Pancreatic Cancer in Children

Four types of pancreatic cancer are commonly found in children, which include:

1. Solid pseudopapillary tumor of the pancreas
   • The most common type of pancreatic tumor in children.
   • Commonly affects older adolescents and younger adult women.
   • A slow-growing tumor that has both solid and cyst-like parts. 
   • Unlikely to spread to other parts of the body, and the prognosis is excellent.
   • Rarely, the tumor may extend to the liver, lung, or lymph nodes.
2. Pancreatoblastoma
   • Usually occurs in children aged 10 years or younger. 
   • Risk factors:
     • Children with Beckwith-Wiedemann syndrome and familial adenomatous polyposis syndrome are at a higher risk.
   • This gradually developing tumor makes the following tumor markers:
     • Alpha-fetoprotein
     • Adrenocorticotropic hormone 
     • Antidiuretic hormone
   • May spread to the liver, lung, or lymph nodes. The prognosis in children is good.
3. Islet cell tumors
   • These benign or malignant tumors are uncommon in children.
   • In children, islet cell tumors may come with multiple endocrine neoplasia type I syndrome. 
   • The types of islet cell tumors include:
     • Insulinoma: Produces excess insulin
     • Gastrinoma: Makes excess gastrin
     • ACTHoma: Produces an excess of adrenocorticotropic hormone
     • VIPoma: Produces excess antidiuretic hormone
   • These tumors are also known as pancreatic neuroendocrine tumors.
4. Pancreatic carcinoma
   • It rarely occurs in children.
   • The two types of pancreatic carcinoma include:
     • Acinar cell carcinoma
     • Ductal adenocarcinoma

Signs and symptoms may vary based on the type of hormone produced by the tumor. However, the general signs and symptoms of childhood may include:

• Feeling exhausted
• Loss of appetite
• Weight loss for an unknown reason
• Lump in the abdomen
• Stomach discomfort

In some children, the tumor may not produce any hormone, showing no signs and symptoms. These tumors may be challenging to diagnose in the early stages.

Other pancreatic tumors that produce hormones cause signs and symptoms that vary depending on the type of hormone secreted.

Tumors secreting insulin may exhibit the following symptoms:

• Changes in behavior
• The following symptoms characterize low blood sugar:
  • Blurred vision
  • Headache
  • Feeling light-headed
  • Tiredness
  • Weakness
  • Feeling shaky
  • Nervousness
  • Irritability
  • Sweatiness
  • Feeling confused
  • Increased hunger

Tumors secreting adrenocorticotropic hormone or antidiuretic hormone may trigger the following signs and symptoms:

• Watery diarrhea
• Low sodium levels
• Dehydration
• Weight loss or gain for unknown reasons
• High blood pressure
• Purple or pink stretch marks on the skin
• Feeling extremely weak or tired
• Round face and thin arms or legs

If the tumor secretes gastrin, it may cause these signs and symptoms:

• Diarrhea
• Recurring stomach ulcers
• Abdominal pain, which radiates to the back 
• Gastroesophageal reflux

Other conditions can also cause similar signs and symptoms. Check with your child’s doctor if you observe the abovementioned symptoms.

The following procedures and tests may be used to diagnose pancreatic cancer:

• Physical examination: The physician assesses the body to check for any signs of lumps or other abnormalities. Your history, habits, and past illnesses and treatments will be noted.
• MRI: A procedure that uses a magnet, radio waves, and a computer to take detailed photos of specific areas of the body, such as the chest and abdomen. This is also known as nuclear magnetic resonance imaging.
• CT scan: This procedure takes detailed photos of the body from various angles and projects them in a series. A dye can be injected in a vein or swallowed to aid in better visualization of tissues and organs. Other names for CT scans include computed tomography, computerized tomography, or computerized axial tomography
• Positron emission tomography (PET) scan: This procedure detects malignant tumor cells within the body. A little amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body to take pictures of the areas where glucose has been used in the body. As malignant tumor cells are more active than normal cells and consume more glucose, they appear brighter in the image.
• Endoscopic ultrasound: An endoscope is inserted into the body through the mouth or rectum. The probe at the end of an endoscope produces high-energy sound waves or ultrasounds that bounce off from the internal tissues and organs to create echoes. These echoes form a picture of the body, commonly known as a sonogram.
• Biopsy: Involves removal of cells or tissues to examine them under a microscope to check for signs of cancer. The types of biopsies may include:
  • Core-needle biopsy
  • Laparoscopy
  • Laparotomy
• Somatostatin receptor scintigraphy: It is a special type of radionuclide scan that helps identify islet cell pancreatic tumors. It involves injecting a small amount of radioactive octreotide into the vein, which travels through the bloodstream to attach to the tumor. With the help of a special camera that detects radioactivity, the exact location of the tumor inside the pancreas can be identified.

Staging is the process of determining whether cancer has spread from the pancreas to other parts of the body. Currently, there is no standard staging system for childhood pancreatic cancer. Various tests and procedures done to diagnose pancreatic cancer are ideal to help make decisions about the treatment.

Pancreatic cancer in children can be treated through the following methods: 

• Surgery: Surgical removal of a benign or malignant tumor is the primary treatment option. 
• Radiation therapy: Uses high-energy rays to destroy cancerous cells. Depending on the type of radiation given, there are two types of radiation therapy:
  • External beam radiation (the radiation comes from a machine kept outside the body)
  • Internal radiation (in this, the doctor puts radioactive material inside the body)
• Chemotherapy: Aims to kill or destroy cancer cells or prevent their differentiation. Chemotherapy can be either taken orally or intravenously and the drug enters the bloodstream to reach the cancer cells.
• Targeted cancer therapy: A newer treatment option for treating oral and salivary gland cancer. It aims to prevent the mutations that cause the cells to grow uncontrollably or directly attack specific cancer cells. They are less harmful than chemotherapy or radiation therapy. Mammalian targets of rapamycin (mTOR) inhibitor drugs block the protein that helps the cancerous cell to divide and survive. mTOR inhibitors are mainly used to treat islet cell tumors.