Extragnathic Adamantinoma

Extragnathic Adamantinoma

Article
Bone, Muscle, & Joint
Diseases & Conditions
+1
Contributed byMaulik P. Purohit MD MPHSep 11, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Adamantinoma of the Long Bones

What is Extragnathic Adamantinoma? (Definition/Background Information)

  • Extragnathic Adamantinoma is a rare primary tumor of the bone. These tumors are most commonly found on the tibia (shinbone), but can also occur on other long bones of the legs and arms, such as the femur, humerus, radius, or ulna
  • The cause of Extragnathic Adamantinoma is currently unknown. The condition affects a wide age range of individuals; however, it is typically seen in children and young adults
  • The tumors may be present as painless or painful masses and may cause pathological fractures in the affected bones. Extragnathic Adamantinomas are associated with osteofibrous dysplasia (a rare and benign bone condition)
  • Complete surgical excision and removal is the preferred method to treat these malignant tumors affecting the long bones. A lack of treatment or a delay in treatment may cause additional complications (such as metastasis) and may even lead to death
  • The prognosis of Extragnathic Adamantinoma largely depends on the stage of the tumor - lower the stage of the tumor, better is the prognosis

Who gets Extragnathic Adamantinoma? (Age and Sex Distribution)

  • Individuals between the ages of 3-72 years can develop Extragnathic Adamantinoma
  • The condition is mainly seen in the 2nd and 3rd decades of life. Thus, it affects children and young adults in the 10-29 year age group, more often
  • Both males and females are equally affected
  • No particular racial or ethnic predilection is observed

What are the Risk Factors for Extragnathic Adamantinoma? (Predisposing Factors)

The risk factors for Extragnathic Adamantinoma may include:

  • Patients with osteofibrous dysplasia have greater chances of developing this bone tumor condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Extragnathic Adamantinoma? (Etiology)

The cause of Extragnathic Adamantinoma development is currently unknown.

What are the Signs and Symptoms of Extragnathic Adamantinoma?

The signs and symptoms of Extragnathic Adamantinoma are as follows:

  • Extragnathic Adamantinomas are slow-growing malignant tumors; the individuals may be asymptomatic for a long period of time
  • The bone tumors may appear as painless or painful masses
  • Fractures may be observed around the tumor site (termed as pathological fractures)
  • The long bones affected by the condition are the shinbones (most frequently), the thighbones, and bones of the upper arm and forearm

How is Extragnathic Adamantinoma Diagnosed?

A diagnosis of Extragnathic Adamantinoma may involve:

  • Through physical exam with evaluation of complete medical history
  • A tissue biopsy of the tumor: Histopathological studies may be conducted on a biopsy specimen; the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
  • X-ray of the affected bone will reveal a multicystic tumor with a classical “soap bubble” appearance

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Extragnathic Adamantinoma?

The possible complications of Extragnathic Adamantinoma are as follows:

  • Recurrence of the condition after treatment
  • Tumor metastasis (spread of the tumor to other regions) can affect the regional lymph nodes and lungs; this tumor metastasis may ultimately result in death
  • Loss of limb due to amputation

How is Extragnathic Adamantinoma Treated?

Extragnathic Adamantinoma treatment measures may include:

  • The most common treatment is surgery involving curettage of the tumor. However, greater than 60% of the patients, who are treated with curettage, will experience a tumor recurrence
  • A complete surgical excision with clear margins is the preferred method, since it has a lower rate of recurrence
  • If complete excision is not possible, then amputation of the limb might have to be performed
  • Post-operative care is important: A minimum activity level is to be ensured until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Extragnathic Adamantinoma be Prevented?

  • Current medical research has not established a way of preventing Extragnathic Adamantinoma
  • Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations, are mandatory for those who have been diagnosed with the tumor
  • Due to both its metastasizing potential and chances of recurrence, often several years of active follow-up vigilance is necessary

What is the Prognosis of Extragnathic Adamantinoma? (Outcomes/Resolutions)

  • The prognosis of Extragnathic Adamantinoma depends on the stage of the tumor
  • Lower stage tumors have a better prognosis, while higher stage tumors have a much worse prognosis

Additional and Relevant Useful Information for Extragnathic Adamantinoma:

  • It has to be observed that Extragnathic Adamantinoma is not related to gnathic adamantinoma (a tumor affecting the jaws)
  • Given the rare nature of Extragnathic Adamantinomas, some of the other diseases and bone conditions that a physician should consider are:
    • Osteofibrous dysplasia
    • Fibrous dysplasia
    • Non-ossifying fibroma
    • Osteomyelitis
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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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