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Musculoskeletal Oncology
Timothy Rapp, MD The Musculoskeletal Oncology Service provides evaluation care and treatment for patients with benign as well as malignant tumors of the bones, muscles and other soft tissues of the spine and extremities.
Timothy Rapp, MD
Patient Information:
Adult and Pediatric Musculoskeletal Oncology

Frequently Asked Questions (FAQ):

[ LINKS to the additional musculoskeletal oncology information ]

Metastatic Bone Disease [ return to top ]
It is an understatement to say that cancer is a common problem in our society. It has likely touched everyone’s life in some way. According to the American Cancer Society in 2000, there are currently 8.2 million Americans alive with cancer. In the year 2000 there were 1.2 million new cases diagnosed. Close to 600,000 deaths occur annually due to cancer related causes.

In regards to metastatic disease (cancer that has spread to other areas of the body), the skeletal system is the third-most common organ system involved. The lungs and liver are the commonest. An important fact to consider is that 20% of patients who are diagnosed with cancer will develop symptomatic metastatic bone disease. Not surprisingly, the most common primary tumors that metastasize to bone are those that are most common in society; cancer of the prostate, breast, lung, and kidney. Therefore in 2000, there were approximately 250,000 patients who developed a skeletal metastasis. Furthermore, around 40,000 people suffered a fracture of their bone due to the spread of their primary cancer.

The role of the orthopaedic surgeon is to evaluate and recommend treatment approaches for patients with cancer and bone pain. Certainly not all body aches and pains in cancer patients are caused from spread of disease to the bone, yet it is the job of the orthopaedic surgeon to evaluate patients for this possibility. Certainly, it is also important to keep in mind as orthopaedic surgeons that 15% of these patients who develop skeletal metastasis will sustain a pathologic fracture secondary to their tumor. Once a cancer has spread to the bone there are a number or potential treatment options. These options are dictated by the type of primary cancer (breast, prostate, etc) the location of the lesion in the skeleton and the size of the tumor. Non-operative treatment options include external beam radiation therapy and possibly chemotherapy. Operative intervention is at times recommended to treat fractures of the bone or to prevent fractures in people whose tumors are causing significant destruction of the bones integrity.

Survival estimates for cancer that has spread to bone vary by the aggressiveness and type of primary tumor. On average, the five-year survival rate for patients with prostate or breast cancers that has spread to bone is quite good, near 90%. Patients with metastatic lung cancer to the bone have a relatively poor prognosis, with a 10% five year survival. Five year survival for metastatic renal cell carcinoma averages 50%. When reading these numbers it is important to keep in mind they are averages,m and ultiple variables specific to each patient’s case can affect each individual’s prognosis.

What is a bone tumor? [ return to top ]
A bone tumor is an overgrowth of cells within the bone. These cells could arise from cells normally present in the bone or other times can arise from the proliferation of cells from other sites of the body. For reasons that aren’t well understood, these cells grow abnormally and form a “tumor”. One very important question to ask regarding a bone tumor is whether it is benign or malignant.

What does benign mean? [ return to top ]
A benign tumor by definition is not life threatening. Nonetheless, many benign bone tumors can be aggressive and cause problems to the affected bone and surrounding joint. These effects and may cause significant long-term effects on the function of the affected limb. Benign bone tumors may also cause the bone to constantly ache and may weaken it to the point that it breaks. Other benign bone tumors are “incidental findings”, or are discovered on an x-ray taken for an unrelated problem. Many of these benign bone tumors do not cause symptoms, do not grow, and need no formal treatment. There is a long list of benign bone tumors listed below (table).

Benign “Non-Aggressive” Bone Tumors Benign “Aggressive” Bone Tumors
Osteoid osteoma
Unicameral (simple) bone cyst
Hemangioma of bone
Intaosseous ganglion cyst
Osteochondroma
Exostoses
Enchondroma
Non-ossifying fibroma
Chondomyxoid Fibroma
Periosteal-Cortical Desmoid		 Fibrous Dysplasia
Giant Cell Tumor
Osteoblastoma
Chondroblastoma
Anuerysmal Bone

What does malignant mean? [ return to top ]
A malignant tumor means the tumor has the potential to spread from its original location, in this case the bone, to other body sites. The most common location of spread is the lung. For this reason a malignant bone tumor is potentially life threatening. The most common malignant tumor of bone is metastatic adenocarcinoma, or a tumor that has spread to the bone from another primary site (e.g. breast, prostate, lung). The most common primary malignant bone tumors are osteosarcoma, Ewing’s sarcoma, chondrosarcoma, multiple myeloma, and lymphoma.

What causes a malignant bone tumor? [ return to top ]
We do not know. Currently there are many scientific studies taking place to attempt to answer this question. Many people theorize that they arise from alterations in the DNA of one's bone cells that cause the cells to grow abnormally. However, this is yet to be definitively proven.

Do malignant bone tumors run in families? [ return to top ]
Not to our knowledge. Many patients with malignant bone tumors have been followed over long periods of time and there has been no evidence that these people's children or family members are at a significant increased risk of a malignant bone tumor versus that of the general population.

What type of x-rays, MRI, or other tests do I need? [ return to top ]
Bone tumors are usually identified with x-rays or MRI’s. Depending on the patient’s symptoms radiographic imaging of some type has been ordered to identify the potential problem. After evaluating these images, the medical team will make a determination of what further imaging is needed to best evaluate your tumor. Depending upon the size, location, and nature of the tumor that may require CT scans, MRI scans, bone scans, PET scans, or a combination thereof to best define the abnormality.

What can be expected from a bone biopsy? [ return to top ]
A bone biopsy is done in the operating room where the orthopaedic surgeon removes a sample of the bone for analysis. The biopsy can cause mild pain, but usually can be done in the outpatient setting.

When will I know the results of the biopsy? [ return to top ]
The worst part of the biopsy for most patients is the one to two weeks time it takes to get the sample analyzed. In order for the pathologists to be able to section (cut) the tissue into small enough pieces to view under the microscope, the bone must be decalcified. Depending on the “hardness” of the bone, this process can take up to a week. Once the specimen is decalcified a number of special tests can be run on the sample to help make a proper diagnosis. This entire process can take as long as 2 weeks.

How is the diagnosis definitively made? [ return to top ]
The final diagnosis is made only after all of the necessary imaging tests have been completed and a biopsy of the tumor has been performed. When all of these “pieces” of the information are completed and analyzed as one larger picture, the final diagnosis is determined and a treatment plan is formulated.

What treatment options are there? [ return to top ]
The type of tumor and its location dictates the treatment plan. The most common treatment plans generally involve a combination of chemotherapy, surgery, and radiation therapy.

In general, patients with malignant bone tumors who are otherwise healthy receive chemotherapy. Although the chemotherapy can be quite strong, it has been shown to significantly improve the survival of patients with particular types of bone cancer. The goals of the chemotherapy are to shrink the tumor (making removing it easier) and to prevent it’s spreading to other areas of the body. The chemotherapeutic medications are administered and monitored by a medical oncologist who works closely with the surgical specialists. The most common drugs administered (adriamycin, ifosfamide, and cisplatin) can cause significant side effects. The most common side effects are hair loss, nausea, loss of appetite and energy, hearing loss and effects on the heart.

After administration of chemotherapy, surgery is performed to remove the diseased tissue. The technique of limb-salvage surgery has significantly advanced in the past twenty years. In these types of operations, the diseased bone is removed and, the bone deficit is reconstructed either with a prosthetic joint (joint replacement) or with a bone transplant. These types of surgeries are technically complicated, and are generally performed by orthopaedic surgeons with specialized training in that area. Although the majority of patients enjoy good function of their extremity after these procedures, there are significant risks and the potential need for multiple revision surgeries in the future.
In certain circumstances where the tumor is too large or if it involves critical nerves and blood vessels to the extremity an amputation of the limb may be necessary. Luckily, with the advancements in chemotherapy and surgical technique, these situations are uncommon in the current day.

Certain malignant bone tumors are sensitive to radiation therapy. However the most common bone tumor, osteosarcoma, is not. Depending on the diagnosis and the ability of the surgeon to remove the tumor, radiation therapy in may be used after surgery as an “insurance policy” to lower the risk of local tumor recurrence. Radiation therapy is generally administered as an outpatient in fractions, or partial doses given every weekday for 4-6 weeks. Although generally considered painless, there are side effects to radiation. The most common are skin changes, and chronic swelling and weakness of the extremity.

Links

Information from the American Cancer Society web site:

Information from the American Academy of Orthopaedic Surgery web site: Bone Tumor Fact Sheet

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