Very rarely, patients with a Ewing’s sarcoma may have radiotherapy as their method of achieving local control rather than surgery. The surgeon needs to remove the tumour with a cuff of normal tissue to ensure ‘clear margins’ around the site and reduce the chance of the tumour recurring.
Most of the time the tumour can be safely removed, the skeleton rebuilt and the limb preserved – this is called ‘limb sparing surgery’.
However, if the tumour is very close to important nerves and vessels then amputation may be the best option. A multi-disciplinary team (MDT) discusses the best treatment for each patient based on the type and site of the tumour, the closeness to major nerves and vessels and the function that will be expected after surgery.
Treatment options will be discussed with the patient and their family by the surgeon who is treating them, and a final decision made about the type of surgery, either limb sparing (in most cases) or amputation. Limb sparing procedures are possible in approximately 80% of childhood sarcomas [2].
There are various types of reconstruction possible in limb sparing surgery. These include replacing the diseased bone with an internal prosthesis, or removing and irradiating the diseased bone, then replacing it (during the same operation) as well as various types of allograft. Generally, reconstruction with an endoprosthesis provides a very good looking limb which functions well. Inevitably over time the implant will loosen and will need to be replaced.
Taking part in contact sports and the like is discouraged because of the risk of fracture around the implant and implant breakage. This restriction can be difficult for families with young children, and active adolescents. The broad advantage of limb sparing surgery is that the patient retains their own limb. The broad disadvantages are the higher incidence of complications such as infection in the prothesis [1].
The decision about what surgery to do is very difficult and there is no right or wrong answer, but a balance of all the individual’s circumstances, future function [3] and patient choice. In either event children and young people are prepared for the forthcoming surgery and, whenever possible, given the chance to meet up with someone who has had similar surgery already.
If it is agreed that amputation is the best way to control the tumour, the limb fitting services will be involved. They will usually make arrangements before surgery for the patient and their family to meet someone with a similar amputation. The limb-fitting team and the amputee will be able to tell the patient what it is like to have an amputation, what things they will be able to do afterwards and to explain what will happen after surgery. Possibilities such as phantom limb pain are discussed and preventative medication started. Prosthetic limb services are involved because if the child or young person has a prosthesis they will continue to attend there long after their treatment is finished. Please see Amputee management (LINK)
Sources of information and support
Osteosarcoma information (CCLG)
Ewing sarcoma information (CCLG)
Bone cancer information (CLIC Sargent)
References
[1] Tomlinson D. and Zupanec, S. (2010) Musculoskeletal System. In Tomlinson D. and Kline, N.E. (eds.) Pediatric Oncology Nursing. 2nd Ed. London: Springer
[2] Kumta, S.M; Cheung J.C, Li C.K., Griffith J.F., Chow, L.T; Quintos, A.D.l. (2002). Scope and limitations of limb-sparing surgery in childhood sarcomas. Journal of Pediatric Orthopedics. 22(2). p.244-248.
[3] Grimer, R.J. (2005) Surgical options for children with osteosarcoma. The Lancet Oncology. 6(2). p.85-92.