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

• Hepatoid Carcinoma of Pancreas
• Hepatoid Variant of Pancreatic Cancer

What is Pancreatic Hepatoid Carcinoma? (Definition/Background Information)

• Pancreatic Hepatoid Carcinoma is a rare, malignant tumor the pancreas. On histological examination of the tumor (under the microscope), it appears like liver tissues; hence the name ‘hepatoid’ is associated with this carcinoma
• These aggressive tumors resemble hepatocellular carcinoma (HCC). Hepatoid carcinomas may originate at other locations too, such as the gastrointestinal tract, ovary, and lungs
• Generally, middle-aged and older adults are affected by Pancreatic Hepatoid Carcinoma, and there is a slight male predilection. There may be no signs and symptoms due to the tumor, during the early stages. However, over the course of time, it may cause abdominal pain, nausea and vomiting, and lead to weight loss
• Pancreatic Hepatoid Carcinoma can cause complications, such as metastasis to liver and lymph nodes (observed in many cases) and diabetes, if sufficient numbers of islet cells of pancreas are destroyed
• 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 Pancreatic Hepatoid Carcinoma depends upon many factors including the stage of the tumor and overall health of the individual. Nevertheless, the prognosis is 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 Pancreatic Hepatoid Carcinoma? (Age and Sex Distribution)

• Pancreatic Hepatoid Carcinoma is a rare cancer of the pancreas that affects adults; average age at diagnosis is 53 years
• It can affect both males and females, though males are affected slightly more (male-female ratio is 3:2)
• The condition can occur worldwide; individuals of all racial and ethnic background may be affected

What are the Risk Factors for Pancreatic Hepatoid Carcinoma? (Predisposing Factors)

The specific risk factors for Pancreatic Hepatoid Carcinoma 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
• There is no association of Hepatoid Carcinoma of Pancreas with hepatitis B virus (HBV) infection and hepatitis C virus (HCV) infection

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 Pancreatic Hepatoid Carcinoma? (Etiology)

The exact cause of Pancreatic Hepatoid Carcinoma development is unknown.

• Some research scientists believe that it is a carcinoma arising from ectopic (abnormally-placed) liver tissue in the pancreas
• While other researchers believe that it is the tumor cells which transform into liver-like cells

What are the Signs and Symptoms of Pancreatic Hepatoid Carcinoma?

The signs and symptoms of Pancreatic Hepatoid Carcinoma 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 following signs and symptoms may be noted, which include:

• 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 Hepatoid Carcinoma include:

• This malignant tumor occurs as a single tumor
• It arises from the exocrine pancreas and can occur in the head, body, or tail of the pancreas

How is Pancreatic Hepatoid Carcinoma 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 Pancreatic Hepatoid Carcinoma:

• 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: Serum levels of alpha fetoprotein may be increased
• 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

Note:

• Pancreatic Hepatoid Carcinoma can be mixed with other tumor cells, such as adenocarcinoma of pancreatic ducts, neuroendocrine carcinoma, and acinar carcinoma. Hence, extensive tumor sampling is important during diagnosis
• These tumors have similar immunohistochemical (IHC) staining pattern as hepatocellular carcinoma

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 Pancreatic Hepatoid Carcinoma?

The complications due to Pancreatic Hepatoid Carcinoma may include the following:

• Gastrointestinal and biliary tract obstruction (causing jaundice)
• Compress adjoining organs if the tumor size is large, affecting their function
• 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, which is often observed in individuals during the time of diagnosis

How is Pancreatic Hepatoid Carcinoma Treated?

The treatment of Pancreatic Hepatoid Carcinoma may be undertaken as:

• In most cases, a surgical excision and removal (surgical resection) 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 Pancreatic Hepatoid Carcinoma be Prevented?

Currently, there are no specific methods or guidelines to prevent Pancreatic Hepatoid Carcinoma. 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 Pancreatic Hepatoid Carcinoma? (Outcomes/Resolutions)

• Pancreatic Hepatoid Carcinoma 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 resectability 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
• However, this pancreatic cancer type is aggressive and difficult to diagnose/treat. Also, in many cases, metastasis would have already taken place to various organs, at the time of diagnosis. Hence, the prognosis is frequently poor
• Regular follow up visits with the healthcare provider are important

Additional and Relevant Useful Information for Pancreatic Hepatoid Carcinoma:

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