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Bone Cancer (Sarcoma)

Types of Bone Cancer

Bone tumors vary widely, from benign bone tumors that can be cured by surgery to highly malignant cancers that need to be treated with multiple therapies. Sarcomas are not benign. Treatment for one sarcoma can include surgery, radiation and chemotherapy. The vast majority of these tumors can be treated with limb preservation, allowing you to remain functionally active throughout your life.

There are 3 main types of bone tumors:

  • Bone sarcoma: Cancer that originates from tissue within the bone.
  • Benign bone tumors: A lump or mass of abnormal cells that form within bone but are not cancerous.
  • Metastatic tumors: Cancer that started somewhere else in the body (such as the breast, prostate, kidney, or bone marrow), and spreads to the bone.

Our center has deep expertise in treating several specific types of bone cancers.

  • An aneurysmal bone cyst is a blood-filled, fibrous, tumor-like cyst that expands the bone, giving it a "blown out" appearance. It generally occurs in patients during their 20s and can emerge in any bone in the arms, legs, trunk or skull. They most commonly appear in the vertebrae and knee.

    An aneurysmal bone cyst is a benign bone tumor, meaning that it does not spread (metastasize) to other regions of the body. It can, however, be destructive locally and is very likely to recur.

    • Site: In a limb, tumors typically occur at the transitional zone where the shaft of the bone joins the end of the bone or the shaft of the bone near the growth plate. In the spine, tumors are likely to occur in the vertebrae.
    • Cause: Unknown. Tumors may emerge in response to disturbances in the bone's capillaries or may be related to chromosomal abnormalities.
    • Symptoms: Varied, but may include pain, swelling, a mass, mild to severe neurological problems or fractures.
    • Diagnosis: Determined by physical examination, medical history, X-ray, MRI, CT scan or bone scan and blood tests.
    • Treatment: Specific to the patient, considering age, overall health and medical history; location and extent of disease; tolerance to medications, procedures and therapies; expectation for progression; and family preference. Procedures may include curettage/bone grafting, excision of the host bone, biopsy, cryotherapy (surgical freezing procedure) or radiation therapy.
    • Outlook: Prompt medical attention, careful planning, aggressive therapy and continuous follow-up are crucial for the best outcome. This benign cyst can recur, often requiring multiple operations, but a cure is eventually obtained in almost all cases.
  • Chondroblastoma is a rare, benign tumor found in the long bones. It occurs most commonly in the femur followed by the humerus and tibia. This type of tumor affects men more often than women and tends to occur around age 20. Chondroblastoma may impact the lungs or invade bone and soft tissue.

    • Site: Long bones, both arms and legs.
    • Cause: Unknown. Chondroblastoma is believed to arise from the cartilaginous plates in bones near joints.
    • Symptoms: Pain near a joint with no history of trauma. Rarely, pathological fracture induced by the tumor.
    • Diagnosis: Determined by radiograph. Cysts – present about 20% of the time – can be identified by MRI and CT scan. CT also is useful for defining the relationship of the tumor to the joint.
    • Treatment: Biopsy and curettage with possible use of liquid nitrogen or phenol, or a mechanical burr. All pulmonary nodules must be surgically removed, and bone reconstruction may be necessary.
    • Outlook: Surgery typically provides a cure to the disease as long as the disease is confined. The outcome worsens if the disease has spread.
  • Chondromyxoid fibroma is a benign cartilage tumor. It accounts for less than 1% of all bone tumors. CMF is found most often around the knee in the tibia, fibula or femur. It most commonly occurs in children and young adults during their 20s or 30s. It impacts men nearly twice as often as women.

    • Site: In the bones of the leg, often around the knee, and also arms, feet, hands, fingers and toes.
    • Cause: Unknown.
    • Symptoms: Chronic pain, swelling, restricted movement and a palpable soft-tissue mass. Just 5% of CMF cases present with a fracture.
    • Diagnosis: Determined by CT scan and MRI, which are also integral to preoperative planning and staging. CMF is similar to chondrosarcoma, so radiology also often helps confirm diagnosis.
    • Treatment: Treatment requires surgery to remove the tumor.
    • Outlook: Recurrence after curettage is common.
  • Chondrosarcomas are the second most common primary malignant bone tumor. They most often arise within the shaft of the bone and can destroy the bone, produce fractures and develop soft-tissue masses. Chondrosarcomas are a group of tumors with diverse features and behavior patterns, ranging from slow-growing to very aggressive.

    In the United States, they account for about 20% of bone tumors and are diagnosed in approximately 600 people each year. Typically, they affect adults between the ages 20-50, and are most common in men.

    • Site: Pelvic bones, femur, humerus, ribs, scapula, sternum or spine.
    • Cause: Unknown.
    • Symptoms: Dull pain, often present for months. Local swelling. Restricted movement. Symptoms are often present for a year or more.
    • Diagnosis: Determined by a series of tests called staging studies. These include a review of signs and symptoms, physical exam and radiographic studies such as X-ray, MRI or bone scan. A CT scan of the lungs determines if the cancer has spread.
    • Treatment: Treatment is based on the tumor's grade, aggressiveness, location and other factors. Surgical removal, without chemotherapy or radiation therapy, affords long-term survival in more than 95% of cases.
    • Outlook: Prognosis is related to the size of the lesion, location and histologic grade. The 5-year survival rate for patients with grade 1 lesions is 90%; that rate decreases to 29% for those with grade 3 tumors. Tumor recurrence often occurs 5 to 10 years following surgery and often is more aggressive.
  • Eosinophilic granuloma is a localized lesion in a bone or the lung that occurs most commonly in children, adolescents and young adults. It makes up 60% to 80% of all cases of Langerhan's cell histiocytosis, and also can be found in the skull, jaw, spine and long bones. It affects about twice as many men as women.

    • Site: Skull, jaw, spine or long bones.
    • Cause: Unknown. May be either infection or immunological.
    • Symptoms: Local pain, swelling and tenderness.
    • Diagnosis: Determined most effectively by CT scan and MRI. The radiologic appearance of EG is nonspecific and differs by location. The skull might have a lesion with sharp, punched-out borders that are uneven across the inner and outer table, causing a beveled edge. Pelvic lesions are often poorly defined. Spine lesions are normally found in the vertebral body.
    • Treatment: Treatment depends on the form of the disease. With localized disease, often a biopsy alone is enough to incite healing. Other treatment options include curettage, excision, steroid injection, radiation and observation. Chemotherapy is recommended for systemic disease.
    • Outlook: Recurrence is possible. Prognosis is more favorable when disease is localized.
  • A malignant primary bone tumor derived from red bone marrow, Ewing sarcoma is usually discovered in children and adolescents between ages 4 and 15. Ewing sarcoma accounts for about 5% of all biopsy-analyzed bone tumors. It is the second most common and the most lethal malignant bone tumor in young patients.

    The condition affects males more often than females. In the United States, occurrence ranges from 0.3 cases per 1 million in children under 3 to as high as 4.6 cases per 1 million in adolescents aged 15 to 19.

    • Site: Most commonly found in the long bones of the extremities and less frequently in the pelvic area, ribs or scapulae, but may occur in any bone of the body.
    • Cause: Genetic mutation of unknown cause.
    • Symptoms: Intermittent pain that intensifies. Fever, mild anemia or weight loss. Eventually, most patients have a large, palpable mass with tender local swelling.
    • Diagnosis: Determined by conventional radiograph and/or MRI examination.
    • Treatment: Standard treatment may include chemotherapy, radiation therapy, surgery, targeted therapy and high-dose chemotherapy with stem cells.
    • Outlook: The 5-year survival rate is 78% for children younger than 15 and 60% for adolescents aged 15 to 19.
  • Osteosarcoma is the most common primary sarcoma of bone, accounting for nearly 80% of all osteosarcoma diagnoses. Osteosarcomas are most frequently diagnosed in children and young adults whose bones are still growing.

    • Site: Long bones, both arms and legs.
    • Cause: Unknown, but research suggests inherited genetic mutations, underlying bone abnormalities (Paget’s disease of the bone and adolescent growth spirts) and prior radiation treatment that damaged healthy bone cells.
    • Symptoms: Bone pain or tenderness, a mass that can be felt through the skin, swelling and redness at the tumor site, bone injury or a break for no apparent reason.
    • Diagnosis: Physical exam, biopsy and imaging tests including bone X-ray, MRI scan, CT scan, PET scan.
    • Treatment: Surgery, including limb salvage resection to remove the tumor without removing the whole limb; rotationplasty to treat bone tumors in children that occur near the knee; chemotherapy, radiation therapy and amputation.
    • Outlook: Long-term survival rate is 76% with prompt treatment.

Bone metastasis

In addition to the types listed above, bone cancer can also develop as metastatic disease, meaning that a different primary type of cancer spreads to the bone tissue. For example, a patient with metastatic breast cancer may require care for breast cancer that spreads to the bone. Other types of cancer that can spread to the bones include prostate cancer, lung cancer, thyroid cancer, myeloma and kidney cancer. Breast and prostate cancer are associated with the majority of bone metastases.

In adults, bone metastases (secondary bone cancers) are more common than primary cancers of the bone. Metastasis in the bone usually means that the primary cancer has spread. When this happens, treatment is more difficult and curing the primary cancer is rare. However, treating the bone metastases and the primary cancer can still lead to slower growth of cancerous cells.

New cancer therapies are helping patients with bone cancer metastases live longer than ever. For this reason, surgery for metastatic disease must be done carefully to minimize complications and ensure a good quality of life.

At The University of Kansas Cancer Center, our expertise in caring for patients with bone metastasis is unmatched. We perform thorough, thoughtful reconstructive surgeries and use customized prostheses that are tailored to your needs and will last many years. We treat metastatic cancer as a chronic disease with an emphasis on improving your quality of life by alleviating pain and preventing complications that could interrupt your cancer care. Our orthopedic surgeons are fully integrated into our cancer center, which is 1 of 71 NCI-designated cancer centers in the country.

Growing prosthetic bone for Ewing sarcoma patient

Alex Goodwin traveled from England to receive lifesaving treatment at The University of Kansas Cancer Center’s Sarcoma Center. Alex suffered from Ewing sarcoma and was given only months to live. His parents refused to give up and sought treatment in Kansas City. Here, Howard Rosenthal, MD, removed Alex’s diseased femur, hip and knee joints and replaced them with a growing prosthetic bone.

Start your path today.

Your journey to health starts here. Call 913-588-1227 or request an appointment at The University of Kansas Cancer Center.

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