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Paget's Disease

Paget’s disease is named after James Paget, the doctor who first described the disease. It affects bones, mostly of the spine, thigh, shin or pelvis. This disease affects the process of breaking down and rebuilding of bones. Since new bones are built at a faster rate, there is likelihood of fractures and deformities. Consequently there is destruction of bone since the new bone that is formed is weak and porous. As a result, these bones are likely to bend easily and break. Paget's disease also leads to additional blood vessels (hypervascularity in the affected area. This condition affecting the bones is more likely to affect men and people over 40. Paget's disease is also known as Osteitis Deformans. Over time, Paget's disease can lead to Osteoarthritis, Kidney Stones and heart disease. In rare cases, severe Paget's disease has been associated with heart failure or cancer.


The symptoms of Paget's Disease include pain and deep ache in the affected bones. The patient is likely to experience bone deformity or fracture in the affected area. There may be damage to the cartilage lining of the joints near the affected ones leading to osteoarthritis. Persons suffering from Paget's disease may suffer pain from nerve compression due to the pressure from bones. A person suffering from Paget's disease is likely to suffer elevated alkaline phosphate levels in the blood. There might be bone abnormalities such as enlargement, reabsorption and bowing.


Bone scans aid diagnosis of Paget's disease. Bone Scintiscan, a kind of imaging test where a Radio isotope like Iodine-131 meta-iodobenzylguanidine (MIBG) is used as a tracer, can help detect the bones that have been affected. X-rays and blood test to check blood serum alkaline phosphatase can help in the diagnosis of Paget's disease. Treatment for Paget's disease is based on the extent of the disease, location of the affected bones and the medical history of the patient. Physical therapy and medication to inhibit abnormal bone reabsorption are prescribed. Bisphosphonates can reduce the activity of Paget's disease and are often prescribed for a period of 2 - 6 months. NSAIDs are given to alleviate pain and reduce inflammation. Surgery to realign affected bones or joint replacement are resorted to in cases where the joints are severely damaged or deformed.


Bone Sarcoma

The word 'sarcoma' is derived from the Greek word for 'fleshy'. Sarcomas arise from Mesenchymal tissue which is the precursor to fibrous tissue, muscle, bone and fat. Bone sarcoma is malignant tumor or cancer of the bone. The difference between the terms sarcoma and carcinoma is that while sarcomas spread through blood, carcinomas through lymph fluid to the nearby lymph nodes.


Symptoms of bone sarcoma

  • Pain in the bones.
  • Pain may worsen in the nights.
  • Swelling, redness and tenderness at the site of bone pain.
  • Swelling of the leg or arm in longer bones such as above or below the knee or upper arm, shoulder etc.
  • Bone fracture after a routine movement – like throwing a ball.
  • As cancer weakens bones, broken bones tend to occur more easily.
  • Fatigue, fever and weight loss and anemia are other signs.

Causes of bone sarcoma

The cause is unknown in most cases although bone sarcoma can develop from benign lesions in the bone and areas that previously received radiation. Therefore persons who received radiation therapy or chemotherapy can be at increased risk. A genetic syndrome called Li Fraumeni and retinoblastoma and a disease called Paget's Disease are also associated with sarcoma.


Types of bone sarcoma

1. Osteosarcoma that occurs in the bones around the knee and upper arm.
2. Chondrosarcoma that begins at the cartilage and ends of bones and lines of joints and in pelvis, upper leg and shoulder.
3. The Ewing sarcoma family of tumors that occurs in bone but may also be present in the connective tissues located in the pelvis, legs and arms.
4. Multiple Myeloma which is a cancer of plasma cells that originates in bones.


Bone sarcoma diagnosis

A doctor examines the patient physically after ascertaining family medical history. Then the physician may recommend the following diagnostic tests:

  • X rays in various parts of the body to show location, size and shape of bone tumor.
  • Bone scans
  • CT scans which reveal cross sectional images to check if the tumor has spread.
  • MRI scans
  • PET scans that image the body's metabolic action to show if cancer has spread.
  • Biopsy

Treating Bone Sarcoma

Surgery plays a primary role in the treatment of bone sarcoma. The goal of surgery is twofold. On the one hand it can remove the cancerous tumors and restore the physical function and appearance. On the other hand, a cuff of normal tissue is taken from around the tumor cells to minimize the risk of tumor recurring at the local resection site.


After surgery, the reconstructive or plastic surgery team performs the delicate task of replacing bone. Musculoskeletal oncologists and reconstructive plastic surgeons use donor bone, the patient's own bone from other sites and metal implants to reconstruct limbs and restore function.

Radiation therapy: This can be performed before or after surgery or even during surgery though the use of brachytherapy. Radiation therapy is used to treat tumors or when there is disease recurrence at the site of the original tumor or other localized site. This is especially used in treating chondrosarcomas more often than bone sarcomas.

Chemotherapy: Chemotherapy is given before surgery in order to shrink the tumor and allow for better resection. Surgery and radiation can only act on a small area tumor site. Chemotherapy kills any cancer cells floating undetected elsewhere in the body.

Follow-up depends upon the grade and type of bone sarcoma. In case of Osteosarcoma and Chondrosarcoma, follow-up includes physical exam, chest x-ray which should be performed every three months for at least two years initially and then after four months for a year and then every six months for two years and subsequently annually.



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Bibliography / Reference

Collection of Pages - Last revised Date: November 15, 2019