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Rare prostate cancers

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About rare prostate cancers

Although prostate cancer is a common cancer in men, there are different types of prostate cancer, and some of these are rare. Because they are rare, we don’t know as much about them. If you are diagnosed with one of the cancers mentioned here, speak to your doctor or nurse about what that means and what treatments are suitable for you.

Like most things in our body, the prostate is made up of different types of cells (see image below). The type of cancer that develops depends on the cell it starts in.

The most common type of prostate cancer starts in some of the cells that line the prostate, called glandular epithelial (gland) cells. There are two types of gland cells – basal cells and luminal cells (see image). Prostate cancer can develop in either of these cells.

When we talk about common prostate cancer here, we mean this type of prostate cancer. You may hear it called adenocarcinoma or acinar adenocarcinoma or see this written in your biopsy results (your 'pathology' report).

Rarer types of cancer can also develop from gland cells, or from other types of cells in the prostate.

prostate tissue structure

Some men have more than one type of prostate cancer. For example, they may have some common prostate cancer as well as a rare cancer.

Some of the rare cancers may be more aggressive than common prostate cancer. This means they may grow faster and are more likely to spread outside the prostate.

Some of the tests used to diagnose prostate cancer may not be as good at picking up rare prostate cancers. For example, some rare prostate cancers – such as small cell prostate cancer – don’t cause your PSA level to rise, so they’re not always picked up by a PSA test. Because of this, some rare cancers may not be diagnosed until after they have spread outside of the prostate.

These different types of prostate cancer look different under a microscope, so may be picked up after having a biopsy to check for prostate cancer, or surgery called  transurethral resection of the prostate (TURP) to treat an enlarged prostate. Rare cancers aren't always given a Gleason score after a biopsy. This is because they can behave differently from common prostate cancer and can’t be measured in the same way.

Because rare cancer can be aggressive and spread outside the prostate, you will probably have more tests, such as a CT scan, an MRI scan or a bone scan, to check if it has spread.

Neuroendocrine prostate cancers

Neuroendocrine prostate cancers develop from neuroendocrine cells in the prostate. There are several types of neuroendocrine prostate cancers, including small cell prostate cancer and large cell prostate cancer. These can be mixed with common prostate cancer.

Small cell prostate cancer

Small cell prostate cancer is the most common neuroendocrine prostate cancer.

Neuroendocrine cells do not produce PSA, so a PSA test won’t help to diagnose small cell prostate cancer. And it won’t be used to monitor it. This means a biopsy or transurethral resection of the prostate (TURP) is needed to diagnose small cell prostate cancer.

Small cell prostate cancer is aggressive and can spread quickly to other parts of the body.

Because it is aggressive and can’t be picked up by PSA tests, most small cell prostate cancers are diagnosed when they have already spread to other parts of the body (advanced prostate cancer).

You probably won’t be given hormone therapy if you have small cell prostate cancer, as it’s unlikely to help. But you may be offered several treatment options, and sometimes a combination of treatments, including surgery and chemotherapy, either on its own or with radiotherapy.

Sometimes men can have common prostate cancer and small cell prostate cancer at the same time. If you have both types of cancer, you may be treated with a combination of hormone therapy and chemotherapy.

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Large cell prostate cancer

This is another type of neuroendocrine prostate cancer. It is also aggressive and can spread quickly to other parts of the body.

Large cell prostate cancer is very rare. Because of this, we don’t yet know how it develops, or the best ways to treat it.

Although neuroendocrine cells don’t produce PSA, studies of men with large cell prostate cancer show that PSA levels range from low PSA levels to high PSA levels. More research is needed before we can know whether PSA tests can help to diagnose large cell prostate cancer.

You probably won’t be given hormone therapy if you have large cell prostate cancer, but you may be offered several treatment options, including surgery and chemotherapy. You may be offered radiotherapy as well as these depending on how much the cancer has grown and whether it has spread. 

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Glandular prostate cancers

Like common prostate cancer, some rare prostate cancers can develop from glandular epithelial (gland) cells in the lining of the prostate. Ductal prostate cancer, mucinous prostate cancer and signet ring cell prostate cancer are all glandular cancers. You may hear them called adenocarcinomas. These can be mixed with common prostate cancer.

Ductal prostate cancer

You may also hear this called ductal adenocarcinoma.

Although glandular epithelial cells produce PSA, men with ductal prostate cancer often have normal PSA levels. So men are often diagnosed when the ductal prostate cancer has already spread out of the prostate.

Ductal prostate cancer usually grows close to the urethra, which is the tube you urinate (pee) through. Because of this it can cause the urethra to narrow, which can cause difficulty urinating and blood in your urine. This means ductal prostate cancer is often diagnosed when men have surgery called transurethral resection of the prostate (TURP) to treat their urinary problems, as the tissue removed during surgery is looked at under the microscope.

Ductal prostate cancer is usually more aggressive than common prostate cancer. Possible treatment options include surgery, hormone therapy, radiotherapy and chemotherapy, depending on whether your cancer has grown and spread to other parts of your body.

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Mucinous prostate cancer

You may also hear this called mucinous adenocarcinoma.This type of prostate cancer is very rare and usually mixed with common prostate cancer.

As with common prostate cancer, mucinous prostate cancer can cause the levels of PSA in the blood to rise.

Similar to other rare cancers, there isn’t much research on mucinous prostate cancer. Early studies suggest it’s aggressive and doesn’t respond well to treatments for common prostate cancer. But more recent research shows it might not be as aggressive as once thought.

Like common prostate cancer, treatment for mucinous prostate cancer will depend on whether or not the cancer has spread outside the prostate. Treatments can include surgery and radiotherapy.

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Signet ring cell prostate cancer

You might also hear this called signet cell prostate cancer or signet ring cell adenocarcinoma.

Signet ring cell cancer often starts in the bladder or stomach, and then spreads to the prostate. A signet ring cell cancer that started in the stomach is stomach cancer, even if it has spread to the prostate. But a small number of signet ring cell cancers actually start in the prostate. These cancers are very rare, and we don’t know very much about them. 

Like common prostate cancer, signet ring cell prostate cancer can produce high levels of PSA. This means a PSA test can be used to help diagnose signet ring cell prostate cancer. But a biopsy is needed to confirm it is a signet ring cell cancer.

If your biopsy shows that you have signet ring cell cancer, you may need further tests to check whether it started in your prostate or somewhere else. If your cancer started in another part of the body, it will affect the type of treatment you have. For example, if the cancer spread to the prostate from your stomach, you will be offered treatment for stomach cancer, not prostate cancer.

Signet ring cell cancer can be very aggressive. You may be offered surgery, radiotherapy, hormone therapy or a combination of these to treat the cancer. Your treatment will depend on how much the cancer has grown and whether it has spread to other parts of the body.

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Basal cell prostate cancer

You might hear this described as adenoid cystic prostate cancer, or basaloid carcinoma. These can be mixed with common prostate cancer.

Basal cell prostate cancer is very rare and we don’t know very much about it.

Basal cells don’t produce PSA, and most men with basal cell prostate cancer have normal levels of PSA in their blood. This means that a PSA test probably won’t help to diagnose basal cell prostate cancer. 

Basal cell cancer can grow big enough to cause the urethra (the tube you urinate through) to narrow. This can cause difficulty urinating and blood in your urine. So basal call prostate cancer is often diagnosed when men have surgery called transurethral resection of the prostate (TURP) to treat their urinary problems, as the tissue removed during surgery is looked at under the microscope.

We don’t know how aggressive it is. Some studies suggest it isn’t very aggressive. But other studies suggest it might be more aggressive than common prostate cancer.

You may be offered surgery, chemotherapy or radiotherapy to treat basal cell prostate cancer. Your treatment options will depend on how much the cancer has grown and whether it has spread to other parts of the body. 

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Transitional cell prostate cancer

This is also known as urothelial carcinoma. This cancer starts in the cells that line the urethra (the tube you urinate through). Transitional cell cancer can start in the bladder and spread to the prostate or, more rarely, it can start in the prostate itself.

Studies of men with transitional cell prostate cancer show that PSA levels can be low or high. More research is needed before we can know whether PSA tests can help to diagnose transitional cell prostate cancer.

Men with this cancer often have difficulty urinating and find blood in their urine. This is because the cancer grows around the urethra, causing it to narrow. This means transitional cell carcinoma is often diagnosed when men have surgery called transurethral resection of the prostate (TURP) to treat their urinary problems, as the tissue removed during surgery is looked at under the microscope.

If the cancer started in the prostate and has not spread outside the prostate, then you may be offered surgery and radiotherapy. If the cancer has spread to areas just outside the prostate (locally advanced prostate cancer) or to more distant areas of the body such as the bones (advanced prostate cancer) then chemotherapy and/or radiotherapy may be an option.

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Prostate sarcomas

Sarcomas are rare cancers that can develop anywhere in the body, including the prostate. Unlike common prostate cancer, sarcomas do not develop from cells that line the prostate (glandular epithelial cells). Instead, they develop from smooth muscle cells in the prostate, called mesenchymal cells (see image above). 

There are several types of prostate sarcomas. Some prostate sarcomas can be aggressive, but others are not.

Leiomyosarcoma

This is the most common type of prostate sarcoma in adults (it is not the same as leiosarcoma).

Leiomyosarcoma is mostly found in men who are 40 years or older. But it can also sometimes be found in children and young men and can be aggressive.

In most cases, men with leiomyosarcoma will have normal levels of PSA in the blood. This means that a PSA test may not help to diagnose leiomyosarcoma.

Leiomyosarcoma can grow big enough to cause the urethra (the tube you urinate through) to narrow. This can cause difficulty urinating and blood in the urine. So it is often diagnosed when men have surgery called transurethral resection of the prostate (TURP) to treat their urinary problems, as the tissue removed during surgery is looked at under the microscope.

Most are aggressive and can spread to other parts of the body. Because of this, surgery is often used to treat it. But you may also be offered other treatments, for example hormone therapy, chemotherapy and radiotherapy, depending on how much the cancer has grown and whether it has spread to other parts of the body.

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Rhabdomyosarcoma

Rhabdomyosarcoma is another type of rare prostate sarcoma. It is mostly found in children, but can also be found in adult men, although this is rare.

As with leiomyosarcoma, boys and men with rhabdomyosarcoma usually have normal blood PSA levels, so a biopsy and scans are needed to diagnose rhabdomyosarcoma.

Men or children with this cancer will often have problems urinating as the cancer can cause the urethra (the tube you urinate through) to narrow. So this type of prostate cancer is often diagnosed following surgery called a transurethral resection of the prostate (TURP) to treat these urinary problems.

Most rhabdomyosarcomas are very aggressive and can spread to other parts of the body. They can be treated with surgery, chemotherapy, radiotherapy or a combination of treatments, depending on whether or not the cancer has spread.

Your doctor or nurse will tell you what treatment options are available to you. Read more about how these cancers are treated below.

Other sarcomas

Some men with prostate sarcoma will have another type of prostate cancer as well, such as a glandular cancer. You may hear this called a sarcomatoid carcinoma or a carcinosarcoma. These cancers may be aggressive.

Other types of sarcoma include granular cell tumour, haemangiomas, chondromas, neural tumours, and prostatic stromal proliferations of uncertain malignant potential (STUMP). Because these are very rare, we don’t know much about them and more research is needed to find out more about whether current tests can diagnose them and what treatments work best.

How are these cancers treated?

Different prostate cancers behave in different ways, and they might respond differently to treatments. Treatment can depend on whether there is a mix of cancer types.

Some rare cancers may be treated in a different way to common prostate cancer. For example, hormone therapy does not work for men with small cell cancer and so isn’t usually given as a main treatment for this.

Because these cancers are so rare, there isn’t enough evidence to say which treatments will work best for all of them. Your treatment will depend on your situation – for example, the type of cancer you have and whether it has spread to other parts of the body. Your symptoms, and any treatments you’ve already had, may also affect the treatment you’re offered.

If you have any questions about your cancer speak to your doctor or nurse. They’ll be able to explain your test results and talk you through your possible treatment options. 

Treatments for localised cancer

If your cancer is contained within the prostate (localised prostate cancer), you may be offered treatment that aims to get rid of the cancer such as surgery or radiotherapy.

Some rare prostate cancers may be more likely to come back after treatment than common prostate cancer. If there’s a risk your cancer could spread outside the prostate after treatment, you might be given other treatments before, at the same time as, or after your main treatment.

For example, if you have surgery, you might have radiotherapy and hormone therapy or chemotherapy as well. These treatments can help make the main treatment more effective. But they can also increase the risk of side effects.

If you have small cell prostate cancer you might have chemotherapy before another treatment.

Treatments for locally advanced and advanced cancer

All types of prostate cancer can spread outside the prostate – to the area around the prostate (locally advanced prostate cancer), and to other parts of the body, such as the bones (advanced prostate cancer). But some rare prostate cancers may be more likely to spread to places like the brain, lungs and liver.

Hormone therapy is often used to treat locally advanced and advanced prostate cancer, but not all rare prostate cancers respond well to it. This means you might have chemotherapy (see below) or a combination of chemotherapy and hormone therapy.

When prostate cancer spreads to other parts of the body it can cause symptoms, such as pain. But there are ways to manage these.

Chemotherapy

You may be offered chemotherapy if hormone therapy stops working for you, or if hormone therapy isn’t suitable for your cancer.

In the UK, docetaxel (Taxotere®) is the standard chemotherapy treatment for men with advanced prostate cancer that is no longer responding to hormone therapy. But you might have a different chemotherapy if you have a rare cancer.

For example if you have small cell prostate cancer, you may have carboplatin or cisplatin chemotherapy. You will have these together with another chemotherapy drug, such as docetaxel or etoposide. You might have them with another treatment such as hormone therapy. This depends on your situation.

Like all treatments, chemotherapy has side effects. And different chemotherapy drugs may have different side effects. Macmillan Cancer Support has more information about the side effects of different chemotherapy drugs and ways of managing them.  

There has been a new large study into the role of chemotherapy and the best time to give it to men with prostate cancer. Our Specialist Nurses can speak to you about these recent findings.

Where can I get support?

Being diagnosed with any kind of prostate cancer can be frightening and overwhelming. If you are told you have a rare prostate cancer you may worry about what this means, and feel frustrated that there isn’t much information available about your diagnosis and treatment.

No matter what you’re feeling or thinking, there is support available if you want it. You can speak to our Specialist Nurses, in confidence, or chat with them online. Our information on Dealing with prostate cancer looks at things you can do to help yourself and people who can help.

The Rarer Cancers Foundation provides information and support to people with rare types of prostate cancer. You can also join their online community.

Questions to ask your doctor or nurse

  • What type of prostate cancer do I have?
  • Do I have a mixture of common and rare prostate cancer?
  • Is my cancer aggressive?
  • How far has my cancer spread?
  • Will I need more tests, such as an MRI, CT or bone scan?
  • What treatments are suitable for me?
  • What are the side effects of the treatments?

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References

Updated: January 2016 | Due for review: January 2018

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