Malignant tumors of the pancreas are rare in infants and children. In most patients, by the time the diagnosis is made, a mass can be felt, and yellowing of the skin and eyes is often present. Patients with malignant pancreatic tumors range in age from 3 months to 16 years with most patients younger than 10 years old.
The most common malignant tumors have been nonfunctioning islet cell carcinomas and adenocarcinomas. Most of the adenocarcinomas occur in the head of the pancreas, and the tumor may be found throughout the pancreas in occasional patients. Complete removal of the tumor with an adequate rim of normal pancreas is the only way to cure the patient, just like in adults. Removal of the head of the pancreas and the first part of the small intestine has provided the best long-term results, and smaller operations are best left for patients who require relief of symptoms but no hope for a cure. Spread is by lymph nodes and later to the liver and lungs.
Although the bigger surgery is the best chance for a cure for these patients, the patient may still die because the tumor is not picked up early enough. The results are better in patients with cancerous endocrine (hormone) tumors which can also be treated with radiation and chemotherapy besides just surgery. Long-term follow-up of children who have had the big surgery shows that these children are capable of long-term survival and achieving satisfactory growth and development. Enzyme replacement and insulin usually are required.
Patients with Zollinger-Ellison syndrome have severe ulcer disease, huge stomach juice production, and islet cell tumors of the pancreas. Huge stomach juice production is caused by far too much release of a hormone, gastrin, by the islet cell tumor. This may be shown when the tumor is stimulated by calcium or another hormone, secretin. A positive calcium test is diagnostic of a tumor called a gastrinoma. Diarrhea caused by huge gastric juice production occurs in more than a third of patients.
Multiple endocrine neoplasia syndrome is a disorder which runs in families. The patients have parathyroid, pituitary, and pancreatic tumors. The first sign of Zollinger-Ellison syndrome may be hyperparathyroidism with the later slow appearance of a pancreatic islet cell tumor.
If the decision is made to operate, the first part of the small intestine and pancreas should be explored thoroughly, and if a single tumor is found, it should be removed. After surgery, blood gastrin tests are drawn to see if the entire tumor was removed. If the tumor cannot be removed, if there are many tumors, or if no tumor is found, as occurs in half of patients, the recommended treatment is complete removal of the stomach.
The esophagus (the tube from the mouth to the stomach) is then connected to the intestine, preferably with a pouch. Most of the patients with this problem have been adults. 75% of patients with this syndrome treated by complete removal of the stomach are alive at 1 year, and 40% to 50% are alive at 10 years.
More than half of the patients have tumor spread to the liver at the time of their first operation. Even so, when tumor has spread, the 10-year survival is only slightly less. Although complete removal of the stomach does not cure the patient, it does provide the best overall results for the patient.
Chemotherapy has been reported to have some value in patients with tumor spread outside of the pancreas. These tumors are usually located in the head (right side) or tail (left side) of the pancreas. 30% of patients have two or more areas of tumor in the pancreas, and 20% of patients have tumor of the entire pancreas. Complete removal of the stomach seems to provide the best results in children, but when children younger than 7 years of age have this operation, a lot of added nutrition is required to make sure the child grows and develops normally. The use of stomach acid-blocking medication such as Zantac has been helpful when the tumor cannot be removed.
Ky A, Shilyanski J, Gerstie J, et al: Experience with papillary and solid epithelial neoplasms of the pancreas in children. J Pediatr Surg 33:42-44, 1998.
Malignant tumors of the pancreas are rare in children. This article stresses that long-term survival can be achieved with aggressive surgery.
Article and graphics adapted from O'Neill: Principles of Pediatric Surgery. © 2003, Elsevier.