Lymphoma is a group of more than 60 types of blood cancer. It develops in a type of white blood cell called lymphocytes. Lymphocytes are found throughout your lymphatic system in places like your lymph nodes, spleen, and bone marrow.

Primary pancreatic lymphoma is an extremely rare tumor of the pancreas. Fewer than 150 cases have been reported in English medical literature. “Primary” means that your pancreas is the first place where the cancer develops.

Pancreatic lymphoma can also spread to the pancreas from other parts of your lymph system. When this happens, it’s known as secondary cancer. Secondary pancreatic lymphoma is more common than primary.

Keep reading to learn more about pancreatic lymphoma including symptoms and how it’s diagnosed and treated.

Most lymphomas originate in your lymph nodes. When lymphoma involves a site other than your lymph nodes, it’s known as extranodal lymphoma.

Extranodal lymphoma makes up 30% to 40% of cases of non-Hodgkin’s lymphoma. It can develop in almost any organ but most commonly develops in the:

  • gastrointestinal tract
  • skin
  • bone
  • brain

Primary lymphoma of the pancreas

Primary lymphoma of the pancreas is rare, making up fewer than 0.1% of lymphomas and fewer than 0.5% of pancreatic tumors.

A limited number of studies suggest that it affects men 7 times more often than women and is most common among immunosuppressed people and elderly adults. It most typically develops in the head of the pancreas, the part that contains the most lymph tissue.

Primary pancreatic lymphoma tends to develop earlier than pancreatic adenocarcinoma, which makes up more than 90% of pancreatic cancers. One review of studies found that the average age that pancreatic lymphoma developed was 53.

Secondary lymphoma of the pancreas

Secondary pancreatic lymphoma is when lymphoma spreads to the pancreas from other parts of your lymph system.

Secondary pancreatic lymphoma is much more common than primary. It may occur in as many as 30% of cases of widespread extranodal lymphoma.

Types of pancreatic lymphomas

More than half of primary pancreatic cancers seem to be a type of non-Hodgkin’s lymphoma called diffuse large B cell lymphoma. Other subtypes that have been described in medical literature include:

Symptoms of pancreatic lymphoma typically resemble those of other pancreatic disorders. Typical symptoms include:

How to tell the difference between pancreatic lymphoma, other pancreatic cancers, and pancreatitis

Pancreatic lymphoma and other pancreatic cancers can cause similar symptoms. A biopsy is usually needed to confirm a diagnosis. In less than 2% of cases, pancreatic lymphoma can cause classic lymphoma symptoms such as:

Pancreatic lymphoma can cause acute pancreatitis as a symptom, but acute pancreatitis can form for many reasons that don’t involve cancer.

If you suspect you may have a problem with your pancreas, it’s important to see a doctor as soon as possible for an evaluation and diagnosis.

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Doctors use a combination of imaging and a biopsy to differentiate pancreatic lymphoma from other pancreatic conditions.

The World Health Organization provides the following diagnostic criteria for pancreatic lymphoma:

  • the bulk of the disease must be in the pancreas
  • although there may be nearby lymph nodes involved and distant spread, the primary signs and symptoms must involve the pancreas

Diagnosis begins with a physical exam and review of your medical history

A doctor will start a diagnosis by considering your medical and family history. They’ll also perform a physical exam and ask you about your symptoms.

They may order a blood test or other tests to rule out other conditions and evaluate your overall health.

Imaging scans can potentially find and identify tumors

Imaging scans can potentially identify a tumor in your pancreas. Ultrasound, endoscopic ultrasound, and computed tomography (CT) scans are potential options.

Finding a tumor that’s limited to the head of the pancreas can be suggestive of pancreatic lymphoma when:

  • it appears as an individualized tumor localized to the head of the pancreas
  • there isn’t significant dilation of pancreatic ducts
  • invasive tumor growth doesn’t follow anatomical boundaries
  • there’s no calcification or dead tissue within the tumor mass

Fine needle aspiration biopsy can confirm diagnosis

Doctors can confirm the pancreatic lymphoma diagnosis with a fine needle aspiration biopsy.

During this procedure, a fine needle is used to take a small tissue sample of the cancer. The procedure is performed endoscopically by guiding a long thin tube called an endoscope through your stomach and intestines until it’s near your pancreas.

Doctors can use a laboratory technique called flow cytometry to analyze cells from the tissue sample and to confirm the presence of lymphoma.

Due to the rarity of pancreatic lymphoma, no standard treatment has been established.

Generally, the same treatments are used for both primary and secondary lymphomas of the pancreas, but there are a variety of treatments that might be used.

A confirmed diagnosis is usually managed without surgery. Surgery is limited to when fine needle aspiration and flow cytometry don’t provide a diagnosis and the type of cancer isn’t clear.

Long-term remission can be achieved with just chemotherapy. The most common chemotherapy regimen includes the medications:

  • cyclophosphamide
  • doxorubicin
  • hydrochloride
  • vincristine
  • prednisone

Radiation therapy may also be used, but researchers continue to examine its role in treatment.

The outlook for both primary and secondary pancreatic lymphoma tends to be better than other cancers that develop in the pancreas, which have very poor outlooks.

Up to 30% of primary pancreatic lymphomas are cured. “Cured” usually means that there haven’t been any signs of the cancer for 5 years. By comparison, the relative 5-year survival rate for all pancreatic cancer is less than about 11%.

Extranodal lymphoma in general has fairly high reoccurrence rates, with the largest studies available reporting reoccurrence rates between 23.52% and 34.21%.

Primary pancreatic lymphoma is a very rare condition that occurs when lymphoma develops in the pancreas. Secondary pancreatic lymphoma is more common. It occurs when lymphoma spreads from another part of your body.

A diagnosis of pancreatic lymphoma begins by visiting a doctor. If they suspect lymphoma, they’ll send you for additional tests that will likely include imaging and a fine needle aspiration.

The outlook for pancreatic lymphoma tends to be better than other cancers that develop in the pancreas.