Adamantinoma

This information has been written for patients, their families and friends and the general public to help them understand more about a rare form of primary bone cancer, known as adamantinoma. This page will detail what an adamantinoma is and how adamantinomas can be diagnosed and treated.

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What is adamantinoma?

Adamantinoma is a rare form of primary bone cancer that is most commonly found in the shinbone (known as the tibia) and can also affect the fibula (the calf bone)(1). This cancer accounts for less than 1% of all primary tumours arising in the bone and requires the surgical removal of the tumour as the gold standard treatment method for adamantinoma(1).

These tumours grow slowly and are therefore referred to as ‘low-grade tumours’. Due to this slow growth, patients often suffer from symptoms for a long period of time before going to see a doctor.

Although adamantinomas grow slowly, they are capable of spreading to other areas of the body over time, including: the central canal of the bone, nearby muscles, nearby soft tissue, other bones and distant organs such as the lungs and lymph nodes. The spread of the cancer to other areas of the body (known as metastasis) happens in around 15-20% of adamantinoma patients and can occur many years after the diagnosis and treatment of the primary adamantinoma has taken place(2).

The slow-growing nature of an adamantinoma can make symptoms non-specific to primary bone cancer, and some patients may not have any symptoms at all for some time.

Symptoms are very dependent on where the tumour is located(1,4).

The most common symptoms reported by adamantinoma patients are:

Intermittent bone pain (pain that may come and go)
Swelling
An unexplained limp
Reduced movement of the affected area

For more information on the symptoms of primary bone cancer please refer to our About Primary Bone Cancer information section.

There are thought to be two types of adamantinoma;

Classic adamantinoma
Differentiated adamantinoma - which is also referred to as osteofibrous dysplasia-like adamantinoma

These two types can be distinguished by radiologists (who assess images of the tumour following diagnostic scans) or by pathologists (who assess the types of cells in the tumour under a microscope).

Classic Adamantinoma

This is the most common type of adamantinoma and is seen in adults over the age of around 20 years old. This form of adamantinoma is more aggressive than differentiated adamantinoma, which means it is capable of spreading elsewhere in the body - most commonly to the lungs(1).

Differentiated Adamantinoma (also referred to as Osteofibrous Dysplasia-like Adamantinoma)

Is less aggressive form of adamantinoma, which means it is less likely to spread to other areas of the body. This tumour tends to occurs in children and young people under the age of around 20 years old. This tumour is made up of a mix of cancerous and non-cancerous cells. The non-cancerous cells in this tumour type are very similar to cells seen in osteofibrous dysplasia (OFD), a non-cancerous childhood tumour, and so it is thought that OFD may be capable of progressing into this form of adamantinoma overtime in some cases(1,5).

The cause of adamantinoma is unknown. Due to the rarity of this cancer it is hard to find a possible cause from the small number of cases that have been reported.

Although there is currently no identifiable cause, approximately 60% of adamantinoma patients have a history of a significant trauma (or previous injury) to the affected bone. This may be a risk factor for developing an adamantinoma, or it may be that the tumour itself weakens the area of bone and increases the likelihood of an injury to this affected area. Whether a significant trauma to the bone is a risk factor for adamantinoma is still under investigation(1,2,6).

Patients with different types of primary bone cancer are assessed in similar ways. For this reason, diagnostic tests are covered in more detail in our About Primary Bone Cancer information section.

This section aims to provide information on the specific details of diagnosing an adamantinoma and to discuss other conditions that may appear diagnostically similar to adamantinoma.

Going to the doctor

The symptoms of adamantinoma are general. There is no one clear sign that doctors can easily look for to make a diagnosis of adamantinoma.

People report a variety of experiences when they seek medical advice about their symptoms. Most people with worrying symptoms go to their GP.

Some patients go to their local hospital emergency department (A&E) or other health care centres.

Some people are referred quickly for further tests or a second opinion, but often patients have to return to their GP three or four times before they are referred for more tests. Primary bone cancers are very rare and many GPs will never come across a case.

If a GP or hospital doctor is concerned about the patient's symptoms, there are national guidelines they should follow. According to the National Institute for Clinical Excellence (NICE) Guidelines for Suspected Bone Cancer and Sarcoma:

Children, teenagers and young adults with unexplained bone swelling or bone pain should have an urgent X-ray within 48 hours. If the X-ray suggests a possible bone cancer, your GP should refer you to a specialist within 48 hours.
Adults should be seen by a specialist within 2 weeks if the results of an X-ray suggest a bone cancer.

If the X-ray is normal but symptoms persist, the patient should be followed up and/or a repeat X-ray or MRI scan should be carried out within 2 weeks (adult) or within 48 hours (child) or a referral requested to a specialist.

Bone Cancer Awareness Initiative

The Bone Cancer Research Trust is trying to find ways to make the time between the start of symptoms and getting the diagnosis much shorter. Our 2020 Patient Survey report is the most comprehensive analysis of presenting symptoms and routes to diagnosis for primary bone cancers & tumours in the UK to date. This is our evidence base on which we will focus our awareness objectives moving forward.

The report focuses on two main areas - the time and routes to diagnosis and the range of presenting symptoms across all anatomical locations and forms of primary bone cancer & bone tumours.

Our analysis found that patients wait, on average, more than 7 months and make 8 visits to the multiple healthcare professionals before receiving an accurate diagnosis.

Going to a Bone Cancer Centre for more tests

Once an abnormality is found in a bone that suggests the possibility of cancer, the patient will be referred to a bone cancer surgical centre.

Bone cancer surgical centres are specialist hospitals. They have a group of healthcare specialists who are experts in the diagnosis and management of bone cancer.

In England, there are currently five bone cancer centres which specialise in the diagnosis and management of primary bone cancers. These centres are at Birmingham, Newcastle, Oswestry, Oxford, and Stanmore.

In the Republic of Ireland, there are no specific bone cancer centres. Patients are initially seen in their local hospital and subsequently referred to specialist hospitals in Dublin or Cork for further tests and, if necessary, for treatment.

Patients in Wales usually travel to Oswestry or Birmingham for these specialist tests.

In Scotland there are five sarcoma centres and so patients travel to one of these centres for diagnosis if primary bone cancer is suspected. These centres are in Edinburgh, Glasgow, Inverness, Aberdeen and Dundee.

In Northern Ireland, patients are usually seen in Belfast.

For a full list of locations patients may be referred to in order to confirm a primary bone cancer diagnosis please click here

The MDT

Specialists in many different areas of medicine at the bone cancer centres, the Regional Cancer Centres in the UK and hospitals in Ireland work together as a 'Multidisciplinary Team' (MDT). The members of the MDT work together to diagnose the patient's condition.

The MDT includes:

Specialist sarcoma surgeons.
Specialist sarcoma oncologists (oncologists are doctors who look after people with cancer).
Specialist sarcoma pathologists (pathologists are doctors who use laboratory techniques to diagnose disease).
Radiologists (doctors who diagnose disease and conditions from looking at x-rays, or scans).
Sarcoma cancer nursing specialists (sometimes called 'CNS') who perform an essential role in treating and caring for primary bone cancer patients.

What tests are done?

When a person is referred to a bone cancer surgical centre, further tests will be done to find out more and to confirm whether the patient has bone cancer, and if so what type.

These tests may include:

X-ray

X-rays of the bone may be taken, including the joints above and below, and are studied. These X-rays may show swelling around the bone or areas of abnormal bone growth. A chest x-ray is sometimes taken to show whether the cancer has spread to the lungs.Cancer Research UK gives more information about X-rays.

Blood tests

These include:

- Blood chemistry (Urea and Electrolytes) is checked to examine the levels of normal salts and urea and creatinine, which are waste products. This test can give clues to how well the kidneys are working
- Full blood count (FBC) counts the numbers of different types of blood cells in the patient's blood at that time.
- Red blood cells - which carry oxygen in the blood.
- White blood cells - which are essential to the immune system (and totals of each type).
- Platelets - which are essential to the making blood clots and scabs.
- Levels of haemoglobin - which is found in red blood cells.
- Liver function tests (LFTs) to see how the liver is working.
- Erythrocyte Sedimentation Rate (ESR) is a test to look for signs of inflammation.
- C-Reactive Protein (CRP) levels are tested as CRP levels increase in inflammation.
- Alkaline phosphatase (ALP) levels are measured in patients with suspected osteosarcoma.

Cancer Research UK gives more information about blood tests.

MRI scan

MRI stands for 'magnetic resonance imaging'. This type of scan is similar to a CT scan but magnetism and radio waves are used instead of x-rays to build up a very detailed 3-dimensional image.

An MRI scan of the entire bone is used to gain more information about the tumour in the bone. An injection of a special dye, known as a contrast agent is also used. This makes certain tissues show up more clearly and with greater detail on the scan. The results of the scan will be examined by a radiologist and a report will be produced. For some patients they may also have a total body MRI scan to look for areas of abnormalities in the other bones such as tumour spread (metastases).

Cancer Research UK gives more information about MRI scans.

CT scan

CT stands for 'computerised tomography'. They may also be called CAT scans, which stands for 'computerised axial tomography'.

The scanner takes x-rays from many different angles and a computer builds up a 3-dimensional picture of the body in great detail. The pictures show cross-sections of the inside of the body.

CT scanning of the lungs shows up any secondary tumours where the cancer may have spread (metastases). It is used if the MRI scan results have not been able to confirm the diagnosis of osteosarcoma.

Cancer Research UK gives more information about CT scans.

A CT scan and MRI scan cannot definitively diagnose an adamantinoma. However, they provide important information on the exact location of the tumour, the stage of the tumour and the presence of the tumour having spread anywhere else in the body. Additionally, these scans are useful for planning the treatment of individual patients and deciding on the best way to surgically remove the tumour(2).

PET scan

PET stands for 'positron emission tomography.' Not all hospitals have PET scanners but they are used to detect spread or metastases in osteosarcoma.

PET scans can examine the whole body, rather than a specific area. They can also detect how well treatments are working.

Before the scan, a small injection of radioactive glucose (a radiotracer) called fluorine18 will be given.

The tracer will take around an hour to spread around the body. During the scan, which can last about an hour, the patient lies on a bed and the scanner passes over them. The scanner detects where the radiation is concentrated and produces images. Hot spots on the PET scan can detect metastases.

The results of the scan will be examined by a radiologist.

Cancer Research UK gives more information about PET scans.

Biopsy

Taking a biopsy of the bone is needed to confirm the diagnosis of adamantinoma. A bone biopsy is a specialised procedure that can be performed by a specialist in orthopaedic surgery or sarcoma radiology at a bone cancer surgical centre. A biopsy involves taking a small sample of a lump or tumour so that a pathologist can examine the cells in the sample and determine whether the lump is cancerous or not.

The biopsy being taken may be a ‘needle biopsy’ or an ‘open biopsy’.

Needle biopsy: a needle is inserted into the tumour to draw out a small amount of tumour tissue (this may be done under local anaesthetic). Often, in order to know exactly where to take the sample from, this test is carried out alongside an X-ray or CT scan to guide the doctor.
Open biopsy (or surgical biopsy): is used less frequently than a needle biopsy. This form of biopsy is carried out during a small, minor, operation to remove a small piece of tumour while under general anaesthetic. This test tends to be used if a needle biopsy does not provide a diagnosis and the doctor wish to investigate further.

Cancer Research UK gives more information about bone biopsies..

Taking a biopsy of the bone is needed to confirm the diagnosis of adamantinoma. This specialist procedure takes a small sample of the tumour so it can be examined by a pathologist. This includes looking at the sample under a microscope and doing special tests for certain bone matrix proteins and molecules that are specific to this tumour type.

Results from a biopsy can take up to two weeks to become available and they enable doctors to confirm the presence of an adamantinoma.

An alternative diagnosis?

When diagnosing an adamantinoma, the patients’ age and clinical history plays a large role in differentiating this rare tumour from other health conditions. These health conditions may present similarly to adamantinoma, in terms of symptoms and signs, but it is important the correct diagnosis is made to ensure the treatment provided is suitable. The location of the suspected adamantinoma is helpful in confirming the diagnosis(2).

It can sometimes take a long time to confirm a diagnosis of primary bone cancer after a biopsy, this is because these tumours are rare and sometimes difficult to identify. Diseases with similar symptoms or signs are known as ‘differential diagnoses’.

Other conditions which can present in the same way as adamantinoma include:

Osteofibrous Dysplasia

Osteofibrous dysplasia is a benign (non-cancerous) childhood condition which regresses at the age of around 20 years old. There are similarities between osteofibrous dysplasia and adamantinoma, such as the tumour’s location and their appearance on an X-ray. The age of the patient when symptoms begin is a major factor in differentiating an adamantinoma from osteofibrous dysplasia(4,7).

Fibrous Dysplasia

Fibrous dysplasia is an uncommon bone disorder which mostly occurs in adolescents and young adults. It occurs when scar-like tissue (fibrous tissue) develops in place of normal bone. Patients experience bone pain in the long bones of the body, such as the arms or legs, in a similar manner to adamantinoma(4).

Osteomyelitis

Osteomyelitis is inflammation (redness, swelling, pain and/or a feeling of heat) of the bone, or bone marrow, due to a bacterial or fungal infection. The infection may reach the bone through the bloodstream or through a direct injury that leaves a deep cut or open wound. This condition often affects the long bones of the body, such as the tibia (shin bone), and causes bone pain(5).

Non-Ossifying Fibromas

Non-ossifying fibromas are the most common benign (non-cancerous) childhood tumour of the bone, however most patients do not experience any symptoms. These tumours occur in the tibia (shin bone) and regress once the patients’ bone structure has fully developed. The location of this benign tumour is very similar to an adamantinoma and so the age of the patient when the symptoms begin is a major factor in differentiating an adamantinoma from a non-ossifying fibroma(8).

If a diagnosis of adamantinoma is confirmed, you will need treatment in a bone tumour centre.

For more information on the locations of Bone Cancer Centres please click here.

It is known that there is little benefit of radiotherapy or chemotherapy in the treatment of adamantinoma. However, as chemotherapy reaches the whole body, it may be given if there is a spread of the tumour to other areas of the body - which occurs in approximately 15-20% of adamantinoma cases.

The most effective treatment is the surgical removal of the tumour, with ‘wide surgical margins’; this means some healthy tissue is removed alongside the tumour to ensure all tumour cells are removed and to lower the risk of the tumour returning at a later date(1,4).
Usually an adamantinoma can be treated with ‘limb-sparing surgery’, which means the tumour is completely removed whilst keeping as much of the normal function and cosmetic appearance of the limb as possible.

Common types of limb-salvaging surgery performed to treat an adamantinoma are:

Autografts - once the tumour is removed the affected area of bone is reconstructed using the patients’ own bone from another area of their body. The downside to this is that the patient will have two surgical sites to recover from, which can increase recovery time.
Allografts - an allograft uses donated bone to reconstruct the affected area of the bone once the tumour has been removed.
Metallic Replacement - once the tumour is removed the area of damaged bone is replaced with a metal implant known as a prosthesis.

All of these operations will require a long period of rehabilitation after surgery (such as physiotherapy), to allow the patient to return to an active day-to-day life.

Amputation (removal of the limb) may be required in certain cases, if the tumour cannot be removed completely leaving a useful limb. There is no evidence to suggest that amputation improves long term survival more than limb-sparing surgery and therefore it is avoided wherever possible.

It is important that the tumour is fully removed during surgery, to avoid the tumour returning later in the patient’s life, which is known as ‘recurrence’. Early diagnosis and the correct treatment, with complete surgical removal of the tumour, leads to an excellent outlook for patients with adamantinoma.

It is important to bear in mind that patients receiving treatment outside of the UK may receive different tests and treatment plans in accordance to the treatment guidelines set out in that specific country. If you have any questions or concerns regarding this please discuss this with your medical team or contact The Bone Cancer Research Trust for more information.

For more information regarding treatment procedures, including surgery, chemotherapy and radiotherapy, please visit our About Primary Bone Cancer information page.

If you would like more information about adamantinoma, please contact us.

Prognosis

The word 'prognosis' refers to what doctors think the chances are of the patient's cancer being cured with treatment or the likelihood of it returning. This depends on many different things, which vary between different patients.

Doctors cannot be absolutely certain about a patient's prognosis because each patient and each cancer can behave differently.

Follow-up care

After finishing treatment, adamantinoma patients require follow-up care. Outpatient hospital visits will be needed on a regular basis for the first few years after treatment, and then yearly after that.

These visits help the clinical team to keep an eye on a patient's general health as well as an opportunity to carry out some tests. These tests are very important because they can show up any 'late effects' from the cancer treatment. Most centres encourage patients to get in touch if they have problems between appointments.

Follow up care with an orthopaedic surgeon also helps to look out for surgery-related complications and to make sure the limb is working well.

Reaching the end of treatment can bring about mixed emotions - the thought of having no more treatment can be a cause for celebration, but this can be mixed with anxiety for the future. Many emotions can arise surrounding the process of returning to a 'normal' life after cancer.

The issues encountered after treatment can be complex and include workplace and financial issues. Macmillan Cancer Care provides a wealth of useful information on living with and after cancer.

Relapse and metastases

In some patients, the cancer returns after treatment.

'Relapse' means that the cancer has returned. This can be in the same bone or area as the original cancer (local relapse) or in a different place, often in the lungs (metastasis).

If the cancer returns it will require further treatment. This treatment may involve more surgery, or possibly radiotherapy or chemotherapy. Your doctor will be able to advise you about which treatments are necessary.

What are late effects?

The surgical treatment of adamantinoma can involve major surgery and this can affect patients' long-term mobility. Some patients who have surgery in the pelvis may need to use a stick or crutches for walking. Patients will work together with physiotherapists and occupational therapists to improve their mobility and independence after such surgery.

Most patients experience side effects during their treatments which go away or improve after treatment is ended. However, some side effects become permanent or develop months or years after treatment has ended. These are known as late effects of cancer treatment. More is becoming known about late effects as people are living longer after being treated for cancer.

The late effects of cancer treatment vary depending on the type of cancer, the treatment and surgeries and the age of the patient when undergoing treatment.

However, not everyone who has cancer treatment will necessarily experience late effects. Different chemotherapy drugs cause different late effects and late effects of radiotherapy and surgery will affect only the area of the body exposed to them.

Signs and Symptoms of Late Effects

You can talk to your doctor or CNS about the late effects of your particular treatment or surgery. They should be able to advise you on which late effects you are at risk from. However, the late effects of many cancer treatments still often go undiagnosed.

At your follow-up appointments you will be screened for late effects of your cancer treatment and surgery. It is also an opportunity to talk to your doctor about any signs or symptoms you may be suffering from and to discuss whether these are late effects.

If you were treated for cancer many years ago or are no longer having follow-up appointments, you should talk to your GP about late effects or contact a Late Effect Clinic.

Late Effects Clinics

If you had your cancer treatment as a child you will be monitored for late effects by your oncology team or a late effects clinic specifically for those patients treated as children.For all other cancer patients, they will be screened by their oncologist whilst under their care but are no longer monitored once they are discharged by their oncology/surgical team.

In September 2014, a late effects clinic was established at Nottingham City Hospital.This Late Effects Clinic is a bespoke service which aims to support people suffering with long-term effects from radiotherapy and chemotherapy and offers practical advice and signposting for a range of physical and psychological effects. It offers a wide range of support and guidance regarding late effects such as bowel and bladder problems, infertility, osteoporosis, sexual dysfunction, pain management and psychological issues to name a few. Patients self-refer and it is open to all patients who are a minimum of 6 months out of treatment. There is no upper limit on time from finishing treatment.

Since this clinic was established, similar clinics have been set up in Derby, Sheffield and Taunton and many more are developing throughout the country.

There is also a Complex Cancer Late Effects Rehabilitation Service based at The Royal National Hospital for Rheumatic Disease in Bath which is a two-week inpatient rehabilitation service for those patients with complex, chronic late effects. Referral to this service is via the late effect clinic or local pain services.

You may find our webinar on the Late Effects Clinic with radiographer, Emma Hallam helpful.

Key References:

1. Jain, D, Jain, V.K, Vasishta, R.K, Ranjan, P, Kumar, Y. Adamantinoma: A Clinicopathological Review and Update. Diagnostic Pathology. 2008; 3:8. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC227648...

2. Maharaj, M, Korowlay, N, Ellmann, P. The Complimentary Role of Methoxy-Isobutyl-Isonitrile and Hand-Held Gamma Probe in Adamantinoma. The World Journal of Nuclear Medicine. 2016; 15(1):50-52. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC472901...

3. Papagelopoulos, P.J, Mavrogenis, A.F, Galanis, E.C, Savvidou, O.D, Inwards, C.Y, Sim, F.H. Clinicopathological Features, Diagnosis, and Treatment of Adamantinoma of the Long Bones. Continuing Medical Education. 2007; 30(3): 211-217. Available at:
http://www.smbs.buffalo.edu/ortho/tumorarticles/Ad...

4. Dodd, L.G and Bui, M.M, Atlas of Soft Tissue and Bone Pathology: With Histologic, Cytologic and Radiologic Corrections, 2015. Desmos Medical Publishing, New York, ISBN 9781620700372.

5. Hatzenbuehler, J and Pulling, T.J. Diagnosis and Management of Osteomyelitis. American Family Physician. 2011; 84(9): 1027-1033. Available at: http://www.ncbi.nlm.nih.gov/pubmed/22046943

6. Camp, M.D, Tompkins, R.K, Spanier, S.S, Bridge, J.A and Bush, C.H. Adamantinoma of the Tibia and Fibula with Cytogenetic Analysis. Radiographics, 2008; 28(4). Available at: http://pubs.rsna.org/doi/full/10.1148/rg.284075173

7. Jung, JY, Jee, WH, Hong, S.H, Kang, H.S, Chung, H.W, Ryu, KN, Kim, JY, Im, SA, Park, JM, Sung, MS, Lee, YS, Hong, SJ, Jung, CK, Chung, YG. MR Findings of the Osteofibrous Dysplasia. Korean Journal of Radiology. 2014; 15(1): 114-122. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC390984...

8. Bowers, L, Cohen, D, Bhattacharyya, I, Pettigrew, J, Stavropoulos, M. The Non-Ossifying Fibroma: A Case Report and Review of the Literature. Head and Neck Pathology. 2013; 7(2):203-210. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC364226...