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FlowerGiant Cell Tumor
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What is a giant cell tumor?
A giant cell tumor is a benign solitary tumor that usually emerges in the ends (epiphyses and metaphyses) of long bones, and contains unusually large, multi-nucleated cells that are called giant cells.

These tumors most commonly occur in the femur (thighbone), tibia (shinbone) and distal radius (wrist.). Approximately 60 percent occur around the knee. It sometimes occurs in the sacrum (lower end of the vertebrae)

A giant cell tumor can be quite destructive locally, and has potential to break through the bone's outer layer (cortex), cause a fracture, known as a pathological fracture, and extend into the surrounding soft tissues.

This condition is considered benign, yet giant cell tumors can metastasize (spread) to the lungs, although this rarely happens . When it does occur, however, the prognosis for the patient is still good and occasionally the tumor regresses without intervention. The tumors in the lung have the same benign appearance under a microscope that the primary tumor does.

What causes a giant cell tumor and whom does it affect?
The cause of a giant cell tumor is unknown. Scientists do know that giant cells form when there is a proliferation of mononuclear cells that fuse together. It is unusual for this to happen prior to skeletal maturity when the growth of bones reaches full potential, although it occasionally does. It usually occurs between the ages of 20 and 40. It seems to affect more women than men.
What are the symptoms of a giant cell tumor?
The following are the most common symptoms in the affected area. (Each individual may experiences the symptoms of a giant cell tumor differently, depending on the location of the tumor.)
  • Pain
  • Decreased joint range of motion
  • A tender hard mass may be felt
  • Muscle atrophy
  • Elevated temperature of the overlying skin
The symptoms of a giant cell tumor may resemble other medical conditions or problems. Always consult a physician for a diagnosis.
How is a giant cell tumor diagnosed?
In addition to taking a complete medical history and performing a physical examination, tests used to diagnose a giant cell tumor include the following:
  • Biopsy of the tumor - a small tissue sample of the tumor is surgically removed and examined under a microscope. Because of the tumor's aggressive nature, it is crucial to rule out other malignant conditions that may resemble a giant cell tumor by observing the tumor's cellular makeup under a microscope. Biopsy also helps doctors to determine how aggressive the tumor is.
  • X-rays - a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. A chest x-ray should be obtained at diagnosis and periodically in follow up.
  • Magnetic Resonance Imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.
  • Computerized Tomography scan (also called CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices) both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat and organs. CT scans are more detailed that general x-rays. CT scans of the lungs are frequently obtained in patients with a giant cell tumor to look for tumors in the lung, although this rarely occurs.
  • Complete blood count (CBC) - a measurement of size, number and maturity of different blood cells in a specific volume of blood
  • Blood tests - (including blood chemistries)
Treatment for a giant cell tumor
Specific treatment for a giant cell tumor be determined by your child's physician based on:
  • Your child's age, overall health, and medical history
  • Extent of the disease
  • Proximity of the tumor to the growth plate
  • You or your child's tolerance for specific medications, procedures, or therapies
  • How your physician expects the disease may progress
  • Your opinion or preference
Treatment of a giant cell tumor may involve one or a combination of the following surgical procedures performed by a pediatric orthopaedic surgeon:

Curettage: This is the most common form of treating a giant cell tumor. Curettage is an operation during which the tumor is scraped out of the bone, usually with a special instrument called a curette that has a scoop, loop or ring at its tip. A powered instrument, called a burr, is also sometimes used in addition to the curette. For this procedure, surgeons make an incision in the bone to create a window to expose the entire tumor... The tumor is then completely curetted.

Cryotherapy: This may used to supplement curettage. Surgeons fill the cavity that remains after curettage with liguid nitrogen to freeze the area. The area is then thawed out and may be frozen again. The freeze and thaw cycle, which would help prevent any missed remnants of the tumor from growing, is repeated once or twice. This technique is associated with serious potential complications such as fracture and nerve injury and is not usually considered as primary treatment in most centers.

Bone Grafting or Cement Packing: Sometimes the remaining cavity is packed with donor bone tissue (allograft), bone chips taken from another bone (autograft), or other materials, including bone cement (the same material used in total joint replacements), depending on the preference of the surgeon.

En bloc resection: If more aggressive resection of the tumor is warranted, the operation will likely involve en bloc resection, which is the surgical removal of bone containing the tumor, rather than curettage. Internal fixation, with pins, may be required to restore the structural integrity of the bone. If significant bone loss results, allograft transplants or metallic replacements are used to reconstruct the defects. Although common in the past, these procedures are not commonly used as the initial treatment today.

What is the long-term prognosis for a patient with a giant cell tumor?
The long-term outlook for a patient with a giant cell tumor varies among individuals depending on:
  • The extent of the disease
  • The presence of absence of metastasis to the lungs
  • The size and location of the tumor
  • The tumor's response to therapy
  • The age and overall health of your child
  • Your child's tolerance for specific medication, procedures or therapies.
Prognosis and long-term survival can vary greatly from child to child. Every child is unique and treatment and prognosis is structured around the child's needs. Generally speaking, these tumors can be treated successfully and the long-term prognosis is good. These tumors can, however, recur. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with a giant cell tumor. Metastases to the lung is rare, but patients are monitored periodically with chest x-rays and sometimes CT scans to look for the occurrence of lung lesions.
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