – 39
• Currently 65,000 – 70,000 AYA cancers
diagnosed each year in the US
– Over 4,000 AYA cancers in Los Angeles
basin alone
• Cancer is the this age group’s leading
disease-related cause of death
What are Common “AYA” Cancers?
Ages 15-29
Top 3
Cancer incidence is illustrated in (Top)
females and (Bottom) males ages 15 to 29
years in the US. Surveillance, Epidemiology,
and End Results (SEER) database from
1995 to 2000 (Bleyer A, O’Leary M, Barr
R, Ries LAG, eds. NIH Pub. No. 06-5767.
#4
#4
25- to 29 year olds
#4
AYA patients are unique
Life transitions
• Adolescence ► Young Adult ►Adulthood
– Shifts in family and peer groups
– Changing and evolving sexual health and
relationships
– Growing independence
– Highly mobile
Independence
• Newfound independence and self awareness +
feelings of invincibility and invulnerability
– Reduced adherence to treatment & follow-up
– Increased likelihood of late stage diagnosis
– Challenges in communication
• AYA – family
• AYA – physician
• AYA – peers
• Family - physician
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Unique needs
• Disruptions in education, employment, and
social life
• Questions about future fertility
• Establishing financial independence
• Navigating the health care system
• Very premature confrontation with mortality
• Changes in physical appearance
• “Long-term follow-up” is really long-term
• Pregnant or a new parent
Survivorship
• The majority of AYA patients become longterm survivors with many potential years of
life ahead of them
Survivorship
• High-risk population with the potential to
develop a wide array of late effects
– For many late effects the risk does not plateau
with aging
– Usually a clinically silent period with intervals as
long as 2 to 3 decades
– Desire to escape the trauma of their cancer
experience may mean that AYA survivors do not
follow-up with their health care; they are hoping
to put the experience of being sick behind them
System challenges of treating
the AYA population
Lack of Dedicated AYA centers
• Very few pediatric and adult centers have
programs that assist AYA patients
– Unable to address the many unique needs of AYA
• Very few centers have reserved inpatient and
outpatient space
– AYA patients are surrounded by patients much
older or younger than themselves
– Increases sense of isolation and reinforces their
perception of being different from their peers
Access to care
• Higher rates of being unemployed, under- or
uninsured
• Lack of a primary care physician
• Difficulty in obtaining appropriate referral
• Continuum of access to care
Lack of Research & clinical trials
Enrollment in clinical trials lags
greatly among AYA population:
A very similar experience to
pediatric cancers in the 1970’s.
AYA Participation in US Clinical Trials
(A. Bleyer)
Research and clinical trials???
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Mobile population – difficult to track
“Avoidance” of the issue in AYA patients
Not want further disruptions to their life
Not appreciate need for follow-up and
research
• Companies and centers have not recognized
the need for dedicated AYA trials and
research
AYA nationwide
AYA programs in the US
• Only a handful of established programs
– Most in early stages of development
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AYA programs in the US
Ages 15-29
Ages 18-39
Ages 15-39
Ages 15-29
Ages 15-25
Ages 15-39
Ages 15-21
Ages 15-30
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AYA community is growing
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www.ihadcancer.com
www.stupidcancer.com
www.planetcancer.org
www.15-40.org
http://www.facebook.com/ayacancer
http://www.teenslivingwithcancer.org
http://www.livestrong.org/
http://www.ulmanfund.org
http://www.youngcancerspouses.org/
http://www.pregnantwithcancer.org/
http://imermanangels.org/index.php
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AYA in the media
Goals of Risk-Based Healthcare
of AYA Cancer Survivors
• Multidisciplinary team approach with communication between
the primary healthcare provider, specialists of pediatric and
adult medicine, and allied/ancillary service providers
• Healthcare of the whole person, not a specific disease or organ
system, including individual’s family, cultural & spiritual values
• Sensitivity to the issues of the cancer experience, including
expressed and unexpressed fears of the survivor
• Comprehensive, anticipatory, proactive care that includes a
systematic plan of prevention and surveillance
• Longitudinal care that is considered a continuum from cancer
diagnosis to eventual death, regardless of age
• Continuity of care consisting of a partnership between the
survivor and a single healthcare provider or program who can
coordinate necessary services
Oeffinger KC. Curr Probl Cancer. 2003;27:143-167.
Empowering the AYA patient
• Individual self-advocacy
– Seeks out information
– Communicates with their healthcare team as an
equal partner
– Is involved in their treatment decisions
• Regional and national advocacy
– Community building: like-minded individuals
connect and support each other
– Combine efforts of family, community, and
societal members to promote awareness and
change
AYA@USC
• USC/CHLA will have the largest AYA program
in Southern California
• USC’s program will be the only NCI
designated comprehensive cancer center
with an AYA program in California and the
Southwestern US
• www.uscnorris.com/about/programs/aya.html
Goals
• Establish a cooperative, multi-disciplinary, patientcentered care model to treat the AYA patient
• Improve AYA patient population’s quality and
survival outcomes
• Develop a robust research program emphasizing
basic, clinical, and translational research
• Create models of care, best practices, and
protocols for treating and curing the AYA patient
• Emphasize the knowledge transfer and education
of healthcare professionals on AYA
• Communicate and raise awareness to the USC and
Los Angeles basin communities about AYA
Services for AYA patients and families
• Overall disease
education
• Anxiety and
depression
• Reproductive
Health including
fertility
preservation
• Genetic counseling
– Tumor
– Family history
• Physical and
Occupational tx
– Group/
Individual
Support
• Peer
• Family
• Friends
• Spousal
• Children
– Relationship
health
– Financial
resource
planning
– Religious/ Faith
needs
– Employment
and educational
counseling
– Peer /
Professional
Networking
– Pain
management
– Dietary and
nutrition needs
– Legal
AYA “Collaborative” Model:
Integrated Research Core
• Collaborative relationship with AYA Care Team
– AYA Patient Coordinator, nursing staff
– AYA Medical director
– Psychosocial services
– Support services
– Survivorship
• Integrated Clinical, Basic, and Population studies
• Emphasis on quality outcomes through strengthened
clinical care and psycho-social support
AYA Thyroid Cancers
What little we know….
• Mean age at presentation for all thyroid
cancers is 3rd decade (AYA)
• Younger (<20) (AYA) and older (>60) present
with more extensive disease
• ONLY cancer where age (< 40 or 45) is part
of staging!! (AYA)
In all age groups…
• Thyroid cancer is increasing
• Majority are papillary or a follicular variant
• Excellent overall survival
• Females better long-term survival vs males
Risk Factors
• Most important risk factor is a history of
head and neck radiation, especially at very
young ages
– In a study of 16,500 leukemia survivors, thyroid
carcinoma was #1 second malignancy in patients
with a history of HD and NHL and #3 in patients
with a history of leukemia (Maule et al, 2007).
– 53X increase risk at 2 years from initial cancer dx
(Tucker et al, 1991)
– Cancer risk continues for up to 30 years
– Average latency period 8.4 years (Winship & Rosvoll, 1970)
Thyroid nodules
• 13% in autopsy studies ages 18-39 (AYA)
(Oertel, Humana Press 2000)
– 1.5% young kids and up to 70% in adults
• About 25% of “pediatric” thyroid nodules (<
21 yrs old) are malignant
– 4-5X increased incidence of malignancy
compared to adults
– ? AYA
But…
• Almost all studies are done in either the
“adult” (≥ 18 or 21 years old) or “pediatric”
age group (≤ 18 or 21 years old)
• It’s difficult to tease out AYA specific
conclusions
Where does AYA fit ?
Comparison of children and adults with PTC
Parameter
Children (%)
Adults (%)
p
DNA nondiploid
10
25
0.047
Nodal metastases
90
35
0.001
Extrathyroidal invasion
24
16
0.100
Distant metastases
7
2
0.001
Local soft tissue recurrence
12
5
0.080
Regional nodal recurrence
30
7
0.001
Late distant metastases
6
5
1.000
• Greater PTC causal mortality
in patients > 40 years old
Zimmerman et al 1988
Historical philosophy for
diagnosis and treatment
Importance of age
– Staging and Prognosis
Importance of age
• Young patients (<20 years old)
– Thyroid more sensitive to external radiation
– Tumors more likely to be sensitive to RAI
– Tumors “classic”/solid variant > regular PTC
– Genetic mutations: RET/PTC > BRAF
– Increased recurrence rate
– Higher survival rate
– Higher rates of lymph node involvement,
multicentricity, distant mets
Importance of age
• 20 - 44 years old
– Generally low-risk patients
– Lower recurrence rates
– RAI sensitive tumors
AYA
paper
• Differences in
expression in a
few candidate
genes
• But no distinct
difference in
cluster gene
profiles or
number of
mutations
Menno R. Vriens, et al. Clinical and Molecular Features of Papillary Thyroid Cancer in Adolescents and Young Adults
Treatment strategy
in 2011
The basics
*** Surgeon Performed
Head and Neck Ultrasound ***
• Neck ultrasound has become an extension of
the physical exam for many clinicians
• Information on: size, shape, composition of
nodules, other thyroid lobe for additional
nodule,; suspicious lymph nodes
• Preoperative planning
Surgery
• Total thyroidectomy
- ? tumors < 1cm
• Lymph nodes ?
– Any suspicious LN on ultrasound in conjunction
with a suspicious nodules is cancer until proven
otherwise
– No “berry picking” of lymph nodes
– Controversy over routine central neck dissection
• Cosmetically favorable incision
Post-op care
• Thyroid hormone suppression
• RAI ???
– Definitely for extensive disease
– Controversy over “low-risk” group –
most of AYA
Areas for research in
AYA Thyroid Cancer
Questions
• Initial presentation
– Are more nodules self-discovered ?
– Are there longer times from first
“symptom” to diagnosis?
(differences in “access to care”; maybe AYA
patients less likely to have PCP or
insurance)
Questions
• History of head and neck radiation
– How often to screen
– Different tumors?
– Do screening modalities work the same
(ultrasound characteristics and FNA
biopsy)
Questions
• Does everyone need post-operative RAI?
– Risk vs benefit
– Can molecular markers play a role in risk
assessment
– How often and how to screen for second
malignancies
RAI and second malignancies
Anna M. Sawka et al. Second Primary Malignancy Risk After Radioactive Iodine Treatment for Thyroid Cancer:
A Systematic Review and Meta-analysis
RAI and second malignancies
N. Gopalakrishna Iyer, et al. Rising Incidence of Second Cancers in Patients With LowRisk (T1N0) Thyroid Cancer Who Receive Radioactive Iodine Therapy
In 2005, close to 70% of low-risk
cancer patients getting RAI therapy
Last 20 years, rates second malignancy,
especially leukemia, greatly increased
Questions
• Why are AYA thyroid cancers different?
– Effect of estrogen ?
– Genetic mutations ?
– Intrinsic biological difference in thyroid
gland ?
– Better immune system ?
Summary
• AYA: patients 15-39 years old
• “Overlooked” patient population
• AYA patients have
– Different predominant cancer types (Thyroid)
– Unique needs surrounding their cancer care
– Unique tumor biology
• Benefit from a multidisciplinary care approach
Thank You