×

Please Remove Adblock
Adverts are the main source of Revenue for DoveMed. Please remove adblock to help us create the best medical content found on the Internet.

Primitive Neuroectodermal Tumor of Pancreas

Last updated Jan. 2, 2019

Approved by: Krish Tangella MD, MBA, FCAP

DoveMed.com

Primitive Neuroectodermal Tumor (PNET) of Pancreas is a rare, malignant, and life-threatening pancreatic tumor. It generally occurs in young individuals (mean age of 18 years). This is a Microscopic pathology image showing Primitive neuroectodermal tumor (PNET).


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

  • Pancreatic PNET
  • Pancreatic Primitive Neuroectodermal Tumor
  • PNET of Pancreas

What is Primitive Neuroectodermal Tumor of Pancreas? (Definition/Background Information)

  • Primitive Neuroectodermal Tumor (PNET) of Pancreas is a rare, malignant, and life-threatening pancreatic tumor. It generally occurs in young individuals (mean age of 18 years)
  • Small tumors and those in the early stages may not cause any signs and symptoms. As they grow larger, there may be jaundice, loss of appetite, and weight loss, due to this pancreatic tumor
  • Primitive Neuroectodermal Tumor of Pancreas can cause complications, such as metastasis to liver and lymph nodes, jaundice, diabetes (if sufficient numbers of islet cells of pancreas are destroyed), and compression of adjoining organs affecting their function
  • 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 Primitive Neuroectodermal Tumor of Pancreas depends upon many factors including the stage of the tumor and overall health of the individual; however, the prognosis of PNET of Pancreas is typically poor

The pancreas is an important organ of the digestive system.

  • Based on function of the pancreas, it can be functionally divided into 2 parts, namely:
    • Exocrine pancreas, which produces digestive enzymes, and
    • Endocrine pancreas that produces hormones such as insulin, glucagon, and somatostatin. Insulin and glucagon helps with controlling sugar levels in blood
  • 95% of the pancreas is exocrine portion and 5% is endocrine portion. Pancreatic tumors (both benign and malignant) can arise from both the exocrine and the endocrine part
  • Based upon 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 area of the pancreas more than the other areas.

Hence, localizing the tumor site can guide the healthcare provider to arrive at a probable diagnosis.

Who gets Primitive Neuroectodermal Tumor of Pancreas? (Age and Sex Distribution)

  • Primitive Neuroectodermal Tumor of Pancreas affects children and young adults in the age group of 6-25 years, with average age of presentation being 18 years
  • PNET is generally rare in pancreas and PNET of Pancreas constitute less than 1% of all pancreatic tumors
  • It can affect both males and females
  • The condition can occur worldwide; individuals of all racial and ethnic background may be affected

What are the Risk Factors for Primitive Neuroectodermal Tumor of Pancreas? (Predisposing Factors)

The specific risk factors for Primitive Neuroectodermal Tumor of Pancreas are unknown or unidentified. However, the general risk factors for pancreatic tumors/cancers include:

  • Advancing age: Pancreatic cancer may develop in individuals of all ages, but is seldom diagnosed in individuals younger than 55 years. An advancing age increases one’s risk for the condition
  • Gender: Generally, men are at a higher risk than women for developing pancreatic cancers
  • Smoking: It is one of the most important risk factor and heavy smokers are at the highest risk
  • Diabetes: Individuals with poorly-controlled and long-standing diabetes are at a greater risk
  • Family history: Pancreatic cancer in close blood-related family members is an important risk factor
  • Inflammation of the pancreas: Individuals with chronic pancreatitis (inflammation of the pancreas) for a long duration have an increased risk. It is important to note that alcohol consumption increases one’s susceptibility to pancreatitis
  • Overweight and obese individuals
  • Chronic exposure to various chemicals, especially due to one’s nature of occupation or residential location
  • Other factors include liver cirrhosis and gastrointestinal tract infections

The presence of certain genetic conditions can increase one’s risk for cancer of the pancreas, and these include:

  • Genetic syndromes increasing one’s risk for exocrine pancreatic cancers:
    • Hereditary breast and ovarian cancer syndromes
    • Familial atypical multiple mole melanoma syndrome
    • von Hippel-Lindau syndrome
    • Peutz-Jeghers syndrome
    • Lynch syndrome
    • Familial pancreatitis
  • Genetic syndromes increasing one’s risk for pancreatic neuroendocrine tumors:
    • Multiple endocrine neoplasia type 1 (MEN 1)
    • Neurofibromatosis type 1 (NF1)

Note: It is important to note that an individual diagnosed with cancer of the pancreas may not have any of the above-mentioned risk factors.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Primitive Neuroectodermal Tumor of Pancreas? (Etiology)

  • The exact cause of Primitive Neuroectodermal Tumor of Pancreas development is unknown
  • Research scientists believe that the cause of the condition is mostly due to genetic mutations, which results in tumor formation
  • In many Pancreatic PNETs, there is translocation of chromosomes 11 and 22 noted

What are the Signs and Symptoms of Primitive Neuroectodermal Tumor of Pancreas?

The signs and symptoms of Primitive Neuroectodermal Tumor of Pancreas depend upon the size and location of the tumor. During the initial stages, small tumors may not cause any signs and 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 signs and symptoms may include the following:

  • 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 PNET of Pancreas include:

  • This malignant tumor commonly affects the head of the pancreas
  • The tumors are single
  • The tumor size may range from 2-12 cm

How is Primitive Neuroectodermal Tumor of Pancreas 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 Primitive Neuroectodermal Tumor of Pancreas:

  • 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 cholangio-pancreatography (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 cholangio-pancreatography (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 they 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 Primitive Neuroectodermal Tumor of Pancreas?

The complications due to Primitive Neuroectodermal Tumor of Pancreas may include the following:

  • Gastrointestinal obstruction
  • Biliary tract obstruction causing jaundice
  • Compress adjoining organs if the tumor size is large, which may cause the organs to dysfunction or function abnormally
  • 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 Primitive Neuroectodermal Tumor of Pancreas Treated?

The treatment measures available for Primitive Neuroectodermal Tumor of Pancreas include the following:

  • 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 Primitive Neuroectodermal Tumor of Pancreas be Prevented?

Currently, there are no specific methods or guidelines to prevent Primitive Neuroectodermal Tumor of Pancreas. However, the condition may be avoided through the following measures:

  • Genetic testing in individuals with a family history of pancreatic tumors or other underlying genetic conditions associated with pancreatic tumors
  • Not smoking
  • Not being obese or overweight
  • Physical activity: A moderate amount of physical activity can help decrease the risk
  • A healthy diet, low in saturated fats and rich in many fruits and vegetables, can also help decrease one’s risk for pancreatic cancer
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations for those who have already endured the tumor

What is the Prognosis of Primitive Neuroectodermal Tumor of Pancreas? (Outcomes/Resolutions)

  • Primitive Neuroectodermal Tumor of Pancreas 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 respectability 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, the prognosis of PNET of Pancreas is poor and the 5 years survival rate is 50% after diagnosis of the condition
  • Regular follow up visits with the healthcare provider are important

Additional and Relevant Useful Information for Primitive Neuroectodermal Tumor of Pancreas:

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

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Nov. 27, 2015
Last updated: Jan. 2, 2019