Pancreatic Tumors
Also covered as: 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
Case-Based Learning
1 item
Pancreatic Masses
Listen →
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
In-Depth Reviews
1 item
Pancreatic Tumors. Presented by David Skarda, MD 04.10.23 Video
Watch →
Dr. Skarda reviews classification and management of pediatric pancreatic tumors, focusing on benign cystic lesions including serous and mucinous cystadenomas and mature cystic teratomas. Discussion covers imaging characteristics, malignant
video50:30 · Apr 2023
Summaries and takeaways+ Show
Summary of this collection+ Show
Key points, with the moment each was said+ Show
Featured picks for families are being prepared — the summaries above are written for you.
Takeaways+ Show
Pancreatic tumors in children encompass a heterogeneous spectrum with distinct biological behavior. Cystic lesions include serous cystadenomas—benign but occasionally symptomatic, cured by resection [e6583-c1, e6583-c2]—and mucinous cystadenomas, which are female-predominant, tail-located, and potentially pre-malignant [e6583-c3, e6583-c4]. Solid pseudopapillary tumors show bimodal age distribution: the most common malignancy in young children (mean 5 years, male-predominant) and in young women (mean 28 years) . Complete resection yields excellent survival despite 15–20% presenting with metastases; enucleation is inadequate [e6583-c12, e6583-c13, e6583-c14]. Pancreatoblastoma associates with Beckwith-Wiedemann and FAP, with AFP elevation useful for surveillance [e6583-c8, e6583-c9]. Acinar cell adenocarcinoma in young children carries favorable prognosis after complete resection without adjuvant therapy [e6583-c16, e6583-c17]. Neuroendocrine tumors include insulinomas—small, encapsulated, diagnosed early via Whipple's triad and C-peptide [e6583-c19, e6583-c20, e6583-c21, e6583-c22]—and gastrinomas, 60% duodenal rather than pancreatic . MEN1 confers multicentric disease and higher malignancy risk in both tumor types [e6583-c24, e6583-c25]. Endoscopic ultrasound guides insulinoma localization and duct-relationship assessment .
- Solid pseudopapillary tumors require formal resection with clear margins; enucleation is inadequate despite excellent survival even with metastatic disease at presentation. [e6583-c12, e6583-c13, e6583-c14]
- Acinar cell adenocarcinoma in young children has markedly better prognosis than ductal adenocarcinoma and does not require adjuvant therapy after complete resection. [e6583-c16, e6583-c17]
- MEN1-associated insulinomas and gastrinomas are multifocal with higher malignancy rates; 60% of gastrinomas localize to the duodenal wall, not pancreas. [e6583-c10, e6583-c24, e6583-c25]
- Insulinoma diagnosis requires Whipple's triad plus C-peptide to exclude factitious hypoglycemia; endoscopic ultrasound localizes tumors and defines pancreatic duct relationship pre-enucleation. [e6583-c21, e6583-c22, e6583-c26]
- Mucinous cystadenomas are pre-malignant, female-predominant, tail-located lesions; differentiation from cystadenocarcinoma is challenging due to sampling limitations of dysplastic foci. [e6583-c3, e6583-c4, e6583-c15]
For patients & families
Pancreatic tumors in children come in several types, each with different characteristics. Some are benign (non-cancerous) cysts that doctors may watch or remove depending on symptoms [e6583-c1, e6583-c2, e6583-c3, e6583-c4]. Others, like mature cystic teratomas, can develop anywhere in or near the pancreas and are removed because they might contain cancer [e6583-c5, e6583-c6]. The most common cancerous pancreatic tumor in young children is called pancreatoblastoma, typically appearing around age 5 and more often in boys . It's sometimes linked to genetic conditions and can be tracked with a blood marker called alpha-fetoprotein [e6583-c8, e6583-c9]. Another tumor type, solid pseudopapillary tumors, occurs mainly in young women around age 28 and has very good survival rates when completely removed with surgery [e6583-c11, e6583-c12, e6583-c13, e6583-c14]. Some tumors produce hormones that cause symptoms—insulinomas make too much insulin, leading to low blood sugar episodes [e6583-c19, e6583-c20, e6583-c21]. Doctors use special imaging and blood tests to find these tumors and determine the best treatment approach [e6583-c26, e6583-c22]. Complete surgical removal is the main treatment for most pancreatic tumors in children [e6583-c12, e6583-c17].
Pancreatic tumors in children come in several types, each with different characteristics. Some are benign (non-cancerous) cysts that doctors may watch or remove depending on symptoms [e6583-c1, e6583-c2, e6583-c3, e6583-c4]. Others, like mature cystic teratomas, can develop anywhere in or near the pancreas and are removed because they might contain cancer [e6583-c5, e6583-c6]. The most common cancerous pancreatic tumor in young children is called pancreatoblastoma, typically appearing around age 5 and more often in boys . It's sometimes linked to genetic conditions and can be tracked with a blood marker called alpha-fetoprotein [e6583-c8, e6583-c9]. Another tumor type, solid pseudopapillary tumors, occurs mainly in young women around age 28 and has very good survival rates when completely removed with surgery [e6583-c11, e6583-c12, e6583-c13, e6583-c14]. Some tumors produce hormones that cause symptoms—insulinomas make too much insulin, leading to low blood sugar episodes [e6583-c19, e6583-c20, e6583-c21]. Doctors use special imaging and blood tests to find these tumors and determine the best treatment approach [e6583-c26, e6583-c22]. Complete surgical removal is the main treatment for most pancreatic tumors in children [e6583-c12, e6583-c17].
The doctors in this collection+ Show
All expert statements+ Show
Every expert statement below comes from the recorded discussions, with its speaker and moment.
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.
clinicalJamie Nathan1:21 ↗
Pancreatoblastoma is the most common malignant pancreatic tumor in children, typically presenting in patients less than 10 years of age.
epidemiologicalJamie Nathan5:14 ↗
In pancreatoblastoma, alpha-fetoprotein is elevated in up to 80% of cases.
clinicalJamie Nathan5:40 ↗
Up to 45-50% of pancreatoblastoma cases present with metastases.
epidemiologicalJamie Nathan5:50 ↗
Pancreatoblastomas respond well to chemotherapy with a cisplatin and doxorubicin-based regimen.
clinicalJamie Nathan6:00 ↗
The number one prognostic factor for pancreatoblastoma is complete surgical excision, whether at initial presentation or following neoadjuvant chemotherapy.
clinicalJamie Nathan6:10 ↗
Solid pseudopapillary neoplasms are more common in young female patients, typically in their second or third decade of life.
epidemiologicalJamie Nathan6:25 ↗
Solid pseudopapillary tumors are indolent and slow-growing, often presenting with very large masses in the body and tail of the pancreas.
clinicalJamie Nathan6:40 ↗
Enucleation or simple biopsy of solid pseudopapillary neoplasms should be avoided due to high recurrence rates; complete surgical resection is required.
clinicalJamie Nathan6:55 ↗
Solid pseudopapillary neoplasms have a recurrence rate of up to 10% but excellent long-term survival with 95% 10-year survival.
epidemiologicalJamie Nathan7:04 ↗
Neuroendocrine tumors make up about 1-2% of all pancreatic tumors and can be either benign adenomas or malignant carcinomas.
epidemiologicalJamie Nathan9:30 ↗
Neuroendocrine tumors tend to present in children over 10 years of age, though they are more common in middle-aged patients.
epidemiologicalJamie 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.
clinicalJamie Nathan9:55 ↗
Insulinoma is the most common neuroendocrine tumor, accounting for almost 50% of pancreatic neuroendocrine tumors, followed by gastrinomas at 30%.
epidemiologicalJamie Nathan10:10 ↗
Insulinomas are typically benign; 6% can be malignant. 90% are solitary, 10% are associated with MEN1.
epidemiologicalJamie Nathan10:25 ↗
Insulinomas present with Whipple's triad: symptoms of hypoglycemia, low fasting blood glucose, and symptom resolution with glucose administration.
clinicalJamie Nathan10:40 ↗
On imaging, a solid lesion in the pancreas is more worrisome than a cystic lesion when found incidentally.
clinicalJamie Nathan12:00 ↗
Ultrasound is low cost and easily accessible but the pancreas is often suboptimally visualized and characterization of pancreatic masses is poor.
clinicalJamie 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.
clinicalJamie Nathan14:00 ↗
MRI provides better differentiation between solid and cystic or fluid components and can better characterize components of a pancreatic mass.
clinicalJamie Nathan14:20 ↗
Cross-sectional imaging (CT or MRI) cannot confidently differentiate a benign versus malignant pancreatic lesion.
clinicalJamie 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.
clinicalJamie 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.
clinicalJamie Nathan17:20 ↗
In the pediatric realm, autoimmune pancreatitis is more common than pancreatic neoplasm.
epidemiologicalJamie 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.
clinicalJamie Nathan18:35 ↗
Type 1 autoimmune pancreatitis is IgG4-mediated; type 2 autoimmune pancreatitis is IgG4-negative.
clinicalJamie Nathan19:05 ↗
Autoimmune pancreatitis with a pancreatic head mass is typically very steroid responsive, with rapid resolution of the mass in most cases.
clinicalJamie 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.
epidemiologicalJamie 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.
clinicalJamie 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.
clinicalJamie Nathan24:50 ↗
What's new+ Show