1. Pancreatic Tumors in Children Presented by Damien W. Carter, MD

2. JW 10y/o Male. No PMH Presented to ED with epigastric abdominal pain 6/10. Exam: Palpable epigastric mass Chem7, CBC, LFT’s  WNL Amylase 60, Lipase 39 Abdominal Ultrasound- Pancreatic Body mass

5. JW Sent Tumor Markers  WNL AFP (Pancreatoblastoma, Hepatoma) CA19-9 (AdenoCA) CEA (Gastrointestinal malignancies) β-HCG (Chorio & other germ cell tumors) VIP (VIPoma) Glucagon (Glucagonoma) Gastrin (Gastrinoma) C-Peptide (Insulinoma)

6. Differential Diagnosis All types of Pancreatitis may form ‘masses’ that can be mistaken for tumors (cystic & solid) on imaging. Benign & Premalignant Serous Cystadenoma IPMN (all grades) MCN Malignant Ductal AdenoCA- >85% IPMN w/ Invasion ≈2-3% MCN w/ Invasion- 1% Pancreatoblastoma-1% Acinar Cell CA- 1% Serous cystadenoCA-1% Solid Pseudopapillary Neoplasm- 1% Resectable Proceed with resection without biopsy Staging laparoscopy Consider Biopsy for Prognosis vs. adjuvant /neoadjuvant Tx No Yes

7. Surgical Management Goal is to resect neoplasm with ‘clear margins’ Enucleation Distal Pancreatectomy Regional pancreatectomy w/w/o Roux-y Drainage Total Pancreatectomy Whipple Procedure (Tumors involving the pancreatic head & duodenum)

8. JW OR: Exploratory Laparoscopy ‘Enucleation’ of Pancreatic Mass. Findings: Spongy tennis ball size mass w/ adhesion to splenic, SMA/SMV & portal vessels Two drains placed in pancreatic bed Proximal duct entering mass oversewn. No distal duct identified Pathology: Solid Pseudopapillary tumor. No evidence of metastasis Largest area 5.5x5.5x3cm extensive surrounding necrotic & inflammatory tissue

9. Solid Pseudopapillary Neoplasm Occur predominantly in females (>90%) Mean Age 20yrs/old. Only pancreatoblastoma has similar predominance in young Begin as solid neoplasm  become cystic. With large size, typical necrotic core Considered very low grade w/ low malignant potential and usually excellent long term survival Resection with clear margins usually curative Polygonal cells w/ moderate cytoplasm. Mostly solid, with areas of cystic change(Green arrow).

10. Solid Pseudopapillary Neoplasm Reddy et. Al. JACS 2009 Single institution retrospective outcomes study Pt’s w/ SPN 1970-2008 89% (33/37) female. Mean age 32y/o Most were symptomatic (81% Abd pain) 36 pts. Underwent resection, 1 had distant mets. Pt. w/ mets died in 11 months. After resection, 34 pts remained alive(94%) Of those with resection, 33 remain disease free @ 4.4yrs(mean). 2 died of unrelated causes. One patient with a recurrence 8 yrs after resection, was treated with Gemcitabine and was alive 2yrs at time of publication. Yang et Al. Am J. Surg, 2009 Case series at a single chinese institution. 26 consecutive (22 female). Similar distribution between head & body/tail of pancreas. All Pts. Except 1 were alive at f/u (33 months). The pt. who died was noted to have Ki-67 immunoreactivity >25%. Developed liver mets and local recurrence and died 6months after surgery.

11. Summary Pancreatic tumors are rare in children & adolescents Pancreatic neoplasms that are most frequently encountered in young patients, are most often amenable to curative resection All forms of pancreatitis can form “pseudotumors” Solid pseudopapillary neoplasms are almost always cured with surgical resection Excellent longterm survival is the norm with SPN’s

12. JW Postop Course Weaned off PCA pain control by POD#4 Decreasing JP output after starting PO diet JP Amylase 35K Pancreatic enzymes downtrending Anticipating discharge with drains in place within next few days

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