Glucagonoma of Pancreas

Glucagonoma of Pancreas

Article
Digestive Health
Diseases & Conditions
+2
Contributed byKrish Tangella MD, MBASep 17, 2018

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

  • Alpha Cell Tumor of Pancreas
  • Pancreatic Alpha Cell Tumor
  • Pancreatic Glucagonoma

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

  • Glucagonoma of Pancreas is a malignant tumor that is caused by an increase in glucagon hormone produced by alpha cells of the pancreas. This leads to a condition known as glucagonoma syndrome
  • These pancreatic tumors generally occur in middle-aged adults, especially in women. Glucagonoma syndrome can cause a skin condition called superficial migratory necrotic erythema, which can lead to blister formation and ulceration
  • These malignant tumors may infiltrate into local tissue or even metastasize (to the liver). Also, additional complications may include diabetes, blindness, and deep vein thrombosis
  • There are various treatment modalities available to address the condition such as zinc replacement therapy and treatment with octreotide. However, a complete surgical excision of the tumor is the preferred mode of treatment
  • The prognosis of Glucagonoma of Pancreas depends upon many factors including the tumor stage and overall health of the individual. If the tumor is only present in the pancreas, then the 5-year survival rate is 85%

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 Glucagonoma of Pancreas? (Age and Sex Distribution)

  • Pancreatic Glucagonoma is an infrequently occurring tumor that typically affects adults. It can occur at any age, but is frequently seen in individuals over the age of 40 years
  • It can affect both males and females, though a preference for females is observed
  • It is seen to occur worldwide and no racial or ethnic preference is observed

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

The specific risk factors for Glucagonoma of Pancreas are generally unknown or unidentified.

  • Multiple endocrine neoplasia type 1 (MEN 1, an inherited genetic disorder)
  • A family history of Glucagonoma or other pancreatic tumors

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 Glucagonoma of Pancreas? (Etiology)

  • The exact cause of Glucagonoma of Pancreas development is unknown
  • Research scientists believe that the cause of the condition is mostly due to abnormal molecular or genetic mutations

What are the Signs and Symptoms of Glucagonoma of Pancreas?

The signs and symptoms of Glucagonoma of Pancreas depend upon the size and location of the tumor (within the pancreas). 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).

Glucagonoma of Pancreas can cause glucagonoma syndrome with the following signs and symptoms:

  • Skin condition called superficial migratory necrotic erythema (MNE), which is a characteristic sign of the syndrome
  • Skin nodules that may ulcerate and bleed
  • Ulcers on the fingertips

Additionally, the signs and symptoms may also include the following:

  • Abdominal pain, back pain
  • Increased thirst and hunger
  • Frequent urination
  • Diarrhea
  • Increased tendency to form blood clots in legs
  • Depression
  • Fatigue (getting tired easily)
  • Weight loss and malnutrition
  • Signs and symptoms of any underlying disorder, such as multiple endocrine neoplasia type 1

General features of Pancreatic Glucagonoma include:

  • The tumors may be small and are sometimes are missed, because of their small size
  • They can arise from the endocrine portion of the pancreas
  • The tumors are generally single, while sometimes multiple tumors are observed

How is Glucagonoma 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 Glucagonoma of Pancreas and the associated glucagonoma syndrome:

  • A thorough physical examination and a complete medical history is very important
  • The examination of the skin by a dermatologist using a special magnified lens (dermoscopy) may be undertaken
  • Blood test may show decreased protein, serum albumin, and globulin proteins levels
  • Lab results may show increased fasting plasma glucagon and blood glucose. They can also show decreased serum zinc levels
  • Pancreatic arteriography to detect small tumors
  • 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
  • Glucagon level testing using selective transhepatic portal venous sampling. In this test, the glucagon levels from various parts of the portal system is measured to localize the tumor
  • 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
  • Skin biopsy

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

Other tests to diagnose Glucagonoma of Pancreas may include the following:

  • 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
  • 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’

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 Glucagonoma of Pancreas?

The complications due to Glucagonoma of Pancreas (that causes glucagonoma syndrome) may include the following:

  • Gastrointestinal and biliary tract obstruction (causing jaundice)
  • Compress adjoining organs if the tumor size is large, which may cause the organs to dysfunction or function abnormally
  • Vision loss
  • Deep vein thrombosis (DVT)
  • If the tumor destroys enough islet cells of the pancreas, it can result in diabetes
  • Cosmetic issues due to the skin being affected severely by nodules and blisters
  • Malignant tumors can cause local invasion or distant metastasis (usual site is the liver)
  • Complications may arise due to the presence of the underlying genetic disorder (MEN type 1)

How is Glucagonoma of Pancreas Treated?

The treatment of Glucagonoma of Pancreas depends upon a set of factors that include the following:

  • Stage of the tumor (how far the tumor has spread from the pancreas)
  • Whether it is a single tumor, or there are multiple tumors
  • Whether the tumor is associated with a syndrome (such as MEN 1)
  • Overall health status of the individual
  • Location of the tumor in the pancreas (head, body, or tail)
  • Size of the tumor

The treatment measures available for Glucagonoma of Pancreas include the following:

  • In most cases, a surgical excision and removal of the entire tumor is the preferred treatment option. In some cases, a portion of the pancreas may have to be removed
  • If the tumor has metastasized, then a combination of radiation therapy and invasive procedures may be used to treat the tumor. This is often based upon the individual’s specific circumstance. Chemotherapy is generally found to be ineffective against this tumor
  • Total parenteral nutrition (TPN) for malnutrition
  • Some studies have shown zinc replacement therapy to be beneficial
  • Treatment with octreotide can help in decreasing the adverse effect of glucagon secretion by inhibiting its release from the pancreas
  • Superficial migratory necrotic erythema, which is caused by the glucagonoma tumor, is often difficult to treat
  • Other treatment options may include:
    • Radiofrequency ablation of the tumor
    • Cryotherapy
    • Selective internal radiotherapy
    • Transarterial chemoembolization (TACE)
  • Treatment of the underlying MEN type 1 (if present)
  • 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 Glucagonoma of Pancreas be Prevented?

Currently, there are no specific methods or guidelines to prevent Glucagonoma 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, such as MEN type 1
  • 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 Glucagonoma of Pancreas? (Outcomes/Resolutions)

  • Glucagonoma 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 malignant tumor generally yields better outcomes than a late diagnosis and delayed treatment
  • Generally, a surgery to remove the pancreas can result in a better prognosis if the tumor is confined to the pancreas. In such a scenario, it may also reduce the severity of the signs and symptoms (such as the skin condition)
  • In case of underlying genetic syndromes, such as multiple endocrine neoplasia 1, the prognosis is further dependent upon the severity of the syndrome
  • Regular follow up visits with the healthcare provider are important

Additional and Relevant Useful Information for Glucagonoma of Pancreas:

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

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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