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Osteosarcoma arising in Paget's Disease of Bone

Last updated Dec. 14, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Osteosarcoma arising in Paget's Disease of Bone is a rare type of malignant osteosarcoma that arises in individuals with Paget’s disease of bone (PDB); a chronic disorder affecting the bones.


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

  • Osteogenic Sarcoma in Paget’s Disease of Bone
  • Osteosarcoma arising in Bone Paget's Disease
  • Paget's Osteosarcoma

What is Osteosarcoma arising in Paget's Disease of Bone? (Definition/Background Information)

  • Osteosarcoma forms a class of highly-cancerous, high-mortality, bone tumor. After multiple myeloma, it is the second-most common primary tumor of the bones
  • Osteosarcoma arising in Paget's Disease of Bone is a rare type of malignant osteosarcoma that arises in individuals with Paget’s disease of bone (PDB); a chronic disorder affecting the bones
  • Osteosarcoma in PDB commonly involves the bones of the pelvic region, upper arm (humerus), thigh (femur), and skull region
  • The exact cause of the condition is unknown and presently, it is not possible to prevent Osteosarcoma in Paget’s Disease of Bone occurrence
  • A treatment of this bone tumor may involve surgery, chemotherapy, and radiation therapy. The prognosis of the condition depends on the stage of cancer; higher grade osteosarcomas indicate a poorer prognosis

Osteosarcomas are classified in several different ways. They may be classified based on their cause/origin, location, or even based on certain diagnostic findings.

Based on where they occur, Osteosarcoma is classified as Medullary Osteosarcoma (occurring in the bone cavity) and Surface Osteosarcoma (occurring on the bone surface).

  • Medullary Osteosarcomas are of several different types, and these include:
    • Conventional Osteosarcoma
    • Osteosarcoma of Jaw Bones
    • Post-Radiation Sarcoma
    • Osteosarcoma arising in Paget's Disease of Bone
    • Osteosarcoma in other Benign Conditions
    • Telangiectatic Osteosarcoma
    • Small Cell Osteosarcoma
    • Low-Grade Osteosarcoma
    • Multicentric Osteosarcoma
  • Surface Osteosarcomas are of several different types, and these include:
    • Parosteal Osteosarcoma
    • Periosteal Osteosarcoma
    • High-Grade Surface Osteosarcoma

Who gets Osteosarcoma arising in Paget's Disease of Bone? (Age and Sex Distribution)

  • Paget's Disease of Bone usually occurs in the 5th or 6th decade of life (generally adults over the age of 40 years and below 55 years); there is a slight male predominance
  • However, osteosarcoma has a bimodal age distribution, having the first peak during adolescence and the second peak in older adulthood
  • The second osteosarcoma peak is in adults older than 65 years of age; it is more likely to represent a second malignancy, frequently related to Paget's disease (hence termed Osteosarcoma arising in Paget's Disease of Bone)
  • Paget's Disease of Bone is rarely seen in Asian countries (such as India, China) and African regions (including the Middle East). Some individual European countries (such as UK, France, Germany) and certain people of European ancestry (in regions such as America, Canada, Australia) show a high prevalence rate of PDB

What are the Risk Factors for Osteosarcoma arising in Paget's Disease of Bone? (Predisposing Factors)

Osteosarcoma arising in Paget's Disease of Bone risk is linked to the following factors:

  • Paget's disease of bone (PDB), a bone disorder causing weak and deformed bones
  • Polyostotic fibrous dysplasia (a bone disease causing the formation of abnormal and weak bones at many locations); if present for a long time period
  • Some genetic mutations are associated with osteosarcoma; including mutations in Rb gene and P53

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 Osteosarcoma arising in Paget's Disease of Bone? (Etiology)

  • The exact cause and mechanism of Osteosarcoma arising in Paget's Disease of Bone is unknown
  • The tumor occurs due to some preexisting conditions and abnormalities (termed as a secondary osteosarcoma), which include Paget’s bone disease and fibrous dysplasia

What are the Signs and Symptoms of Osteosarcoma arising in Paget's Disease of Bone?

The presentations are based on the location of the tumor. Osteosarcoma in Paget’s Disease of Bone signs and symptoms include:

  • Signs and symptoms of Paget’s disease include bone pain and misshapen bones
  • In the initial growing phase of the tumors, they are normally asymptomatic
  • The soft tissue tumors grow at a moderate rate, and then they suddenly start to rapidly progress
  • Due to the tumor size, the adjoining organs, nerves, and muscles may be compressed or restricted. Often, these signs along with pain and tenderness, are the first indications of Osteosarcoma in PDB
  • Pathological fractures may occur at the tumor site
  • In some individuals, organ dysfunction and internal hemorrhages may be observed. These may be sudden and spontaneous developments
  • Lesions beneath the skin (in rare cases), may appear as painful inflammations
  • Frequently, the long bones of the legs (femur), arms (humerus), and the pelvic bones are affected. Other affected bones include the skull and shinbone (tibia)

How is Osteosarcoma arising in Paget's Disease of Bone Diagnosed?

A diagnosis of Osteosarcoma in Paget’s Disease of Bone is made using the following tools:

  • Physical examination, evaluation of patient’s medical history including history of bone Paget’s disease
  • Histopathological studies conducted on a biopsy specimen - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
  • X-ray studies of the tumor
  • MRI scan or CT scan of affected area

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 Osteosarcoma arising in Paget's Disease of Bone?

Complications of Osteosarcoma arising in Bone Paget’s Disease are dependent on the site and severity of the tumor. These include:

  • Damage of the following organs due to metastasis - liver, bones, and lungs
  • Usually by the time osteosarcomas are detected, chances are that they would have proliferated and metastasized aggressively, damaging organs and tissues beyond repair
  • This kind of osteosarcoma is known to having a high recurrence rate, even after its surgical excision and removal
  • Deep-seated tumors (those buried inside the body tissues) may cause damage to adjoining tissues and organs
  • Blood loss during invasive treatment methods may be heavy
  • Damage to vital nerves, blood vessels, and surrounding structures, during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy
  • Other complications due to Paget’s bone disease

How is Osteosarcoma arising in Paget's Disease of Bone Treated?

Treatment measures for Osteosarcoma arising in Paget’s Disease of Bone include the following:

  • Any combination of chemotherapy, radiation therapy, and invasive procedures, maybe used to treat the tumor
  • Wide surgical excision of osteosarcoma and removal of the entire lesion is the standard treatment mode. If the tumor is not fully removed, then it will recur
  • Embolization of the tumor is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
  • When the tumor is at an inaccessible location, or is unsafe for surgical intervention, non-invasive procedures are adopted
  • 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 Osteosarcoma arising in Paget's Disease of Bone be Prevented?

  • Current medical research have not established a way of preventing Paget’s Osteosarcoma occurrence
  • It is advised that the healthcare provider suspect osteosarcoma and start suitable work-up, in patients with bone Paget’s disease, when they start complaining of increasing bone pain
  • However, the presence of any tumor or lesion should be immediately informed to the physician and periodic follow-up and screening maintained
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor. This is due to both its high metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Osteosarcoma arising in Paget's Disease of Bone? (Outcomes/Resolutions)

  • The prognosis of Osteosarcoma arising in Paget's Disease of Bone is unknown

However, several prognostic markers are identified by research which helps determine the prognosis of Paget's Osteosarcoma. Many of these prognostic markers are based upon defective genes or defective proteins found in the tumor. Some of these genetic defects that alter the prognosis are explained below.

The following conditions result in or indicate a poor prognosis:

  • 13q14 loss of heterozygosity (gene loss) and mutations in Rb gene
  • Presence of MDR-1 gene mutation, which is a multi-drug resistant gene, results in tumor not responding to certain chemotherapy agents
  • The presence of c-FAS mutations in patients result in poorer prognosis, due to a poor response of the condition to chemotherapy and a higher chances of recurrent and metastatic osteosarcoma
  • HER-2 over expression in tumor
  • Loss of M-TAP gene

Other factors that are related to osteosarcoma prognosis include:

  • Loss of heterozygosity of 18q in patients with Paget’s disease of the bone, has been shown to increase the development of osteosarcoma, in such patients
  • Some studies seem to indicate that men with these bone tumors have poorer prognosis, than women; but, this has not been substantiated
  • Location of the tumor also determines its outcome; tumor in distal parts of the knee and elbow, have better outcomes, than those at other locations
  • The size of the tumor also determines its prognosis; normally, larger tumors have much poorer prognosis than smaller-sized tumors
  • Duration of symptoms are important factors too:
    • A shorter duration of symptoms lead to a worse prognosis
    • A longer duration of symptoms lead to a better prognosis
  • A presence of spontaneous necrosis leads to the worst prognosis. Spontaneous necrosis means a type of necrosis that is present due to tumor growth and not due to treatment with chemotherapy

Additional and Relevant Useful Information for Osteosarcoma arising in Paget's Disease of Bone:

There are approximately 1000 cases of osteosarcomas, diagnosed each year in the United States.

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: June 18, 2014
Last updated: Dec. 14, 2018