Pancreatic Tumors in Children
Isolina R. Rossi
An extremely rare entity, and until 2004, studies relied on case reports and series for their data.
Most recently, the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) published data from 1973 to 2004.1
The first successful laparoscopic distal pancreatectomy in a child was documented in 2003.2
RELEVANT ANATOMY
Normal pancreas anatomy is demonstrated in Figure 49.1.
Lying in an oblique orientation, the pancreas is a retroperitoneal organ.
There are 4 regions to the pancreas: head, neck, body, and tail.
Its head rests in the C-shaped duodenum, anterior to the vena cava, right renal artery, and both renal veins. The common bile duct is in the deep posterior groove behind the head.
The splenic artery and vein run along the posterosuperior aspect of the body (Figure 49.2).
The lesser sac lies in the space between the pancreas and the stomach, which are lined by peritoneum.
A plane can usually be developed between the neck of the pancreas and the portal and superior mesenteric vein during a pancreatic resection.
The vascular supply to the pancreas originates from the celiac trunk and is shared with the duodenum.
The exception to this rule is the inferior pancreatic head and uncinate process, which is supplied by the superior mesenteric artery.3
 Figure 49.1 Normal pancreas anatomy. A diagram (A) and an ultrasound in transverse plane (B) demonstrate the normal anatomy of the pancreas. The majority of the pancreas (p) lies anterior to the splenic vein (sv) and its junction with the superior mesenteric vein (SMV) forming the portal vein (pv). The head (H) and uncinate process (U) of the pancreas cradle the origin of the portal vein. The pancreatic neck (N) is anterior to the SV-SMV confluence, and the uncinate process and inferior vena cava (IVC) are posterior to the confluence. The superior mesenteric artery (SMA, arrow) arises from the aorta (Ao) dorsal to the splenic vein. The left renal vein (lrv) passes between the SMA and aorta to the inferior vena cava. The left lobe of the liver (L) offers a good sonographic window to the pancreas. The stomach (st) and lesser sac (collapsed) are anterior to the pancreas. CBD, common bile duct; S, spine; B, body of the pancreas; T, tail of the pancreas; p, pancreas. (Reprinted with permission from Brant WE, Helms C. Fundamentals of Diagnostic Radiology. 4th ed. Philadelphia, PA: Wolters Kluwer/Lippincott Williams & Wilkins Health; 2012.) |
 Figure 49.2 Arterial supply and venous drainage of pancreas. Because of the close relationship of the pancreas and duodenum, their blood vessels are the same in whole or in part. A, Arteries (anterior view). Except for the inferior part of the pancreatic head (including uncinate process), the spleen and pancreas receive blood from the celiac artery. B, Venous drainage (anterior view). C, Celiac (anteroposterior view). (Reprinted with permission from Moore KL, Dalley AF, Agur AMR. Clinically Oriented Anatomy. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2014.) |
EPIDEMIOLOGY AND ETIOLOGY
Incidence: A review of all documented cases in the United States from 1973 to 2004 suggests an incidence of 1.8 cases of pediatric pancreatic tumors per million children.
Has a female to male ratio of 1.9:1 and is more common in Asian ethnicities.
The strongest independent risk factors are stage, histology, and surgical intervention.1
Classification: The 3 most common pediatric pancreatic neoplasms are pancreatoblastoma, solid pseudopapillary neoplasms, and pancreatic endocrine tumors.
Tumors can arise from either exocrine or endocrine cells.
Pancreatoblastoma (ie, Frantz tumor) is the most common malignant pediatric pancreatic tumor, with both recurrence and metastasis.
Can be associated with Beckwith-Wiedemann and familial adenomatous polyposis; has a predicted 5-year survival of 66%.1
Solid pseudopapillary neoplasm is most common in 20-year-old women but less gender predominant in the pediatric groups.1
Has a 5-year predicted survival of 95% after complete resection, recurrence rate of 10%, and metastatic potential of 19.5% metastatic rate.2
Pancreatic endocrine tumor is most common in genetic syndromes such as multiple endocrine neoplasia 1/2 (MEN1/2), von Hippel-Lindau, neurofibromatosis 1, and tuberous sclerosis.1
Insulinomas have a 5-year predicted survival of 85% after surgical resection and 6% incidence of malignancy.
Higher rates of recurrence have been associated with syndromic tumors.2
Etiology
Pancreatoblastomas make up 30% to 50% of all pediatric pancreatic tumors.4
They are embryogenic in origin, considered to be the analogous tumor of hepatoblastoma to the liver and nephroblastoma to the kidney.
It has both epithelial and mesenchymal cellular makeup.5
Solid pseudopapillary neoplasms make up 1% to 3% of all pancreatic tumors.
They are not generally associated with genetic syndromes or tumor markers.
They are often thought to originate from embryonic stem cells or ovarian-related cells that migrate during embryogenesis.6
Pancreatic endocrine tumors: One-third of gastroenteropancreatic neuroendocrine tumors are located in the pancreas.
They can be associated with genetic syndromes such as MEN1/2, von Hippel-Lindau, and tuberous sclerosis.2
CLINICAL PRESENTATION
An otherwise healthy child presenting with incidental mass on imaging, palpable mass with abdominal pain, or hypoglycemia.
Pancreatoblastoma: incidental abdominal mass noticed in the first decade of life with a mean of 2.4 years.
Equal occurrence in tail and head of pancreas.
Recurrence and metastasis are more common than in other pancreatic tumors.2
Rarely occurs in children over the age of 10 years.
Congenital forms are almost always associated with Beckwith-Wiedemann syndrome.
They account for 30% to 50% of pediatric pancreatic neoplasms.2
Solid pseudopapillary neoplasm: most common in 20-year-old women, but less gender predominant in the pediatric group.
Generally a low-grade neoplasm. unlike adults, children are usually symptomatic with abdominal discomfort and pain.2
Pancreatic endocrine tumor: most commonly an insulinoma.
Generally, they are benign lesion with equal occurrence in head and tail of pancreas.
Children often present with severe postprandial hypoglycemia and may often be initially worked up for seizures.
Whipple triad is classic: fasting hypoglycemia, symptoms of hypoglycemia, relief of symptoms with glucose administration.2
Insulinomas (pancreatic endocrine tumor) must be differentiated from congenital hyperinsulinism.
On histopathology, insulinomas do NOT preserve the lobular architecture.7
DIAGNOSIS
Laboratory Findings
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