1. Pancreatic Neoplasms in Children: A Review of
Institutional Experience and Literature
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Presented by: Sebastian Hurtado
Bishop O’Dowd High School, Senior
Mentor: Dr. Wendy Su, M.D.
CHORI Summer Symposium
August 15, 2014

2. Introduction: What We Will Cover
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-Background information regarding pancreatic
malignancies in children
-Summer research project objectives
-Methods of review and analysis
-Results of studies, analysis of trends and data,
collection of images
-Future Directions
-Acknowledgements
-Questions

3. Background Information
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The Pancreas
-Glandular organ
-Digestive and endocrine systems
-Histology of pancreatic tissue: islets
(endocrine/hormonal role), ducts,
and acini (exocrine/digestive role)
-Malignant pancreatic tumors
are extremely rare tumors in
the pediatric population.
The pancreatic cancer
incidence is 0.2% of all
pediatric malignancies
Head Tail
Body

4. Objectives of My Project
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To give the public a better understanding of these rare neoplasms, we aim to:
-Identify and characterize pancreatic neoplasms in children to
provide more readily accessible information for the general medical
community so that timely referral and better patient/family
education can be provided
-Identify and characterize:
-Tumor types
-Presentation and diagnosis
-Treatment
-Prognostic factors and outcome

5. Methods
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-Review the databases of CHO
-Analyze literature on global experiences
-Assess the national Kid’s Inpatient Database
(KID) from the Healthcare Cost and Utilization Project
-Create spreadsheets to synthesize collected data in an organized
form
-Use descriptive statistics such as tables and graphs to represent
statistical trends
-Examine pathology slides of pancreatic neoplastic tissue to better
understand the histology of the disease

6. Results-Types of Pancreatic Tumors found in
children
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The Big Four
-Pancreatoblastoma
-Solid Pseudopapillary Tumor
-Pancreatic Neuroendocrine Tumor
-Pancreatic Ductal
Adenocarcinoma

7. 1) Pancreatoblastoma
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-Derived from
blastoma/pluripotential stem cells
-One of the most common in children
-Mostly occur in early childhood
-Commonly seen in Africans, Asians, and other non-Caucasian races
-Account for 1%-3% of pancreatic tumors in children overall
-Common in teenage females
2) Solid Pseudopapillary Tumor
Squamoid Corpuscle
Pseudopapillae
H&E
Staining
used

8. 3) Pancreatic Neuroendocrine Tumor
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-Common in teenage population
-Associated with MEN 1 syndrome and tuberous
sclerosis complex
-Origins in Langerhan islet cells
Acini
Islet
PNT
Immunohistochemical staining used: The tumor is positive for Synaptophysin,
which is a neuroendocrine cell marker. The islets of Langerhans are also positive

9. 4) Pancreatic Ductal Adenocarcinoma
-Arise from mutations in ductal cells of pancreas (try to for normal ducts)
-Very rare in children
Normal Pancreatic Duct

10. Results-Presentation and Diagnosis
-Detected using CT scans, MRI scans, or
ultrasonography
-Common symptoms include palpable masses,
abdominal pain, jaundice, weight loss, and
nausea
-Common treatments include
resection of the tumor via
pancreaticoduodenectomy or partial/distal
pancreatectomy
-Much better prognosis in children than adults, in
which the majority (80%-99%) do not live past 6
months to 1 year after initial diagnosis
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11. Results-Literature Review
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1) The Memorial-Sloan Kettering Cancer Center Experience,
New York, USA
2) The Italian TREP Project Experience, Italy (nationwide)
3) Kyungpook National University Medical Center Experience,
Korea
4) Bambino Gesu Children’s Hospital Experience, Rome, Italy
5) General Hospital of Vienna, University of Vienna Medical
School Experience, Vienna, Austria
6) Hospital for Sick Children, Toronto, Ontario, Canada
7) Children’s Hospital Research Center Oakland, Oakland, USA

12. The Memorial-Sloan Kettering Experience
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Statistics from retrospective review from 1967 to Present
-17 patients
-2 lost to follow up, 12/15 alive without disease
PB
29%
SPT
41%
PNT
12%
PADC
6%
Others
12%
Distribution of Types
Palpable mass
19%
Jaundice
15%
Weight Loss
23%
Anorexia
14%
Malaise
10%
Nausea
19%
Common Symptoms
PB=5
SPT=7
PNT=2
PADC=1
Others=2 (peripheral
neuroectodernal tumors)

13. The Italian TREP Project Experience
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Statistics on Retrospective Study from Tumori Rari in Eta Pediatrica (TREP) from January
2000-July 2009
-Out of 488 patients with rare tumors, 21 patients with pancreatic tumors identified
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2
4
6
8
10
12
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Males Females
Gender and Type Distribution
PB PADC PNT SPT

14. Conclusions from Memorial Sloan-Kettering
Experience
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-PB more common in males (4/5), SPT more common in females (5/7)
-Positive prognosis=12/15 alive (80% survival rate)
-PB and SPT most common
-PB more common in males=good prognosis, all alive but one (toxic
death)
-SPT more common in teenage females
-Good prognosis for SPT resection: 11/12 alive in 1st remission, 1 relapse
-Pancreatic tumors very rare: 4% of all rare tumors registered
Conclusions from Italian TREP Experience

15. Demographics of Pancreatic Neoplasm Patients at CHRCO
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*Age during treatment; **Metastases/Chemotherapy/other treatment; ***Intermittent lower abdominal pain; ****post-2003, in 2003: AWD

16. CHRCO Conclusions
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-Majority of SPTs are present in teen females (3/4)
-7/9 alive today=excellent prognosis
-Tuberous sclerosis, anemia, or previous abdominal issues are risk factors (warnings)
-Size does not seem to be indicator of specific type
-Most commonly occurring in tail (in females and PNTs)
-SPT most common type
All of these conclusions… …help primary care physicians with parent
consultation/consolation and expand understanding!
45%
11%
33%
11%
Type Distribution in Oakland
SPT PB PNT PADC

17. 16
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10
20
30
40
50
60
70
Alive Without Evidence of Disease Died of Disease Lost to Follow Up
Overall Patient Outcomes
Outcomes
1 Relapse
63 AWD
6 DOD
4 LTFU
58%
15%
14%
8%
5%
Overall Type Distribution
SPT PB PNT PADC Others
SPT=42
PB=11
PNT=10
PADC=6
Others=4
Overall Results

18. Overall Conclusions
-Four main types of pancreatic tumors in children: pancreatoblastoma, solid
pseudopapillary tumor, pancreatic neuroendocrine tumor, pancreatic ductal
adenocarcinoma
-Overall prognosis is much better, with 86% survival rate in our project,
compared to adults with a 1-10% survival rate
-Surgical resection via PT or PDD provided curative treatment
-Specific trends/clues can help determine the type and grade of pancreatic
tumors
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19. Future Directions
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-Portion of project not completed:
-Analysis of KID national database from Healthcare Cost and
Utilization Project
-To continue the project, we could:
-Collect more specific experiences from institutions
worldwide to minimize bias of the representation of only
certain tumor types/institutions
-In the future, we hope to create a presentable database for
surgery conventions

20. Presented by: Sebastian Hurtado
Mentor: Dr. Wendy Su, M.D.
Pediatric Surgical Associates of East Bay
Acknowledgements:
A special thank you to . . .
END
-Wendy Su, M.D.
-The Doris Duke Charitable Foundation
-Ellen Fung, PhD RD CCD
-Ward Hagar, M.D.
-Elaine Cham, M.D. CHRCO Pathologist
-CHORI Summer Student Program Faculty and Staff

21. Answers to Possible Questions
 Hypotheses for better prognosis in children than adults:
-increased check-ups/visits in children, cancer caught at
lower stage
-risk factors from lifestyle choices in adults (i.e. smoking,
alcohol consumption) vs. genetics
-biology of the tumor for each age group (SPT vs.
pancreatic adenocarcinoma): “better” and “worse” types
-types of tumors are different
-overall “youth factor”: younger and more intact
immune system
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