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

• Pancreatic Lymphangioma

What is Lymphangioma of Pancreas? (Definition/Background Information)

• Lymphangioma of Pancreas is a rare, benign tumor of the pancreas that develops spontaneously, due to certain abnormalities in the lymphatic system
• It typically occurs in newborns and very young children; the tumor may be congenital in nature. The tumor forms when there is an obstruction in the lymphatic system that occurs due to various reasons
• Most tumors do not cause any signs and symptoms, but in some individuals, there is the presence of an abdominal mass, indigestion, and loss of appetite, due to the lymphangioma
• Complications that arise from Lymphangioma of Pancreas are rare, but may include injury to local region, complications from invasive procedures, and recurrence after treatment
• Lymphangioma of Pancreas may be treated using steroids or injections, while surgery is an option for tumors that present more severe symptoms or complications
• The prognosis of Pancreatic Lymphangioma is good with appropriate treatment, though it depends upon the lack of any significant complication (before or after delivery of the child)

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 Lymphangioma of Pancreas? (Age and Sex Distribution)

• Lymphangioma of Pancreas can occur at any age, though they are frequently seen in newborns and very young children. A lymphangioma usually forms during the fetal stage or before the age of 2 years
• 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 Lymphangioma of Pancreas? (Predisposing Factors)

The specific risk factors for Lymphangioma of Pancreas may include the following:

• Genetic disorders, such as Turner syndrome, Down syndrome, and Noonan syndrome, are associated with Lymphangiomas
• Genetic disorders, such as Patau’s syndrome (trisomy 13), Edwards syndrome (trisomy 18), and Down syndrome (trisomy 21), can increase the risk
• Alcohol consumption in excess
• Viral infection during pregnancy

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 Lymphangioma of Pancreas? (Etiology)

• The exact cause of Lymphangioma of Pancreas development is unknown
• It may occur when lymphatic channels are blocked and get enlarged during the fetal stage. This obstruction may occur due to genetic and developmental abnormalities

What are the Signs and Symptoms of Lymphangioma of Pancreas?

The signs and symptoms of Lymphangioma 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 due to abdominal mass, back pain
• Loss of appetite
• Weight loss
• Indigestion
• Yellowing of skin (jaundice)
• Nausea and vomiting
• Dark-colored urine
• Fatigue (getting tired easily)

How is Lymphangioma 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 Lymphangioma 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’
• Amniocentesis or chorionic villus sampling may be performed during pregnancy
• 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 Lymphangioma of Pancreas?

The complications due to Lymphangioma of Pancreas may include the following:

• Gastrointestinal or 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
• Injury or infection during surgery to remove the tumor
• Recurrence after treatment (due to incomplete removal of the lymphangioma)
• Complications in the fetal stage or during pregnancy

How is Lymphangioma of Pancreas Treated?

The following treatment measures may be considered for Lymphangioma of Pancreas.

• If there are no signs and symptoms, then a “wait and watch” approach with close monitor of tumor growth may be recommended, since it is a benign condition
• Surgical removal of the tissue mass may be performed in stages and is the most effective method for a complete cure
• Combination treatment using chemotherapy and radiation therapy, if the tumor is recurrent, or if surgical treatment is not possible
• Use of steroids and sclerosing injections into the tumor
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important, to watch for any recurrences

How can Lymphangioma of Pancreas be Prevented?

Currently, there are no specific methods or guidelines to prevent Lymphangioma 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
• Regular medical screening during pregnancy
• Regular medical screening at periodic intervals for those who have already endured the condition

What is the Prognosis of Lymphangioma of Pancreas? (Outcomes/Resolutions)

• The prognosis of Lymphangioma of Pancreas is excellent with appropriate treatment, if no additional complications develop. Some tumors are known to resolve spontaneously
• It may be difficult to predict the outcome, if there are associated genetic syndromes and the lymphangioma develops during early pregnancy stage
• If lymphangiomas develop during late pregnancy stages, then the outcome is generally favorable with proper treatment
• However, regular follow up visits with the healthcare provider are important

Additional and Relevant Useful Information for Lymphangioma of Pancreas:

• It is estimated that the average lifetime risk of developing pancreatic cancer is about 1.5%
• There are two types of lymphangioma tumors:
  • Cystic lymphangioma: Occurs around the head, neck, and groin region
  • Cavernous lymphangioma: Occurs around the head, neck, and groin region, and additionally, are found inside the mouth, torso, limbs, and abdomen (such as in the pancreas)