Bone Cancer Treatment in Germany

Multiple myeloma

Multiple myeloma is considered the most common primary malignant tumour of bone, but it is often regarded as a tumour of bone marrow cells located within bone rather than as a bone tumour proper. It is of haematopoietic origin and occurs mainly in elderly people. The tumour usually develops and progresses as a multifocal lesion and often involves the bone marrow so diffusely that marrow aspiration is of diagnostic value. Imaging reveals clearly demarcated lytic changes or diffuse demineralisation of bone tissue. Sclerotic changes or diffuse osteopenia are less common, particularly in the vertebral bodies. An isolated solitary myeloma without systemic bone marrow involvement is termed a plasmacytoma. Some bone lesions respond well to radiotherapy.

Osteosarcoma

Osteosarcoma, or osteogenic sarcoma, is the second most common primary malignant bone tumour and is highly malignant. It most often occurs between the ages of 10 and 25, although it can develop at any age. Osteosarcoma gives rise to malignant osteoid (immature bone) produced by tumour bone cells. It usually develops around the knee joint (more often in the distal femur than in the proximal tibia) or in other long bones, and it can metastasise, usually to other bones or to the lungs. The usual symptoms of osteosarcoma are pain and swelling.

Imaging may reveal sclerotic or lytic changes in the tissue. A biopsy is mandatory for the diagnosis. Chest radiography or CT is also required in order to detect metastatic lung involvement, together with scintigraphy to identify possible bone metastases.

Treatment consists of surgery combined with chemotherapy. The use of adjuvant chemotherapy raises the 5-year survival rate from 20% to more than 65%. Chemotherapy is usually started before surgery. A reduction in tumour size on the radiograph, a reduction in pain and a fall in the serum alkaline phosphatase level indicate that treatment is effective; preoperative chemotherapy should achieve more than 95% tumour necrosis. After several cycles of chemotherapy, limb-sparing removal of the tumour and reconstruction of the limb are performed. The tumour is removed together with all the reactively altered tissue around it and with a margin of surrounding healthy tissue, in order to prevent further spread of tumour cells. In more than 85% of patients, limb-sparing surgery can be performed without reducing long-term survival. Chemotherapy must be continued after the operation. If almost complete tumour necrosis (about 95%) is achieved before surgery, the 5-year survival rate is more than 90%.

Far rarer variants of osteosarcoma include such superficial cortical lesions as parosteal osteosarcoma and periosteal osteosarcoma. Parosteal osteosarcomas most often affect the posterior cortex of the distal femur and are usually well differentiated. Periosteal osteosarcoma is a cartilaginous surface tumour. It is often located in the mid-shaft of the femur and has a sunburst appearance on the radiograph. Periosteal osteosarcoma is far more likely to metastasise than well-differentiated parosteal osteosarcoma, but somewhat less likely than typical osteosarcoma. Parosteal osteosarcomas require surgical removal without chemotherapy. Periosteal osteosarcoma is treated in the same way as conventional osteosarcoma: chemotherapy and removal of the tumour.

Fibrosarcoma

Fibrosarcomas are similar in their characteristics to osteosarcomas, but they produce fibrous tumour cells; they affect the same age group and pose similar problems. Treatment and outcome in advanced disease are similar to those in osteosarcoma.

Malignant fibrous histiocytoma

Malignant fibrous histiocytoma clinically resembles osteosarcoma and fibrosarcoma, although it is classified as a disease outside the osteosarcoma group because it has a different histological picture. It tends to affect children and adolescents more often, but it can also occur in elderly people as secondary lesions at sites of bone infarcts and irradiated areas. The principles of treatment and the outcomes are similar to those described for osteosarcoma.

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Chondrosarcoma

Chondrosarcomas are malignant tumours of cartilage tissue. They differ from osteosarcomas both in their clinical presentation and in treatment strategy and prognosis. 90% of all chondrosarcomas are primary. Chondrosarcomas develop against the background of other conditions, such as multiple osteochondroma and multiple enchondromatosis. Chondrosarcomas appear more often in elderly people. The tumour arises most often in older people in the flat bones of the pelvis and in the scapula, but it can affect virtually any bone and the surrounding soft tissues.

Radiography often reveals fine focal calcification. Primary chondrosarcomas frequently present with destruction of the bone cortex and loss of its structure. Secondary chondrosarcoma may be suspected when punctate calcifications are found or when an osteochondroma increases in size. Scintigraphy with 99mTc refines the results of the screening examination: all areas of cartilage pathology show increased isotope uptake, but chondrosarcomas show particularly high uptake. A biopsy is mandatory; it makes it possible to determine the stage of the tumour and the likelihood that it will metastasise.

In early-stage tumours, tissue-sparing operations (wide curettage) with adjuvant therapy are performed more often (freezing with liquid nitrogen, the heat of methyl methacrylate, radiofrequency ablation or phenol are commonly used). For other tumours, total resection is used. If resection with preservation of function is not possible, amputation is performed. During surgery and biopsy, trauma to the tumour and seeding of its cells into the soft tissues of the surgical wound must be avoided in order to prevent tumour implantation. Otherwise recurrence is inevitable. If no seeding occurs, the cure rate is determined by the stage of the tumour. With adequate treatment at an early stage, patients are almost always cured. These tumours have limited vascularisation (the formation and growth of blood vessels), so chemotherapy and radiotherapy are largely ineffective.

Ewing sarcoma of bone

Ewing sarcoma of bone is a round cell bone tumour that most often develops in patients between the ages of 10 and 20. It mainly affects the bones of the limbs, although it can involve other bones as well. Ewing sarcoma tends to spread extensively and sometimes involves the whole bone. The most common symptoms are pain and swelling.

Lytic destruction, and especially invasive infiltrating growth without clear borders, is the most common sign found on imaging, but the formation of multiple layers of reactive new bone may create an onion-skin appearance. Radiography usually does not reliably show the extent of the disease, and the affected bone is normally surrounded by large soft-tissue masses. The margins of the tumour can be defined more precisely with MRI, which helps in choosing the optimal treatment. The diagnosis must also be based on biopsy findings, since a similar picture is seen in many other benign and malignant bone tumours. In some cases this type of tumour can be mistaken for an infection. Accurate histological diagnosis can be supplemented by testing for molecular markers, including assessment of the typical clonal chromosomal abnormalities.

Treatment involves various combinations of surgery, chemotherapy and radiotherapy. At present more than 60% of patients with primary localised Ewing sarcoma  are cured with this combined approach. In some cases cure is possible even in the presence of metastases. Chemotherapy combined with resection of the tumour often gives favourable long-term results.

Lymphoma of bone

Lymphoma of bone occurs in adults, usually between the ages of 40 and 50. It can arise in any bone. The tumour consists of small round cells, often mixed with reticular cells, lymphoblasts and lymphocytes. It may develop as a primary bone tumour, in combination with similar tumours in other tissues, or as a metastasis of a known lymphoproliferative disease. The usual symptoms are pain and swelling. Pathological bone fractures are not uncommon.

Imaging reveals signs of bone destruction, which may be patchy, infiltrating or invasive in character, often together with large soft-tissue masses. In advanced cases the outer contour of the affected bone sometimes disappears almost completely.

In isolated primary lymphoma of bone, the 5-year survival rate is 50% or more. A combination of radiotherapy and chemotherapy is comparable in effectiveness to removal of the tumour and other surgical procedures. Stabilisation of the long bones is often required to prevent a pathological fracture. Amputation is indicated in the rare cases where limb function has been lost because of a pathological fracture, or where extensive soft-tissue involvement cannot be dealt with in any other way.

Malignant giant cell tumour

Malignant giant cell tumour is rare and is usually located at the very end of a long bone. Radiographs show the classic signs of malignant bone destruction (predominantly lytic changes, destruction of the cortex, spread of the process into the surrounding soft tissues and pathological fractures). A malignant giant cell tumour that has developed from a pre-existing benign giant cell tumour is characteristically resistant to radiotherapy. Treatment follows the same principles as for osteosarcoma, but the results of therapy are poorer.

Chordoma

Chordoma is a rare tumour that develops from remnants of the embryonic notochord. It is most often located at the ends of the spine, usually in the sacrum or near the base of the skull. A chordoma of the sacrococcygeal region causes almost constant pain. A chordoma at the base of the skull may produce symptoms of cranial nerve involvement, most often of the oculomotor nerves.

Several months or even years usually pass before the correct diagnosis is made. On imaging, a chordoma appears as extensive destructive bone changes, which may be accompanied by a soft-tissue component. Metastasis is extremely rare, but there is a tendency to local recurrence. If the tumour is located in the sacrococcygeal region, radical removal is often possible. Chordomas of the skull base are usually inaccessible for removal, but they respond well to radiotherapy.