Pancreatic Cancer in Children

What Is Pancreatic Cancer in Children?

Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues of the pancreas. The pancreas is a pear-shaped gland about 6 inches long. The wide end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. Many different kinds of tumors can form in the pancreas. Some tumors are benign (not cancer).

The pancreas has two main jobs in the body:

  • Make juices that help digest (break down) food. These juices are secreted into the small intestine.
  • Make hormones that help control the sugar and salt levels in the blood. These hormones are secreted into the bloodstream.

What Types of Pancreatic Cancer Affect Children?

There are four types of pancreatic cancer in children:

  1. Solid pseudopapillary tumor of the pancreas. This is the most common type of pancreatic tumor. It most commonly affects females that are older adolescents and young adults. The tumors have both cyst -like and solid parts. Solid pseudopapillary tumor of the pancreas is unlikely to spread to other parts of the body and the prognosis is very good.
  2. Pancreatoblastoma. It usually occurs in children aged 10 years or younger. Children with Beckwith-Wiedemann syndrome and familial adenomatous polyposis (FAP) syndrome have an increased risk of developing pancreatoblastoma. These tumors may make adrenocorticotropic hormone (ACTH) and antidiuretic hormone (ADH). Pancreatoblastoma may spread to the liver, lungs, and lymph nodes. The prognosis for children with pancreatoblastoma is good.
  3. Islet cell tumors. These tumors are not common in children and can be benign or malignant. Islet cell tumors may occur in children with multiple endocrine neoplasia type 1 (MEN1) syndrome. The most common types of islet cell tumors are insulinomas and gastrinomas. These tumors may make hormones, such as insulin and gastrin, that cause signs and symptoms.
  4. Pancreatic carcinoma. Pancreatic carcinoma is very rare in children. The two types of pancreatic carcinoma are acinar cell carcinoma and ductal adenocarcinoma.

What Are the Signs and Symptoms of Pancreatic Cancer in Children?

In children, some pancreatic tumors do not secrete hormones and there are no signs and symptoms of disease. This makes it hard to diagnose pancreatic cancer early.

Pancreatic tumors that do secrete hormones may cause signs and symptoms. The signs and symptoms depend on the type of hormone being made.

If the tumor secretes insulin, signs and symptoms that may occur include the following:

  • Low blood sugar, which can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry
  • Changes in behavior
  • Seizures
  • Coma

If the tumor secretes gastrin, signs and symptoms that may occur include the following:

  • Stomach ulcers that keep coming back
  • Pain in the abdomen, which may spread to the back, may come and go, and go away after taking an antacid
  • The flow of stomach contents back into the esophagus (gastroesophageal reflux)
  • Diarrhea

Signs and symptoms caused by tumors that make other types of hormones may include the following:

If cancer is in the head of the pancreas, there may be blockage of the bile duct or blood flow to the stomach and the following signs may occur:

  • Jaundice (yellowing of the skin and whites of the eyes)
  • Blood in the stool or vomit

Check with your child’s doctor if you see any of these problems in your child. Other conditions that are not pancreatic cancer may cause these same signs and symptoms.


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What Tests Diagnose and Stage Pancreatic Cancer in Children?

Tests to diagnose and stage pancreatic cancer may include the following:

  • Physical exam and history
  • X-ray of the chest
  • CT scan
  • MRI
  • PET scan
  • Biopsy

Other tests used to diagnose pancreatic cancer include the following:

  • Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.
  • Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope (a thin, lighted tube) is passed through the mouth, esophagus, and stomach into the first part of the small intestine. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube, called a stent, may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs for cancer.
  • Somatostatin receptor scintigraphy: A type of radionuclide scan used to find pancreatic tumors. A very small amount of radioactive octreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is used to diagnose islet cell tumors.
  • Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.
  • Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.

What Is the Treatment for Pancreatic Cancer in Children?

Treatment of solid pseudopapillary tumor of the pancreas in children may include the following:

  • Surgery to remove the tumor
  • Chemotherapy for tumors that cannot be removed by surgery or have spread to other parts of the body

Treatment of pancreatoblastoma in children may include the following:

  • Surgery to remove the tumor (a Whipple procedure may be done for tumors in the head of the pancreas)
  • Chemotherapy may be given to shrink the tumor before surgery
  • More chemotherapy may be given after surgery for large tumors, tumors that cannot be removed by surgery, and tumors that have spread to other parts of the body
  • Chemotherapy may be given if the tumor does not respond to treatment or comes back

Treatment of islet cell tumors in children may include drugs to treat symptoms caused by hormones and the following:

  • Surgery to remove the tumor
  • Chemotherapy and targeted therapy for tumors that cannot be removed by surgery or that have spread to other parts of the body

Treatment of recurrent pancreatic carcinoma in children may include the following:

  • A clinical trial that checks a sample of the patient's tumor for certain gene changes
  • The type of targeted therapy that will be given to the patient depends on the type of gene change

For more information, read our full medical article on pancreatic cancer symptoms, signs, treatment, and prognosis.

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Last updated Oct. 6, 2017

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