Malignant tumours of the pancreas are referred to as pancreatic carcinomas.

Tumours of the pancreas account for 2-3% of all malignant neoplasms in adults. In Germany and Europe, 10-12 males and 7-9 females per 100,000 inhabitants are diagnosed with this tumour each year. In recent years, cancers of the pancreas (pancreatic carcinomas) have steadily increased.

The tumours are often only discovered at an advanced stage. Complete surgical resection (operation) is currently the only chance of a cure. Due to the often late diagnosis and aggressive course of the disease, this cancer is the fifth most common cause of tumour-related death worldwide, despite its relatively low incidence.

The peak age is between 60 and 75 years. Around 80% of tumours are found in the head, 20% in the body and 10% in the tail of the pancreas. Around 80-90% of pancreatic tumours are glandular carcinomas that resemble the ductal structures of the normal pancreas in appearance (ductal adenocarcinoma). The other tumours are rather rare. These include endocrine tumours or acinar cell carcinomas.

The causes of pancreatic cancer are still largely unknown. Of the external influences, only cigarette smoking has been proven to be a definite risk factor. A low-fibre, high-calorie and high-fat diet is also being discussed as a possible risk factor.

Around one in 20 patients with pancreatic cancer has another first-degree family member with this tumour. This is referred to as familial pancreatic cancer. It is not yet clear to what extent shared lifestyle habits or hereditary predispositions are responsible. In a few families, special features in the genetic information (gene mutations) have been identified that are associated with a hereditary predisposition to the development of malignant tumours. These include families in which not only pancreatic cancer but also black skin cancer (malignant melanoma) occurs, the so-called pancreatic carcinoma-melanoma syndrome, and other rare hereditary diseases. These families are very rare.

Furthermore, families with the very rare form of hereditary chronic inflammation of the pancreas (hereditary pancreatitis) have a significantly increased risk of developing pancreatic cancer up to the age of 70. Some studies indicate that patients with non-hereditary forms of chronic pancreatitis (e.g. chronic alcohol abuse) also have an increased risk of developing pancreatic cancer.

Unfortunately, the symptoms of pancreatic cancer are often uncharacteristic. Patients report symptoms such as upper abdominal pain, fatigue, weight loss, fever and back pain. More specific symptoms are jaundice (icterus), a new onset of diabetes or inflammation of the pancreas. Pancreatic carcinoma should always be considered, particularly in patients >60 years of age with new back pain or diabetes, in order to avoid delays in diagnosis.

If cancer of the pancreas is suspected, a number of examinations are required to make a diagnosis, which are briefly described below.

Medical history and physical examination

During a detailed consultation, you will tell the doctor about all your symptoms and previous illnesses. A thorough physical examination will then be carried out.

Laboratory tests

A series of blood tests will be carried out on you to provide information about your general condition and certain organ functions. Changes in the blood such as anaemia, changes in blood proteins, increased calcium levels, increased levels of liver and pancreatic enzymes or an increased drop in blood cell counts may be an expression of the tumour disease. Elevated liver values may indicate that the tumour is narrowing the bile duct and preventing bile from flowing into the small intestine. The tumour markers known for pancreatic carcinoma are not specific and are only of significance in the follow-up after a possible operation.

Transabdominal ultrasound (sonography)

Due to its inexpensive availability and the absence of side effects or radiation exposure, sonography is now the first and most important examination for suspected pancreatic cancer. The assessment is often complicated by the location of the organ and frequent air overlays, so that other imaging procedures must be used. An important advantage of sonography is the ability to assess the spread of the tumour to other organs or to detect existing metastases (tumour metastases), e.g. in the liver. Sonography can also detect whether a pancreatic tumour is obstructing the bile duct and causing a build-up of bile that needs to be treated quickly.

Endosonography (EUS)

Endosonography involves an endoscopy of the stomach/small intestine using a device with an ultrasound probe at the tip. This ultrasound probe can be guided directly to the pancreas in the stomach or small intestine so that even small tumours can be detected. Endosonography also allows a precise assessment of local tumour spread, e.g. to neighbouring organs or lymph nodes.

Fine needle biopsy

If it was not possible to make a clear diagnosis based on the imaging procedures, your doctor may have arranged for a so-called fine needle biopsy of the tumour in the pancreas or of metastases. In this procedure, a fine needle is inserted into the tumour under ultrasound guidance, either through the abdominal wall or via the stomach wall during an endosonography, in order to obtain cell material. This cell material is then examined by a specialist (pathologist/cytologist) under a microscope using various special stains. The accuracy of the imaging procedure can be significantly increased by an additional fine needle puncture.

Computer tomography (CT)

Computed tomography is performed if sonography and/or endosonography were not conclusive, or for surgical planning.

Computed tomography is a painless X-ray examination in which a contrast medium is administered intravenously. The relevant section of the body is examined layer by layer so that the location and size of the tumour can be precisely determined. The exact spread of the tumour and any existing metastases, e.g. in the liver, lungs or lymph nodes, can be determined in this way

Magnetic resonance imaging (MRI)

You may also have undergone magnetic resonance imaging (MRI). MRI is not an X-ray examination, but is based on the effects of magnetic fields. This examination is carried out in exceptional cases, e.g. in the case of contrast agent intolerance or impaired kidney function, sometimes in addition to computer tomography. The procedure is comparable to computed tomography in terms of assessing the tumour stage and operability. In the diagnosis of pancreatic carcinoma, special examination techniques can also be used to image both the pancreatic duct and the bile duct (MRCP = magnetic resonance cholangiopancreatography).

Endoscopic retrograde cholangiopancreatography (ERCP)

In ERCP, an endoscopy is performed using a flexible endoscope, which is passed through the mouth to the point where the bile duct and pancreatic duct open into the small intestine. There, various instruments such as catheters can be inserted into both ducts. After administering a contrast agent, both ducts are visualised, allowing the detection of tumour-related constrictions. By using special instruments such as forceps or brushes, cell and tissue material can be obtained from the tumour, which is then examined by the pathologist to make a diagnosis. In addition, ERCP offers the possibility of treating constrictions in the bile duct during the same examination by inserting plastic or metal mesh tubes to allow the bile to drain into the small intestine (a more detailed description can be found below in the section on therapy).

Laparoscopy (laparoscopy)

In exceptional cases, a laparoscopy is performed before a planned operation. Under anaesthetic, various instruments are inserted into the abdominal cavity via the abdominal wall. After filling the abdominal cavity with a gas, the abdominal organs can be assessed directly and larger tissue samples can be taken. This is particularly important if metastases in the liver are to be reliably ruled out.

Tumour markers

Tumour markers are molecules derived from tumour cells that can be detected in the blood and thus provide an indication of the tumour disease. The marker CA 19-9 is most commonly used for pancreatic carcinoma.

The problem with these markers is their low sensitivity and limited accuracy, meaning that an increase is not suitable for making a diagnosis. For these reasons, this marker is primarily used in follow-up examinations after an operation in order to be able to detect a recurrence of the tumour on the basis of an increase.

In order to determine the most suitable therapy, the exact extent to which the tumour has spread, i.e. the tumour stage, must be determined using the diagnostics described above before starting therapy. The TNM classification is used for this purpose (see table below). T stands for the size and extent of the primary tumour, N stands for the number of affected lymph nodes and M stands for the occurrence and localisation of distant metastases (tumour metastases).

These TNM criteria are used to classify the tumour disease into different stages, which are of decisive importance for determining the therapy. Stage I represents the least spread of the tumour, stage IV stands for an advanced tumour with distant metastases. The exact staging based on the TNM criteria is shown in the table below.

Furthermore, it is of decisive importance after an operation whether the tumour has been completely removed. The success of the operation is described as follows:

As mentioned above, the treatment options for pancreatic carcinoma depend on the stage of the tumour. In general, the earlier the pancreatic carcinoma is detected, the more favourable the treatment options. Unfortunately, pancreatic carcinoma is diagnosed very late due to its unspecific symptoms, which means that curative therapy is rarely possible. The patient's age and general state of health also play a decisive role in the choice of treatment.

The available therapies are surgery, radiotherapy, chemotherapy and endoscopic procedures.

Surgical therapy

Curative (healing) surgery

Complete surgical removal of the tumour is the only way to cure the tumour. The tumour must be confined to the pancreas and there must be no metastases. Depending on the location of the tumour, either the head, body or tail, or several parts of the pancreas are removed. In addition, the associated lymphatic drainage areas are removed and, if the tumour is located in the head of the pancreas, adjacent parts of the duodenum, bile duct and possibly the stomach are removed. The connection between the stomach and bile ducts with the lower sections of the intestine is reconstructed using loops of small intestine. Your surgeon will discuss the details of the operation with you in detail, taking into account the individual spread of the tumour.

This type of operation is only suitable for stages of pancreatic carcinoma limited to the pancreas (I, II and possibly III). Even after the operation, there is a risk of the tumour recurring either in the operated area or as a tumour metastasis, e.g. in the liver or peritoneum (=recurrence).

Palliative (symptom-relieving) surgery

Under certain circumstances, surgery may be necessary, even if it is clear from the examinations that complete removal of the tumour will not be possible. This may be the case if the tumour in the pancreas is constricting the small intestine so that food intake is no longer possible, or if the tumour is obstructing the outflow of bile and endoscopic therapy is not possible. In this case, the surgeon will bypass the constrictions by suturing loops of small intestine, e.g. to the bile duct (biliodigestive anastomosis) or to the stomach (gastroenterostomy).

Chemotherapy

After an operation, you will be offered supportive (adjuvant) chemotherapy. If the tumour can no longer be removed by surgery, you will be recommended palliative chemotherapy. You will be given so-called cytostatic drugs for this purpose. These are drugs that kill rapidly growing tumour cells in the body. Chemotherapy is not expected to cure this tumour. In the case of pancreatic carcinoma, these therapies can generally only slow down tumour growth in some patients, possibly reduce the size of the tumour slightly and alleviate tumour-related symptoms. If possible, you will be offered therapy within a trial of a new treatment method.

Radiotherapy

For some patients with an advanced, non-operable tumour stage, radiotherapy will be recommended to relieve pain, e.g. in the case of metastases or pain caused by the spread of the pancreatic tumour.

Endoscopy

In patients with a pancreatic tumour, especially if it is located in the head of the pancreas, the bile duct often becomes blocked. As a result, bile can no longer flow out via the small intestine, causing symptoms such as jaundice (icterus), dark discolouration of the urine, discolouration of the stool and the associated agonising itching. The bile can become infected with bacteria and cause a severe clinical picture with fever and shock (septicaemia). Endoscopic drainage of the bile may therefore be necessary if surgery is not planned immediately. The endoscopic procedure used is called ERCP (endoscopic retrograde cholangiopancreatography). This involves an endoscopy of the stomach and small intestine using a device that allows the opening of the bile duct and pancreatic duct in the small intestine (papilla) to be located. The ducts can be visualised with contrast medium to confirm the diagnosis and for orientation. Following endoscopic splitting of the papilla, so-called stents can then be inserted into the bile duct to allow bile and possibly pus to drain into the small intestine. For this purpose, small plastic tubes (plastic stents) can be placed in the bile duct, which remain open for an average of 3-4 months and can be easily replaced if they become blocked. Alternatively, so-called metal mesh stents can be inserted, which consist of a wire mesh, have a larger inner opening and remain open for longer. These stents can usually no longer be removed. Your attending physician will discuss the various stent therapy options with you in detail and decide on the type of stent in consultation with you.

Supportive (palliative) therapy

For all patients with a pancreatic tumour, we will initiate supportive measures to alleviate symptoms in addition to the specific therapies already mentioned. As a rule, this mainly concerns patients with advanced tumours. Supportive therapy includes, for example, pain therapy, physiotherapy, nutritional advice, ensuring nutrition, care or the organisation of home care.

Recurrence is the recurrence of the tumour after surgical treatment. Early palliative treatment is carried out with endoscopic, supportive, chemo- and radiotherapeutic procedures as described in detail for the palliative treatment of primary non-surgical patients.

The most important further development is to develop new procedures for the diagnosis of precursors or early stages of the tumour. This should increase the number of patients who can be operated on with a curative objective. Various procedures are being developed here, including the use of molecular biology techniques. Individual procedures are being tested as part of studies.

Furthermore, new therapeutic procedures are constantly being tested, some of which use gene therapy and others immunological approaches.

In recent years, the introduction of new chemotherapies has significantly improved the treatment of pancreatic carcinoma, particularly for patients in good general health.