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Pancreatic Tumors
Also covers: pancreatoblastoma · pancreatic ductal adenocarcinoma · insulinoma · gastrinoma · multiple endocrine neoplasia type 1 · hypoglycemia
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
Content of this collection
The diagnosis of autoimmune pancreatitis in children can be made with a combination of clinical and imaging findings because the risk of pediatric neoplasm is lower than autoimmune pancreatitis. — Jaimie Nathan, Pancreatic Masses · 33:50
Pancreatic Masses
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Drs Jaimie Nathan, Rae Hanke, Alex Gibbons, and Todd Ponsky discuss diagnosis and management of pancreatic masses Intro and outro tracks are adapted from "I dunno" by grapes, featuring J Lang, Morusque. Artist URL: ccmixter.org/files/grapes
podcast37:28 · Dec 2020
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Expert statements from the recordings in this collection.
Pancreatic Masses
Elevated lipase and elevated bilirubin suggest obstruction in the pancreatic head, which can be caused by neoplasms or more commonly in pediatrics by non-neoplastic findings such as autoimmune pancreatitis or complications of pancreatitis.
Jaimie Nathan1:21
Pancreatoblastoma is the most common malignant pancreatic tumor in children, typically presenting in patients less than 10 years of age.
Jaimie Nathan5:14
In pancreatoblastoma, alpha-fetoprotein is elevated in up to 80% of cases.
Jaimie Nathan5:40
Up to 45-50% of pancreatoblastoma cases present with metastases.
Jaimie Nathan5:50
Pancreatoblastomas respond well to chemotherapy with a cisplatin and doxorubicin-based regimen.
Jaimie Nathan6:00
The number one prognostic factor for pancreatoblastoma is complete surgical excision, whether at initial presentation or following neoadjuvant chemotherapy.
Jaimie Nathan6:10
Solid pseudopapillary neoplasms are more common in young female patients, typically in their second or third decade of life.
Jaimie Nathan6:25
Solid pseudopapillary tumors are indolent and slow-growing, often presenting with very large masses in the body and tail of the pancreas.
Jaimie Nathan6:40
Enucleation or simple biopsy of solid pseudopapillary neoplasms should be avoided due to high recurrence rates; complete surgical resection is required.
Jaimie Nathan6:55
Solid pseudopapillary neoplasms have a recurrence rate of up to 10% but excellent long-term survival with 95% 10-year survival.
Jaimie Nathan7:04
Neuroendocrine tumors make up about 1-2% of all pancreatic tumors and can be either benign adenomas or malignant carcinomas.
Jaimie Nathan9:30
Neuroendocrine tumors tend to present in children over 10 years of age, though they are more common in middle-aged patients.
Jaimie Nathan9:45
In 10% of patients, neuroendocrine tumors may present in the setting of multiple endocrine neoplasia type 1, von Hippel-Lindau, or tuberous sclerosis.
Jaimie Nathan9:55
Insulinoma is the most common neuroendocrine tumor, accounting for almost 50% of pancreatic neuroendocrine tumors, followed by gastrinomas at 30%.
Jaimie Nathan10:10
Insulinomas are typically benign; 6% can be malignant. 90% are solitary, 10% are associated with MEN1.
Jaimie Nathan10:25
Insulinomas present with Whipple's triad: symptoms of hypoglycemia, low fasting blood glucose, and symptom resolution with glucose administration.
Jaimie Nathan10:40
On imaging, a solid lesion in the pancreas is more worrisome than a cystic lesion when found incidentally.
Jaimie Nathan12:00
Ultrasound is low cost and easily accessible but the pancreas is often suboptimally visualized and characterization of pancreatic masses is poor.
Jaimie Nathan13:40
CT scan is rapidly acquired with good resolution but has downsides of radiation and need for contrast; it is often used for solid tumor staging.
Jaimie Nathan14:00
MRI provides better differentiation between solid and cystic or fluid components and can better characterize components of a pancreatic mass.
Jaimie Nathan14:20
Cross-sectional imaging (CT or MRI) cannot confidently differentiate a benign versus malignant pancreatic lesion.
Jaimie Nathan15:03
A completely cystic pancreatic lesion is less concerning for malignancy, but if the mass has solid components, it becomes more concerning for neoplasm.
Jaimie Nathan15:25
Pediatric EUS is not practical in many institutions because there are not many practitioners in the pediatric GI community comfortable with pediatric EUS.
Jaimie Nathan17:20
In the pediatric realm, autoimmune pancreatitis is more common than pancreatic neoplasm.
Jaimie Nathan18:24
With negative biliary cytology and after biliary and pancreatic duct stenting, a four-week steroid trial with taper can be initiated for presumed autoimmune pancreatitis, even if IgG4 findings are normal.
Jaimie Nathan18:35
Type 1 autoimmune pancreatitis is IgG4-mediated; type 2 autoimmune pancreatitis is IgG4-negative.
Jaimie Nathan19:05
Autoimmune pancreatitis with a pancreatic head mass is typically very steroid responsive, with rapid resolution of the mass in most cases.
Jaimie Nathan20:03
Studies report up to about a 10% risk of diabetes (endocrine impairment) after just a distal pancreatectomy in the setting of otherwise normal pancreas.
Jaimie Nathan23:20
For malignant pancreatic head lesions, a radical resection (Whipple pancreaticoduodenectomy) is required; for benign or borderline benign lesions, duodenum-preserving pancreatic head resections (Beger or Berne procedures) may be considered.
Jaimie Nathan22:40
Enucleation may be appropriate for pancreatic neuroendocrine tumors but should be used sparingly; it is not recommended for solid pseudopapillary neoplasms due to higher recurrence risk.
Jaimie Nathan24:50