Chemotherapy (systemic therapy)
Systemic therapy is a treatment working throughout the whole body, and is usually called chemotherapy. The goal of systemic therapy is trying to kill (part of) the sarcoma or let it shrink, as well as treating visible and invisible distant metastases of the sarcoma. Systemic therapy may be necessary before and/or after surgery. Click here for more information on systemic therapy types.
Irradiation is a treatment offering better local control of the sarcoma. High doses are needed (50 – 80 Gy) during approximately 6 weeks, 5 days a week. The irradiation can be necessary before and/or after the surgery. Irradiation before surgery is used to make the tumor shrink or die (partially), while keeping the dose to the healthy tissue surrounding the tumor minimal. If irradiation is administered after the operation, the purpose is to decrease the risk of a local recurrence of the sarcoma. The treatment has several side effects: discoloration and hardening of the skin, muscle shortening and joint stiffness. Sometimes lymphedema (video) may occur.
With most patients, the removal of the sarcoma is necessary. Such an operation is never a standard procedure, it is always patient-tailored as no two patients present with exactly the same localisation, size or age. The goal of the surgery is a complete removal of the malignant tissue, while preserving a functional limb. This means that after the operation, your limb can be moved and sensitivity remains.
In order to achieve free resection margins, the surgeon has to resect a certain amount of healthy tissue. As higher malignant tumors penetrate into the surrounding tissue with small fingerlike extensions, this healthy margin has to be larger if the malignancy grade is higher. Sometimes blood vessels have to be removed and bridged by other vessels. Sometimes nerves have to be removed, resulting in a (partial) paralysis. If your surgeon cannot preserve an acceptable level of function of your limb, an amputation will be proposed. This will always be discussed with you. Resected muscles and tendons do not regenerate, not even in children. The surgeon may transfer other muscles to allow a maximum of movement, but regaining a normal function is not always possible.
The surgery type is determined by the localisation of the sarcoma:
If your bone is affected...
There are 2 options for surgery: removing the bone without reconstruction (for smaller lesions or low grade lesions) or removal with reconstruction of the defect.
Usually the bone is replaced by other bone (biological reconstruction) or by an internal prosthesis . A biological reconstruction is preferred. This can be done by ‘recycling’ the affected bone: the affected bone is removed and treated by irradiation or another sterilisation method to kill all sarcoma cells. Afterwards the treated bone is replaced and fixed by plates and screws. This bone is conversed into new, living bone by your body, creating a durable solution. Alternatively, bone from the donor bone bank can also be used to reconstruct the bone defect. This bone is tested extensively to avoid disease transfer from the donor to you.
If a joint is affected...
Then the reconstruction of your joint usually requires a prosthesis. These prostheses should not be compared to classical hip, knee or shoulder prostheses that are used in elderly people with arthrosis.
Reconstruction prostheses are much larger as a much larger part of the bone needs to be replaced, contrary to replacement of the worn cartilage in case of arthrosis. Extendable prostheses are sometimes used in growing children, and sometimes it is necessary to close the growth cartilage of the healthy leg to avoid leg length discrepancy. These prostheses have a more limited lifespan as young people are more active, leading to more wear and tear. Biological fixation systems allowing ingrowth of the prostheses are preferred, as this limits the risk of loosening of the prosthesis.
If only the soft tissues are affected...
The surgeon will remove all affected structures with a margin of healthy tissue, keeping vital structures intact. In case of limb surgery, the surgeon aims to preserve the function of your limb. As soft tissue tumors can also occur in your chest, or abdomen or on the chest or abdominal wall, the surgeon may have to remove (part of) an organ (eg a kidney, part of an intestine, part of a lung) in order to obtain a complete removal of the tumor. Sometimes a part of your skin has to be removed, whereafter the plastic surgeon will help to repair the defect. Your surgeon will discuss the surgery and its consequences with you. Structures that have been removed will not grow back, not even in children.
It is important to keep in mind that, whether either a biological or a prosthetic reconstruction is used, the treated bone remains very sensitive to infection, especially after administration of chemotherapy. Because of limited blood supply of the tissues surrounding the prosthesis or the bone graft, antibiotics cannot easily reach possible bacteria that are attached to the prosthesis or the graft. In case an infection cannot be controlled, additional surgery is needed, sometimes even warranting a secondary amputation. Your doctor may advise you to take antibiotics at the slightest sign of a bacterial infection or at the time of dental treatment, an advice to be followed for the rest of your life. This is only in cases where bone is being reconstructed, not for tumors only in the soft tissues.
How about your function?
The resection of a part of the bone or of soft tissues usually results in functional loss . Your surgeon will discuss the outcome with you in order to make you understand what you can expect, and to avoid unrealistic expectations. You may need additional information at a later time, by other specialists (e.g. a rehabilitation physician in case of amputation), or by other patients. The goal of the rehabilitation is to regain the maximal restoration of your daily life activities. Sometimes it is hard to accept that your function remains limited for the rest of your life. The functional limitation may also hamper your psychosocial and economical life.
How about the treatment per sarcoma type?
An osteosarcoma is high-grade malignant, but is sensitive to chemotherapy. This is the reason why the treatment includes high-dose chemotherapy before and after the surgery. Your oncologist can explain the exact schedule and concomitant side-effects to you. If possible, the tumor needs to be removed by the surgeon.
A Ewing sarcoma is high-grade malignant, but is sensitive to chemotherapy. This is the reason why the treatment includes high-dose chemotherapy before and after the surgery. Your oncologist can explain the exact schedule and concomitant side-effects to you. If possible, the tumor needs to be removed by the surgeon.
Sometimes the site where the tumor has been before removal needs additional irradiation in order to achieve local control. If your bone marrow is affected, you may need a bone marrow transplant.
The more aggressive the chondrosarcoma is, the more aggressive the treatment needs to be.
A low-grade chondrosarcoma can be removed by scraping it out of the bone (curettage), or by removing it completely, and reconstruct the bone either recycling the affected bone, or using a bone graft or a prosthesis. If your surgeon fears incomplete removal in case of curettage, a complete removal of the bone will be proposed. This decision will be discussed with you.
Grade 2 and 3 chondrosarcoma has a higher propensity to metastasise, and it is important to remove these tumors completely. This is only possible by removing the entire affected bone and reconstructing the gap.
A chondrosarcoma is not sensitive to irradiation or systemic therapy.
The sensitivity to irradiation and/or systemic therapy of soft tissue sarcomas is variable and depends on the tumor type. When the biopsy has revealed the tumor type, the multidisciplinary team will be able to compose a fitting treatment for you. If possible, the sarcoma will be removed surgically.lk type je te maken hebt, kan het multidisciplinaire team een behandeling op maat voor jou opstellen. Indien mogelijk wordt het sarcoma chirurgisch verwijderd.
How about follow-up?
When are you considered cured?
After treatment you will be followed-up between 5 to 10 years, depending on the sarcoma type you have had. This follow-up encompasses medical imaging of the tumor site and the distant organs your sarcoma type usually metastasises to. Your physician will evaluate whether the tumor has returned and whether distant metastases are present. You have to keep in mind that even the most modern apparatus cannot show lesions smaller than 5 mm. This means that, even if all imaging fails to show tumor lesions, your doctor cannot guarantee that no more sarcoma cells are present in your body. The longer the time interval after treatment, the higher your chances of a complete cure. If no local or metastatic tumor lesions are found after 10 years, you will be considered cured.
What if there are metastases?
The treatment options depend on the number of metastatic lesions and the sarcoma type. The options will be discussed during a multidisciplinary meeting, and afterwards your physician will discuss these options with you.
What if the sarcoma has returned?
The risk of local recurrence after removal is bigger if the malignancy grade of the tumor is higher, or if complete removal by the surgeon was not feasible. Your treating physician will discuss the treatment options with you, after a multidisciplinary meeting.
How about rehabilitation?
After a surgery, you will need rehabilitation therapy. The more severe the surgery, the longer the rehabilitation period. Your body has to adapt to the sequels following the removal of bone, muscles, nerves or blood vessels. Sometimes you need to learn how to walk again. The goal is to resume your daily activities as good as possible. Your surgeon will tell you to what extent you will be able to resume sports activities. It is important to follow the surgeon’s advice to avoid complications. A physiotherapist will coach and support you during this period.