What are the other Names for this Condition? (Also known as/Synonyms)

• Infantile Adenocarcinoma of Pancreas
• Infantile Pancreatic Adenocarcinoma

What is Pancreatoblastoma? (Definition/Background Information)

• Pancreatoblastoma is a rare, malignant, exocrine tumor of the pancreas. Even though it is a rare tumor. It is the most common pancreatic tumor of infancy and early childhood. The malignant tumor can occur in the head, body, or tail region of the pancreas
• Initially there may be no signs and symptoms due to the tumor. However, over the course of time, it may cause abdominal pain, nausea and vomiting, and lead to weight loss
• Pancreatoblastoma can cause complications, such as metastasis to liver and lymph nodes, and diabetes (if sufficient numbers of islet cells of pancreas are destroyed)
• In majority of cases, a complete surgical excision is the preferred mode of treating this malignant tumor. In cases of metastasis, a combination of chemotherapy, radiation therapy, and surgical procedures may be used
• The prognosis of Pancreatoblastoma depends upon many factors including the stage of the tumor and overall health of the individual. Nevertheless, the prognosis in children is better than the prognosis in adults

The pancreas is an important organ of the digestive system.

• Functionally, the pancreas can be divided into 2 parts, namely:
  • Exocrine pancreas, which produces digestive enzymes, and constitute about 95% of this important organ
  • Endocrine pancreas, which secretes hormones such as insulin, glucagon, gastrin, and somatostatin. Insulin and glucagon regulate sugar levels in blood

Pancreatic tumors (benign and malignant) can arise from both the exocrine and the endocrine components of the organ. Most endocrine tumors are benign and develop at a slower rate than exocrine tumors.

• Based on the anatomy of the pancreas, it can be divided into 3 main parts, namely the:
  • Head,
  • Body, and
  • Tail

Pancreatic tumors can affect the head, body, and tail region of the pancreas. Some tumors can affect one region of the pancreas more than the other. Hence, localizing the tumor site can guide the healthcare provider to arrive at a probable diagnosis.

Who gets Pancreatoblastoma? (Age and Sex Distribution)

• Pancreatoblastoma is a rare cancer of the pancreas that primarily affects newborns, infants, and young children, with the mean age of occurrence being 5 years 
• It can also occur during adulthood, between the ages 30-40 years, but it is extremely rare
• This type of cancer accounts for approximately 0.5% of exocrine tumors of the pancreas
• Pancreatoblastoma can affect both males and females and no specific gender bias is observed
• The cancer is seen worldwide; individuals of all racial and ethnic background may be affected

What are the Risk Factors for Pancreatoblastoma? (Predisposing Factors)

• The specific risk factors for Pancreatoblastoma are unknown or unidentified
• However, this type of tumor has been associated with Beckwith-Wiedemann syndrome in some newborns
• Individuals with familial adenomatous polyposis may also be at an increased risk of developing Pancreatoblastoma

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pancreatoblastoma? (Etiology)

The exact cause of Pancreatoblastoma development is unknown.

• Research scientists believe that the cause of the condition is mostly due to genetic mutations that leads to tumor formation
• It is theorized that the tumor begins to develop in the embryonic stages (embryonal tumor), whose cells originate from pancreatic stem cells
• The majority of Pancreatoblastoma tumors develop sporadically, and in such cases, chromosome 11 abnormalities, as well as disruption in the APC/beta-catenin pathway, have been reported
• In Beckwith-Wiedemann syndrome-associated Pancreatoblastoma, dysregulation of certain genes on chromosome 11 may be involved in tumor development
• When associated with familial polyposis syndrome (which increases an individual’s risk for colon cancer), mutation(s) in the tumor suppressor APC gene on chromosome 5, and aberrations in chromosome 11, may play a role in Pancreatoblastoma development

What are the Signs and Symptoms of Pancreatoblastoma?

The signs and symptoms of Pancreatoblastoma depend upon the size and location of the tumor. During the initial stages, small tumors may not cause symptoms that are readily recognized. Hence, these tumors are only detected incidentally, when being worked-up for other conditions (i.e., diagnostic tests and exams undertaken for other health conditions).

The following signs and symptoms may be noted:

• Failure to thrive (infants)
• Distended abdomen
• Abdominal pain, back pain
• Loss of appetite
• Weight loss
• Indigestion
• Yellowing of skin (jaundice)
• Nausea and vomiting
• Dark-colored urine
• Fatigue (getting tired easily)

General features of Pancreatoblastoma include:

• This malignant tumor occurs as a single tumor
• The tumor size may range from 2-20 cm (mean size of 10 cm); the tumor can grow to large sizes

How is Pancreatoblastoma Diagnosed?

Frequently, pancreatic tumors are difficult to detect/diagnose in the early stages. The signs and symptoms can be very similar to other conditions. Since the pancreas is located in the peritoneum, behind many organs, there is a lot of space for the tumor to grow (often unnoticed).

The following are the diagnostic methods that may be used to detect Pancreatoblastoma:

• A thorough physical examination and a complete medical history is very important
• Blood test to check for serum amylase levels
• Complete blood count with differential
• Blood tests that may involve tumors markers, such as:
  • Carcinoembryonic antigen (CEA)
  • CA 19.9
  • CA 15.3
  • Alpha fetoprotein
• Radiological studies that may include:
  • Abdominal ultrasound: A procedure where high-frequency sound waves are used to produce real-time images
  • Endoscopic ultrasound: It is a minimally-invasive procedure that uses high-frequency sound waves to obtain detailed images of the pancreas
  • CT scan of abdomen: It may be helpful in detecting recurrences, or if metastasis to other organs has occurred
  • MRI scan of abdomen: It helps produce high-quality pictures of certain body parts including the tissues, muscles, nerves, and bones
• Additionally, the following magnetic resonance imaging procedures may be used particularly in individuals suspected of having pancreatic tumor/cancer:
  • MR cholangiopancreatography (MRCP)
  • MR angiography (MRA)
• Positron emission tomography (PET) scan: It is particularly helpful in visualizing the spread of the cancer to other body parts (metastasis) and/or recurrence
• Percutaneous transhepatic cholangiography (PTC): A contrast material is injected into the bile ducts to visualize the structure
• Endoscopic retrograde cholangiopancreatography (ERCP): This technique is used when symptoms of pancreatic cancer are present in the individual. A special equipment and dye injections are used to obtain a series of images. If required, a biopsy sample may also be collected during the procedure
• Invasive diagnostic procedures such as:
  • Laparoscopy: A special device is inserted through a small hole into the abdomen, to visually examine it. If necessary, a tissue sample is obtained for further analysis. Exploration of the abdomen using a laparoscope is called ‘exploratory laparoscopy’
  • Laparotomy: The abdomen is opened through an incision for examination, and if required, a biopsy sample obtained. Exploration of the abdomen using laparotomy procedure is called ‘exploratory laparotomy’
• Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination to the pathologist, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

The tissue for diagnosis can be procured in multiple different ways, and these include:

• Fine needle aspiration (FNA) biopsy of the pancreatic tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
• Core biopsy of the pancreatic tumor
• Open biopsy of the pancreatic tumor

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pancreatoblastoma?

The complications due to Pancreatoblastoma may include the following:

• Gastrointestinal obstruction
• Biliary tract obstruction causing jaundice
• Compress adjoining organs if the tumor size is large, affecting their function
• Injury to local region
• If the tumor destroys enough islet cells of the pancreas, it can result in diabetes
• The tumor can metastasize to the liver and lymph nodes

How is Pancreatoblastoma Treated?

The following are the methods for treatment of Pancreatoblastoma, in both children and adults:

• In most cases, a surgical excision and removal of the entire tumor is the preferred treatment option. This may be followed by radiation therapy and/or chemotherapy
• If the tumor has metastasized, then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor
• Other treatment options may include:
  • Chemotherapy-radiotherapy: Where a combination therapy is used to treat the cancer
  • Targeted therapy: It used to target abnormal cancer cells and reduce the chance of cancer spreading to other parts of the body
  • Biological therapy: Also, known as immunotherapy, where certain bacteria or vaccines are used to indirectly treat the cancer
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important and encouraged

How can Pancreatoblastoma be Prevented?

• Currently, there are no specific methods or guidelines to prevent Pancreatoblastoma
• Children with a family history of Beckwith-Wiedemann syndrome or familial polyposis syndrome may benefit from increased monitoring for pancreatic tumors

What is the Prognosis of Pancreatoblastoma? (Outcomes/Resolutions)

• Pancreatoblastoma is a malignant tumor and its prognosis depends upon a set of several factors, which include:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • The surgical resectability of the tumor (meaning, if the tumor can be removed completely) 
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared to those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have a poorer prognosis compared to tumors that do not recur 
  • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond so well to treatment
• An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
• In general, it has been observed that children respond better to treatment than adults, and hence, the prognosis in them is slightly better than adults. 50% of the affected children are cured after a surgical removal of the tumor
• However, this type of pancreatic cancer is difficult to diagnose/treat and the prognosis is frequently poor. The average survival is 18 months after diagnosis in adults
• Regular follow up visits with the healthcare provider are important

Additional and Relevant Useful Information for Pancreatoblastoma:

It is estimated that the average lifetime risk of developing pancreatic cancer is about 1.5%.