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Solid-Pseudopapillary Neoplasm of Pancreas

Last updated Dec. 25, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Solid-Pseudopapillary Neoplasm (SPN) of Pancreas is a type of exocrine tumor of the pancreas, with a low potential for malignancy.

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

  • Frantz Tumor
  • Gruber-Frantz Tumor
  • Solid Papillary Tumor (SPT) of Pancreas

What is Solid-Pseudopapillary Neoplasm of Pancreas? (Definition/Background Information)

  • Solid-Pseudopapillary Neoplasm (SPN) of Pancreas is a type of exocrine tumor of the pancreas, with a low potential for malignancy
  • These pancreatic tumors constitute about 1-2% of all exocrine tumors, and is almost exclusively observed in adolescent girls and women. The majority of these tumors occur in the head and tail region of the pancreas
  • The exact cause of formation of Solid-Pseudopapillary Neoplasm of Pancreas is not known. Owing to its occurrence predominantly in young women, it has been suggested that the female sex hormones may play a role in tumor formation. However, no direct evidence has been reported
  • The symptoms of SPN of Pancreas may be non-specific and include upper abdominal pain, abdominal discomfort, and fullness
  • The tumors may increase in size, and/or transform to a malignant tumor, leading to metastasis to the lymph nodes and other organs/tissues of the abdominal cavity
  • The preferred treatment of the tumor is a surgical excision. The prognosis of Solid-Pseudopapillary Neoplasm of Pancreas is generally good, despite its potential for malignancy

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.

Who gets Solid-Pseudopapillary Neoplasm of Pancreas? (Age and Sex Distribution)

  • Solid-Pseudopapillary Neoplasm of Pancreas constitutes less than 2% of all endocrine tumors
  • The tumor is primarily observed in women. When compared to men, women are 10 times more likely to get SPN of Pancreas
  • SPN of Pancreas is reported to occur between the ages of 2-85; however, young women are more vulnerable (mean age of diagnosis being 22 years)
  • The tumor may occur worldwide, although a study of 718 cases reported that most occurrences are observed in Japan, Europe, and North Africa
  • No racial or ethnic bias is observed in the occurrence of these types of tumor

What are the Risk Factors for Solid-Pseudopapillary Neoplasm of Pancreas? (Predisposing Factors)

The following factors may increase the risk of one being diagnosed with Solid-Pseudopapillary Neoplasm of Pancreas:

  • Being an adolescent girl or young woman in her twenties
  • A family history of pancreatic tumor or cancer 

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 Solid-Pseudopapillary Neoplasm of Pancreas? (Etiology)

The exact cause of Solid-Pseudopapillary Neoplasm of Pancreas is not known.

  • Since young women have a strong predilection for this pancreatic tumor, it has been proposed that the female sex hormones may play a role in tumorigenesis. The presence of estrogen and progesterone receptors in the tumors of some affected women forms the basis for this theory
  • A mutation in APC gene is reported in many SPN of Pancreas tumors
    • The protein coded by this gene associates with β-catenin to regulate how and when a cell divides, and how the cells interact/attach with each other
    • A mutation in this pathway may lead to uncontrolled proliferation of cells, leading to tumor formation

What are the Signs and Symptoms of Solid-Pseudopapillary Neoplasm of Pancreas?

The signs and symptoms of Solid-Pseudopapillary Neoplasm of Pancreas depend on a number of factors such as the following:

  • Size of the tumor
  • Local spread of the tumor
  • Extent of bleeding within the tumor

Individuals with Pancreatic Solid-Pseudopapillary Neoplasm may be asymptomatic, or present with the following symptoms:

  • Abdominal discomfort
  • Anorexia (loss of appetite)
  • Pain in the upper part of the abdomen or middle of the back
  • Persistent feeling of abdominal bloating with nausea or vomiting
  • Feeling full soon after eating less (having a feeling of satiety after eating less)
  • Changes in bowel movements, such as constipation
  • Fatigue, feeling tired easily
  • Depression

How is Solid-Pseudopapillary Neoplasm of Pancreas Diagnosed?

The following methods may be employed in the diagnosis of Solid-Pseudopapillary Neoplasm of Pancreas, and to rule-out any malignancy:

  • Physical examination and medical history evaluation: Diagnosing Solid-Pseudopapillary Neoplasm of Pancreas usually begins with a thorough physical examination and evaluation of complete medical history. During a physical exam, the overall health status and symptoms (such as pain, loss of appetite, and weight loss) of the affected individual are checked
  • Computerized tomography (CT) scan of the pancreas: With this radiological procedure, detailed three-dimensional images of structures inside the body are created. CT scans may be also helpful in detecting recurrences, or if SPN of Pancreas has metastasized to other organs
  • Magnetic resonance imaging (MRI) scan of the pancreas: An MRI scan uses magnetic fields that create high-quality pictures of certain body parts, such as tissues, muscles, nerves, and bones. These high-quality images may indicate to a physician if any pancreatic tumor is present
  • In addition, there are certain specific types of MRI scans that can be used in an individual who may have pancreatic cancer. Such radiological procedures include:
    • MR cholangiopancreatography (MRCP): It is a noninvasive test that uses a powerful magnetic field to produce images of soft tissues, bones, organs, and all other internal body structures
    • MR angiography (MRA): It is a noninvasive test that uses a powerful magnetic field to evaluate the blood vessels
  • Endoscopic ultrasound (EUS): This is a minimally invasive procedure recommended for individuals who are suspected to have pancreatic tumor. An ultrasound device is inserted through a thin tube (called endoscope) down the stomach and into a part of the small intestine. It uses high-frequency sound waves to generate detailed images of the pancreas
  • Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used when an individual has developed symptoms of pancreatic tumor. An ultrasound device is inserted through a thin tube (called endoscope) down the stomach and into the first part of the small intestine. A dye is injected into the pancreas and bile ducts. The movement of the dye is followed through a series of images. A small tissue sample (biopsy) can be collected during this procedure
  • Tissue biopsy: A tissue biopsy of the cyst (including the fluid inside the cyst) or mass is performed and sent to a laboratory for a pathological examination. A pathologist 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. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
    • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
    • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies, to assist in the 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
    • Endoscopic retrograde cholangiopancreatography
  • Positron emission tomography (PET): A PET scan is a nuclear medicine imaging technique that generates three-dimensional images to show how tissue and organs are functioning. A small amount of radioactive material may be injected into a vein, inhaled or swallowed. A PET scan is also helpful in detecting recurrences, or if any metastasis (to the surrounding lymph nodes of the pancreas) has occurred

A number of blood tests can also be helpful in the diagnosis of pancreatic cancer or to help determine the treatment options. These tests may include:

  • Liver function blood tests
  • Blood tests that may involve tumor markers, such as carcinoembryonic antigen (CEA) and CA 19.9

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 Solid-Pseudopapillary Neoplasm of Pancreas?

The potential complications of Solid-Pseudopapillary Neoplasm of Pancreas may occur as the tumor progresses, and these may include:

  • Jaundice owing to biliary tract obstruction (rare)
  • Abdominal pain due to tumor growth pressing on the nerves in the abdomen
  • Gastrointestinal obstruction
  • Diabetes, if the tumor destroys enough islet cells of the pancreas
  • Weight loss
  • Transition to malignant tumor
  • Metastases to the liver and lymph nodes
  • Side effects from the chemotherapy (such as toxicity) and radiation therapy

How is Solid-Pseudopapillary Neoplasm of Pancreas Treated?

The treatment methods Solid-Pseudopapillary Neoplasm of Pancreas are determined by several factors, such as the size and location of tumor, how advanced the condition is, the overall health of the affected individual, as well as his/her personal preference(s). The healthcare provider typically determines and plans the best course of treatment on a case-by-case basis.

  • The treatment for choice for Pancreatic Solid-Pseudopapillary Neoplasm is a complete removal of the lesion
  • For tumors that are noted to be malignant, a combination therapy that includes radiation therapy and chemotherapy may be additionally necessary
  • Post-operative care is important: A minimal physical activity is advised, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important (to monitor the status of the tumor)

How can Solid-Pseudopapillary Neoplasm of Pancreas be Prevented?

Presently, no specific methods or guidelines exist for the prevention of Solid-Pseudopapillary Neoplasm of Pancreas.

  • If there is a family history of pancreatic cancer, regular medical screening at periodic intervals may be helpful in diagnosing Solid-Pseudopapillary Neoplasm of Pancreas during the initial stages 
  • In general, modifications in certain lifestyle/behavioral choices may help decrease the risk of many types of pancreatic cancer. These measures include
    • Smoking cessation
    • Regular physical activity
    • Inclusion of fruits and vegetables in the diet
  • Due to the metastasizing potential of SPN of Pancreas, regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations are often needed, once an individual is diagnosed with the cancer

What is the Prognosis of Solid-Pseudopapillary Neoplasm of Pancreas? (Outcomes/Resolutions)

  • The prognosis is generally good for Solid-Pseudopapillary Neoplasm of Pancreas that are in early stages of development and are benign
  • However, it is possible for a lesion to progress to malignancy. Even with metastasis, the outcome is better than some other forms of pancreatic cancers (such as pancreatic adenocarcinoma), with long-term survival reported in many cases
  • If an individual is diagnosed with SPN of Pancreas (with or without malignancy), regular follow-up is necessary

Additional and Relevant Useful Information for Solid-Pseudopapillary Neoplasm of Pancreas:

  • It is estimated that the average lifetime risk of developing pancreatic cancer is about 1 in 67 (1.5%)
  • Individuals can reduce their risk of developing pancreatic cancer through lifestyle or behavioral changes

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: Sept. 3, 2017
Last updated: Dec. 25, 2018