Medical Information
Lymphoepithelial Cyst of Pancreas

Lymphoepithelial Cyst of Pancreas

Digestive Health
Diseases & Conditions
Contributed byKrish Tangella MD, MBAAug 22, 2023

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

  • LEC of Pancreas
  • Pancreatic LEC
  • Pancreatic Lymphoepithelial Cyst

What is Lymphoepithelial Cyst of Pancreas? (Definition/Background Information)

  • Lymphoepithelial Cyst (LEC) of Pancreas is a rare and benign cyst of the pancreas. It typically occurs in middle-aged and older men
  • Most tumors do not cause any signs and symptoms, but in some individuals, there may be abdominal pain, nausea, vomiting, and appetite loss
  • In many cases, these tumors are detected on radiological exams undertaken for some other health condition (an incidental finding). Generally, the tumors are well-circumscribed and occur in the head, body, or tail of the pancreas
  • Complications that arise from Lymphoepithelial Cyst of Pancreas are uncommon, but may include gastrointestinal and biliary tract obstruction (causing jaundice) and injury to local region
  • Even though no treatment may be necessary for Lymphoepithelial Cyst of Pancreas, surgery is an option for tumors that present severe symptoms or complications
  • The prognosis of Pancreatic Lymphoepithelial Cyst is excellent with appropriate treatment. However, it is advisable to maintain regular health checkups after treatment

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 Lymphoepithelial Cyst of Pancreas? (Age and Sex Distribution)

  • Lymphoepithelial Cyst of Pancreas is a rare, benign condition that affects adults in the age group of 35-80 years, with average age of presentation being 56 years
  • It constitute only about 0.5% of all pancreatic cysts
  • It can affect both males and females, though males are affected more (male-female ratio is 4:1)
  • The condition can occur worldwide; individuals of all racial and ethnic background may be affected

What are the Risk Factors for Lymphoepithelial Cyst of Pancreas? (Predisposing Factors)

The specific risk factors for Lymphoepithelial Cyst 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 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 Lymphoepithelial Cyst of Pancreas? (Etiology)

  • The exact cause of Lymphoepithelial Cyst of Pancreas development is presently unknown
  • Research scientists believe that the cause of the condition is mostly due to genetic mutations, which results in formation of the cysts

What are the Signs and Symptoms of Lymphoepithelial Cyst of Pancreas?

The signs and symptoms of Lymphoepithelial Cyst 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 Pancreatic Lymphoepithelial Cyst include:

  • This benign cyst can occur in the head, body, or tail of the pancreas
  • The tumors are single and well-circumscribed
  • The tumor size may range from 1-17 cm, with an average tumor size being 5 cm

How is Lymphoepithelial Cyst 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 Lymphoepithelial Cyst 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
    • A fine needle aspiration of the cyst has limited diagnostic value; however, the aspirated fluid can be analyzed for a variety of proteins such as CEA, CA 19.9, CA 15.3, alpha fetoprotein, and others. Such an evaluation will help in arriving at a diagnosis and classifying the type of cyst
  • 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 Lymphoepithelial Cyst of Pancreas?

There are no significant complications due to Lymphoepithelial Cyst of Pancreas, but a few complications may include:

  • 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 cyst destroys enough islet cells of the pancreas, it can result in diabetes

How is Lymphoepithelial Cyst of Pancreas Treated?

In majority of cases, no treatment is necessary for Lymphoepithelial Cyst of Pancreas, unless there are severe signs and symptoms.

  • If there are no signs and symptoms, then a “wait and watch” approach may be recommended by the healthcare provider, since it is a benign condition. A close follow-up and monitoring of the condition is important
  • If there are noticeable signs and symptoms, then removal of the tumor may be necessary and surgery is a treatment option. Under such circumstances, a complete surgical excision can be curative
  • Follow-up care with regular screening and checkups are important

How can Lymphoepithelial Cyst of Pancreas be Prevented?

Currently, there are no specific methods or guidelines to prevent Lymphoepithelial Cyst 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 Lymphoepithelial Cyst of Pancreas? (Outcomes/Resolutions)

  • The prognosis of Lymphoepithelial Cyst of Pancreas is excellent with appropriate treatment. Most of the signs and symptoms recede and disappear on removal of the tumor
  • However, regular follow up visits with the healthcare provider are important

Additional and Relevant Useful Information for Lymphoepithelial Cyst of Pancreas:

  • Lymphoepithelial Cyst of Pancreas is not associated with autoimmune disorders or immunosuppression
    • The cyst fluid has increased levels of carcinoembryonic antigen and CA 19.9. The cyst lining consists of squamous epithelial cells with surrounding lymphocytes
    • They are thought to occur from ectopic embryological tissue that occurs during fetal development
  • It is estimated that the average lifetime risk of developing pancreatic cancer is about 1.5%
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Krish Tangella MD, MBA

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