Bone Sarcomas

Introduction

The other category of sarcoma is bone cancer. It is very rare, with approximately 3,000 new cases diagnosed in the United States each year. The incidence is slightly higher in males than females. No race has a higher incidence than another, although Ewing’s sarcoma is particularly rare among African and American blacks. Due to the rarity and severity of bone cancer, a bone cancer specialist such as an orthopaedic oncologist should be consulted in the treatment of this disease. Visit the Musculoskeletal Tumor Society to find a sarcoma surgeon.

Bones consist of three types of tissue: compact tissue, the hard outer portion of the bone; cancellous tissue, the spongy tissue inside the bone containing the bone marrow; and subchondral tissue, the smooth bone tissue of the joints. Cartilage surrounds the subchondral tissue to form a cushion around the joints.

Bone tumors can be benign (non-cancerous) or malignant (cancerous). Benign bone tumors are rarely life threatening, and do not spread within the body; however, they can grow and compress healthy bone tissue. Cancer that develops in the bone is called primary bone cancer. It is differentiated from secondary bone cancer, which spreads to the bone from another part of the body.

Scientists are uncertain about what causes bone cancer, but they have been able to identify certain factors that may put a person at risk. Children and young adults who have undergone radiation therapy or chemotherapy for other diseases are at an increased risk for bone cancer. Additionally, adults with Paget's disease, which is a disease characterized by abnormal growth of new bone cells, have an increased risk of osteosarcoma. There are also some hereditary conditions that can increase the risk of bone cancer.

Symptoms of bone cancer can vary depending on the size and location of the tumor. Pain is the most common symptom. Tumors arising in or around the joints often cause swelling and tenderness. Tumors also can weaken the bones, causing fractures. Other symptoms can include weight loss, fatigue, and anemia.

The first step in diagnosing primary bone cancer is a complete medical history and a physical examination performed by a physician. The doctor may order a blood test to determine the level of an enzyme called alkaline phosphatase. Approximately 55% of patients with primary bone cancer will have elevated levels of alkaline phosphatase. However, it isn't a completely reliable indicator for bone cancer, because growing bones in children will also cause the enzyme to be elevated.

X-rays are also used to locate a bone tumor. If an x-ray suggests that a bone tumor is present, a doctor may order further testing, such as a CT scan, Magnetic Resonance Imaging (MRI), or a bone scan. Finally, a biopsy must be performed to determine if cancer is present.

A biopsy is a procedure used to remove sample tissue from the tumor. A surgeon, usually an orthopaedic oncologist, performs either a needle biopsy or an incisional biopsy. During a needle biopsy, the surgeon makes a small hole in the bone and removes sample tissue with a small instrument. For an incisional biopsy, the surgeon cuts into the tumor and removes sample tissue. Certain tumors are can be appropriately biopsied by a radiologist using imaging guidance with a CT scan or under ultrasound. A pathologist—a doctor specializing in identifying disease—will then study the cells and tissues under a microscope to determine whether the tumor is cancerous.

The treatment of bone cancer depends on the size, location, type, and stage of the cancer. Surgery is often the primary treatment. While amputation of a limb is sometimes necessary, using chemotherapy either before or after surgery has allowed physicians to save the limb in many cases. Radiation is also used sometimes, in combination with the other treatments.

New and more effective treatments are being developed in clinical trials at many sarcoma centers and cancer centers. The most common types of bone sarcomas are described below.

Adamantinoma

Adamantinoma of the long bones, or extragnathic adamantinoma, is an extremely rare, slow growing, low-grade malignant tumor of epithelial origin, that occurs almost exclusively in the tibia and the fibula.

The tumor usually occurs in the second to fifth decade of life.
The patient usually has swelling that may be painful. The duration of symptoms can vary from a few weeks to years.

Adamantinoma is treated by wide surgical excision

Angiosarcoma

Angiosarcoma is an aggressive sarcoma of bone that is most common in the femur, tibia, and pelvis. It is a rare form of bone sarcoma. This tumor may arise in association with a bone infarct or Paget's disease.

High grade angiosarcoma seems to have two distinct clinical presentations. First, the lesion can present as multiple lesions in a single bone, two or more adjacent bones, or perhaps all the bones of a limb. These lesions seem to have an slow-going course and the prognosis remains good. The second presentation is that of single or multiple rapidly progressive lesions that metastasize to other bones or to the lung this form of the disease has a very poor prognosis.
Patients present with increasingly severe bone pain. Treatment involves wide surgical resection and secondary therapies..

Chondrosarcoma

This is a cancer of that usually grows within a bone or on its surface. It is most typically found in middle-aged adults. Chondrosarcoma is usually a slow-growing tumor and the most common sites are the pelvis, shoulder, and the upper part of the arms and legs.

The treatment for chondrosarcoma relies heavily on surgical removal, as there is usually no response to radiation and/or chemotherapy. One of the reasons for aggressive treatment is that 10% of low-grade tumors have been reported to develop a much more aggressive biologic behavior, a process called dedifferentiation.

Chordoma

affects bones in the spine and the base of the skull. This type of bone cancer occurs most frequently in adults 30 or older, particularly men. It tends to be a slow growing tumor with a low risk of spreading to distant sites, but may return at the original site if not removed completely during surgery, and may eventually spread to the lungs, liver or lymph nodes.

Clear Cell Chondrosarcoma

Clear Cell Chondrosarcoma is a destructive low-grade malignant tumor which presents in adults. Clear cell chondrosarcoma is uncommon and accounts for about 2% of all chondrosarcomas.

This tumor appears to occur more often in men than in women. Adults in their 30,s, 40's and 50's are mot commonly affected, but older and younger patients have been reported. The most common sites of involvement are the proximal femur, proximal humerus, distal femur and proximal tibia.
The most common symptoms are pain and swelling, that usually are of long duration. There may be limitation of the range of motion of the involved joint. En bloc resection with a wide margin of normal bone and soft tissue is the procedure of choice.

Ewing’s Sarcoma Family of Tumors (EWST)

Ewing’s Sarcoma Family of Tumors (EWST) begins in the soft tissues (extraosseous Ewing’s sarcoma). Doctors do not know the exact cell of origin, but it is thought to be neuroectodermal. Recently, doctors have defined the disease to include a family of tumors, which includes Ewing’s sarcoma of bone or soft tissue, primitive neuroectodermal tumor (PNET), and Askin tumor of the chest wall. Sometimes these tumors are called small blue cell (round cell) tumors, because of their appearance under a microscope.

Compared with other cancers, all malignant bone tumors are rare. Among malignant bone tumors in children and young adults, Ewing’s sarcoma is the second most common. It occurs in 1.7 per million to 2.7 per million in children younger than 15 years old. There is no known cause.

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Note: This section is used with permission from the author, Edward Y. Cheng, M.D., Mairs Family Professor, Department of Orthopaedic Surgery, University of Minnesota Cancer Center, and a charter KWRISFBoard member. It was originally in the Journal of the American Academy of Orthopaedic Surgeons, October 2004. The full article, including figures, can be found here.

Fibrosarcoma of Bone

This type of bone cancer is also most commonly found in adults, particularly during middle age. A typical site is the thighbone. Fibrosarcoma cancers arise in the fibroblast-type cells— any cells from which connective tissue is developed in the body. These cells form scars as well as perform other important connective functions. This sarcoma contains much connective tissue.

Malignant Fibrous Histiocytoma/Undifferentiated Pleomorphic Sarcoma

This is a very rare type of bone cancer that occurs in adults. It is usually found in the arms and legs, especially around the knee joints. 

Mesenchymal Chondrosarcoma

This rare form of chondrosarcoma is a deadly high grade sarcoma consisting of primitive mesenchymal cells and islands of cartilage which occurs most often in the femur, ribs, jaw, skull, and spine, and may occur in any bone.

Most commonly affects people under 40, and may be from 10 to 70. Males and females are equally affected.
Patients present with pain and a mass.

Complete wide resection, with aggressively wide margins. Long-term follow up is needed. Chemotherapy and radiation are used when complete resection is not possible, but the role of chemotherapy is not well defined. Multi-modal treatment may be considered in some centers. Chemotherapy appears to be linked to better survival.

Osteosarcoma (Osteogenic Sarcoma)

Most cancers in the bone have spread or metastasized to the bone from another part of the body such as the lung, breast, or kidney. These areas of origin are considered the “primary” sites. Bone cancers may also be related to a cancerous tumor of blood cells, as in myeloma. On rare occasion, however, a cancer can begin in the bone itself from cancerous cells that make bone tissue. A malignant tumor that forms bone tissue from cancerous cells is called an osteosarcoma. Although cancers that begin in the bone (primary bone cancer) are much less common than those that metastasize to the bone, among the primary bone cancers, osteosarcoma is the most common type.

 

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Note: This section is used with permission from the author, Edward Y. Cheng, M.D., Mairs Family Professor, Department of Orthopaedic Surgery, University of Minnesota Cancer Center, and a charter RISF Board member. It is based on an article entitled: “Osteosarcoma, An unusual cancer arising in the bone.”

Paget's Sarcoma

Paget's disease of bone is characterized by an excessive and abnormal remodeling of bone. Paget's sarcoma is a rare form of osteosarcoma that occurs in about 5% of patients with advanced polyostotic Paget's disease. The most common sites of malignant transformation are the femur, pelvis, humerus and craniofacial bones in that order. Other tumors that occur in Paget's disease include malignant fibrous hystiocytomas and chondrosarcomas.
Most patients are 55-80 years old.Patients often complain of pain before the tumor can be easily seen on a plain radiography and the diagnosis can often be delayed. If one Paget's sarcoma is found, the entire body should be surveyed as the tumor often arises in more than one site.
Treatment for Paget's sarcoma depends on the tumor stage and the general status of the patient, and can vary from wide resection and chemotherapy to palliative radiation for pain control.

Periosteal Chondrosarcoma

Periosteal chondroma is a rare benign surface lesion composed of cartilage. It is similar in appearance and location to periosteal osteosarcoma. The potential for confusion with periosteal and even parosteal osteosarcoma mandates a thorough investigation and biopsy of these lesions

This tumor may occur in both children and adults. Males are affected 2:1 over females. Common sites include the humerus, femur, and fingers and toes. Patients complain of a tender swelling or mass.Symptoms are present for 1 to 5 years.

Simple excision of this tumor usually results in a cure.

References:

  1. American Academy of Orthopaedic Surgeons. (Nov. 2014).Soft Tissue Sarcomas. Retrieved on January 28, 2016 at http://www.orthoinfo.org/topic.cfm?topic=A00508
  2. American Cancer Society. (2015). What is a soft tissue sarcoma? Retrieved on January 28, 2016 at http://www.cancer.org/cancer/sarcoma-adultsofttissuecancer/detailedguide/sarcoma-adult-soft-tissue-cancer-soft-tissue-sarcoma
  3. Amschwand Sarcoma Cancer Foundation
  4. Cancer Information Network. Aug. 2000.
  5. Cheng, Edward Y.D., Maheshwari, Aditya V. MD. Ewing’s Sarcoma Family of Tumors (EWST). Journal of the American Academy of Orthopedic Surgeons, February 2010. http://journals.lww.com/jaaos/Fulltext/2010/02000/Ewing_Sarcoma_Family_of_Tumors.4.aspx 
  6. Cheng, M.D., Edward Y., Department of Orthopaedic Surgery, University of Minnesota Masonic Cancer Center Newsletter, Summer 2008.
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  9. The Doctor’s Doctor Web site
  10. The Sarcoma Alliance Retrieved January 28, 2016 from http://sarcomaalliance.org/what-you-need-to-know/bone-sarcoma/
  11. Yasko M.D., Alan W. and Somlo M.D., George. "Bone Sarcomas." Cancer Management: A Multidisciplinary Approach. Ed. PRR Inc. Aug. 2000. Cancer Information Network. Aug. 2000.