Acinar cell carcinoma of the pancreas: what it is, symptoms and treatment

The pancreas is a gland behind the stomach. It has two jobs: making digestive juices that flow down small tubes into the bowel, and making hormones such as insulin that are released into the blood. Cancer Research UK explains that cancer can start in different types of pancreas cell, and that knowing the cell type and where in the pancreas it began helps doctors plan the best treatment.

Most pancreatic cancers are of the exocrine type, meaning they start in cells that produce digestive juices. The great majority of these are ductal adenocarcinomas, which start in the cells lining the ducts. Acinar cell carcinoma is a much less common exocrine cancer. It starts in acinar cells, which are in the smallest tubes at the ends of the ducts and make the enzymes in pancreatic juice.

Because it is rare, there is far less patient-level information about acinar cell carcinoma than about pancreatic cancer in general. The UK sources read for this page describe the cell type briefly and then cover pancreatic cancer as a whole. Where this guide describes symptoms, tests and treatment, it is therefore describing the general pancreatic cancer pathway, and your own specialist team will tell you how your particular tumour differs.

Cancer Research UK states two differences in plain terms. Cancers of the acinar cells are generally diagnosed at a younger age than adenocarcinomas, and they tend to have a better outlook. The same page lists several other rarer types, including cystic tumours, neuroendocrine cancers and very rare childhood tumours, and says some of these other rare types are treated differently to the more common types.

That is worth holding in mind if you search online. Pancreatic cancer statistics usually describe ductal adenocarcinoma, which is the most frequent type, and they may not apply to someone with an acinar tumour. A general figure on a charity page is a population average for the common type, not a prediction for you.

The practical consequence is that the pathology report matters. If you or a relative have been told the tumour is of acinar origin, it is reasonable to ask the specialist team what that means for the treatment plan and for the outlook, and to ask them to explain any terms in the report that are unclear.

The NHS says pancreatic cancer may not have any symptoms, or they might be hard to spot. Symptoms listed by the NHS can include the whites of the eyes or skin turning yellow (jaundice), sometimes with itchy skin, darker pee and paler poo than usual; loss of appetite or losing weight without trying; feeling tired or having no energy; and a high temperature or feeling hot, cold or shivery.

Other symptoms can affect digestion: feeling or being sick, diarrhoea or constipation, other changes in your poo, pain at the top of the tummy and back that may feel worse when eating or lying down and better when leaning forward, and symptoms of indigestion such as feeling bloated.

The NHS adds that some of these symptoms are very common and can be caused by other conditions. Having them does not definitely mean you have pancreatic cancer, but it is important to get checked by a GP.

• Ask for an urgent GP appointment or get help from NHS 111 if the whites of your eyes or your skin turn yellow, if you are being sick for more than 2 days, or if you have diarrhoea for more than 7 days.

• See a GP if you have lost a noticeable amount of weight, or if other symptoms get worse or do not get better after 2 weeks.

• See a GP if you have a digestive condition whose symptoms are not improving after 2 weeks of your usual treatments, or if long-standing symptoms change or no longer feel normal for you.

The sources read do not give a specific cause for acinar cell carcinoma. For pancreatic cancer generally, the NHS says anyone can get it and that it is not always clear what causes it. It lists being over 65, certain medical conditions such as chronic pancreatitis, and a family history of pancreatic cancer as things that may make it more likely, and notes that many pancreatic cancers are linked to lifestyle.

The NHS suggests that losing weight if you are overweight, cutting down on red and processed meat, cutting down on alcohol and trying to quit smoking can lower the chance of getting pancreatic cancer, while accepting that it cannot always be prevented. These points come from advice about pancreatic cancer in general, and they are not specific to the acinar type, which tends to occur at a younger age than the common type according to Cancer Research UK.

If a GP thinks your symptoms could be cancer they may arrange an urgent referral to a specialist. The NHS says this does not definitely mean you have cancer. At the GP appointment the doctor may feel your tummy and may ask for a pee sample or a blood test.

The NHS lists the main tests for pancreatic cancer as blood tests; scans including ultrasound (sometimes from inside the body using an endoscope), CT, PET or MRI; collecting a small sample of cells from the pancreas (a biopsy); a small operation to look inside the tummy (laparoscopy); and ERCP, where an endoscope is passed through the mouth to the stomach and X-rays are taken of the pancreas. You may not have all of them.

It is the biopsy and the pathology report that identify the cell type, which is how an acinar cell carcinoma is distinguished from the more common adenocarcinoma. The NHS says results can take several weeks and that a long wait does not mean anything is definitely wrong. A specialist explains the results and what happens next, and your team will include a clinical nurse specialist as your main point of contact.

The NHS says treatment for pancreatic cancer depends on the size and type of cancer, where it is, whether it has spread and your general health. It may include surgery, chemotherapy, radiotherapy and supportive care. If the cancer is found early and has not spread, surgery to remove it may be possible. The NHS explains that surgery removes part or, in a small number of cases, all of the pancreas, and that other organs nearby may also need to be removed. Recovery can take a long time.

Chemotherapy can be used before surgery to make the cancer smaller, after surgery to help stop it coming back, to control symptoms when surgery is not possible, and sometimes alongside radiotherapy. Radiotherapy is not often used for pancreatic cancer, but may be offered in some situations.

Supportive care matters at every stage. The NHS lists help with eating and weight loss, including enzyme replacement tablets to help you digest food, pain relief, treatment for tiredness and anti-sickness tablets. If a cancer cannot be cured, a palliative or symptom control team works alongside your specialists to help you feel more comfortable.

How much of this applies to an acinar tumour in particular is a decision for the multidisciplinary specialist team. Cancer Research UK says the better outlook of acinar cancers is a general tendency; it does not change the need for a full assessment of stage and fitness for treatment.

In the UK, suspected pancreatic cancer is usually investigated through an urgent GP referral to hospital specialists, and treatment is planned by a team of specialists. The NHS notes that a group of specialists looks after you through diagnosis and treatment. Some people also use private care for scans or consultations, which can run alongside NHS care; any private results should be shared with the NHS team so that one team holds the whole picture.

Because acinar cell carcinoma is rare, some people ask for a second opinion on the pathology or the plan. It is reasonable to raise this with your specialist or GP, who can arrange for your records and slides to be reviewed.