PRINCIPLES OF MANAGEMENT For all but the simplest and most obvious of benign tumours, management calls for

a multidisciplinary. Team approach and is best conducted in a tertiary centre specializing in the treatment of bone and soft-tissue tumours. Consultation and cooperation between the orthopaedic surgeon, radiologist, patho -logist and (certainly in the case of malignant tumours) the oncologist is essential in the initial management. In many cases physiotherapists, occupational thera-pists and prosthetists will also be involved. Once clinical and radiological examination have suggested the most likely diagnosis, further manage-ment proceeds as follows. Benign, asymptomatic lesions If the diagnosis is beyond doubt (e.g. a non-ossifying fibroma or a small osteo-chondroma) one can afford to temporize; treatment may never be needed. However, if the appearances are not pathognomonic, a biopsy is advisable and this may take the form of excision or curettage of the lesion . Benign, symptomatic or enlarging tumours Painful lesions, or tumours that continue to enlarge after the end of normal bone growth, require biopsy and con-firmation of the diagnosis. Unless they are unusually aggressive, they can generally be removed by local (marginal) excision or (in the case of benign cysts) by curettage. Suspected malignant tumours If the lesion is thought to be a primary malignant tumour, the patient is admitted for more detailed examination, blood tests, chest x-ray, further imaging (including pulmonary CT) and biopsy. This should allow a firm diagnosis and staging to be established. The various treatment options can then be discussed with the patient (or the parents, in the case of a young child). A choice needs to be made between amputation, limb-sparing opera-tions and different types of adjuvant therapy, and the patient must be fully informed about the pros and cons of each. METHODS OF TREATMENT TUMOUR EXCISION The more aggressive the lesion the more widely does it need to be excised, in order to ensure that the tumour as well as any dubious marginal tissue is com-pletely removed.

Intracapsular (intralesional) excision

and

curettage are incomplete forms of tumour

ablation and there-fore applicable only to benign lesions with a very low risk of recurrence, or to incurable tumours which need debulking to relieve local symptoms. Adjunctive treatment such as the use of acrylic cement after curettage decreases the risk of local recurrence. Marginal excision goes beyond the tumour, but only just. If the dissection of a malignant lesion is car-ried through the reactive zone, there is a significant risk of recurrence (up to 50 per cent). For benign lesions, however, this is a suitable method; the result-ing cavity can be filled with graft bone. Wide excision implies that the dissection is carried out well clear of the tumour, through normal tissue. This is appropriate for low-grade intracompartmental lesions (grade IA), providing a risk of local recurrence below 10 per cent. However, wide excision is also used in conjunction with chemotherapy for grade IIA lesions. Radical resection means that the entire compart-ment in which the tumour lies is removed en bloc without exposing the lesion. It may be possible to do this while still sparing the limb, but the surrounding muscles, ligaments and connective tissues will have to be sacrificed; in some cases a true radical resection can be achieved only by amputating at a level above the compartment involved. This method is required for high-grade tumours (IIA or IIB).

LIMB SALVAGE Amputation is no longer the automatic choice for grade II sarcomas. Improved methods of imaging and advances in chemotherapy have made limb salvage the treatment of choice for many patients. However, this option should be considered only if the local control of the tumour is likely to be as good as that obtainedby amputation, if it is certain that there are no skip lesions and if a functional limb can be preserved. The ongoing debate around limb sparing versus amputa-tion is addressed in an excellent paper by DiCaprio and Friedlaender (2003). Advanced surgical facilities for bone grafting and endoprosthetic replacement at various sites must be available. The first step consists of wide excision of the tumour with preservation of the neurovascular struc-tures. The resulting defect is then dealt with in one of several ways. Short diaphyseal segments can be replaced by vascularized or non-vascularized bone grafts. Longer gaps may require custom-made implants . Osteoarticular segments can be replaced by large allografts , endoprostheses or allograftprosthetic composites. It is recognized, however, that the use of large allografts carries a high risk of infection and frac-ture; this has led to them not being used as widely as in the past. Endoprostheses used to be custom-made but nowadays modular systems for tumour reconstruction are available. In growing children, extendible implantshave been used in order to avoid the need for repeated opera-tions; however, they may need to be replaced at the end of growth. Other procedures, such as grafting and arthrodesis or distraction osteosynthesis, are suit-able for some situations. Sarcomas around the hip and shoulder present spe-cial problems. Complete excision is difficult and reconstruction involves complex grafting and replace-ment procedures (OConnor et al., 1996). Outcome Tumour replacement by massive endopros-thesis carries a

high risk of complications such as wound breakdown and infection; the 10-year survival rate of these prostheses with mechanical failure as the end point is 75 per cent and for failure due to any cause is 58 per cent. The limb salvage rate at 20 years is 84 per cent (Jeys et al., 2008). AMPUTATION Considering the difficulties of limb-sparing surgery particularly for high-grade tumours or if there is doubt about whether the lesion is intracompartmen-tal amputation and early rehabilitation may be the wisest option. Preoperative planning and the defini-tive operation are

best carried out in a specialized unit, so as to minimize the risk of complications and permit early rehabilitation. Amputation may be curative but it is sometimes performed essentially to achieve local control of a tumour which is resistant to chemotherapy and radia-tion therapy. MULTI-AGENT CHEMOTHERAPY Multi-agent chemotherapy is now the preferred neoadjuvant and adjuvant treatment for malignant bone and soft-tissue tumours. There is good evidence to show that, for sensitive tumours, modern chemotherapy regimens effectively reduce the size of the primary lesion, prevent metastatic seeding and improve the chances of survival. When combined with surgery for osteosarcoma and Ewings tumours, the long-term disease-free survival rate in the best series is now about 60 per cent. Drugs currently in use are methotrexate, doxoru-bicin (Adriamycin), cyclophosphamide, vincristine and cis -platinum. Treatment is started 812 weeks preoperatively and the effect is assessed by examining the resected tissue for tumour necrosis; greater than 90 per cent necrosis is taken as a good response. If there is little or no necrosis, a different drug may be selected for postoperative treatment. Maintenance chemotherapy is continued for another 612 months. RADIOTHERAPY High-energy irradiation has long been used to destroy radiosensitive tumours or as adjuvant therapy before operation. Nowadays the indications are more restricted. For highly sensitive tumours (such as Ewings sarcoma) it offers an alternative to amputa-tion; it is then combined with adjuvant chemotherapy. The same combination can be used as adjunctive treatment for high-grade tumours, for tumours in inaccessible sites, lesions that are inoperable because of their size, proximity to major blood vessels or advanced local spread, for marrow-cell tumours such as myeloma and malignant lymphoma, for metastatic deposits and for palliative local tumour control where no surgery is planned. Radiotherapy may also be employed postoperatively when a marginal or intrale-sional excision has occurred, so as to sterilize the tumour bed.

The main complications of this treatment are the occurrence of post-irradiation spindle-cell sarcoma and pathological fracture in weightbearing bones, par-ticularly in the proximal half of the femur.