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 section will detail what an adamantinoma is and how adamantinomas can be diagnosed and treated.
For a downloadable source of this information, please view our 'Downloadable Information Materials' page to view all of our fact sheets.
Adamantinoma is a rare form of primary bone cancer that is most commonly found in the shinbone (known as the tibia). 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).
Adamantinoma is a rare form of primary bone cancer that most commonly occurs in the centre of the tibia (the shinbone) and can also affect the fibula (the calf bone)(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).
Adamantinomas can develop in anyone, at any age, but most commonly occur in the age range of 20-35 years of age(4).
This cancer is slightly more common in males than in females and tends to affect younger men once their bones have stopped growing and developing - which is known as reaching skeletal maturity(3).
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)
- An unexplained limp
- Reduced movement of the affected area
There are thought to be two types of adamantinoma;
- Classic adamantinoma
- Differentiated adamantinoma - which is also referred to as osteofibrous dysplasia-like 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.
The first step in diagnosing any primary bone cancer is usually a trip to the GP. Diagnosis of a suspected bone tumour usually follows a clinical examination and an X-ray. It is very common to be referred to a bone cancer specialist for a second opinion and confirmation of the diagnosis.
Further tests to confirm the presence of adamantinoma include: CT scans, MRI scans, a biopsy of the bone and blood tests.
More information on X-rays, CT Scans, MRI scans, bone biopsies and blood tests can be found here.
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).
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 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.
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.
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:
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
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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...
Clarke, R.K, Anatomy and Physiology: Understanding the Human Body, 2005. Jones and Bartlett Publishers, Inc, Massachusetts, ISBN 0-7637-4816-1.
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Hazelbag, H.M, Laforga, J.B, Roels, H.J, Hogendoorn, P.C.Dedifferentiated Adamantinoma with Revertant Mesenchymal Phenotype. The American Journal of Surgical Pathology. 2003; 27(2): 1530-1537. Available at: http://www.ncbi.nlm.nih.gov/pubmed/14657712
Philipose, T.R, Somayaji, B.V, Pai, M.R, Monteiro, F, Bhagavath, P, Hegde, P, Raj, B, Shetty, S. Adamantinoma and Ameloblastoma: Histologic Homologues. Journal of Evolution of Medical and Dental Sciences. 2014; 3(39). Available at: http://jemds.com/latest-articles.php?at_id=5123
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Version 2 produced September 2016
Information will be reviewed in 2019
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