Frequently Asked Questions (FAQs) about Sarcoma

What is Sarcoma?

Sarcoma is a rare cancer of bones and connective tissue. Connective tissue refers to highly specialised group of issues that maintain the form of body and provide internal support. For example, the muscles, bones, adipose tissue (fat), cartilage, ligaments etc are all a type of connective tissue. So any cancer arising from muscles, bones, fat etc is termed Sarcoma. Since it can arise from variety of tissues (fat, bone, cartilage, muscle etc), there are more than 60 subtypes of Sarcoma.

What are symptoms of Sarcoma?

Symptoms usually depend on location of the disease. It can begin as a painless swelling in the arms or legs, which gradually increase in size and may become painful as the size increases. Sometimes, sarcoma originating in the thigh or pelvis can attain very large size before it gets noticed. Sarcoma of soft tissues usually occurs in adults and older age groups. Sometimes, the swelling is mistaken for an abscess (pus collection) and patient is wrongly subjected to an unplanned surgery in the form of I&D (incision and drainage) which can be detrimental not only in future limb salvage but also delays the start of correct treatment.

Sarcoma originating in the bone is painful to begin with which worsens over time and is associated with swelling in the affected region. Bone sarcoma (Osteosarcoma, Ewing’s Sarcoma) usually occurs in growing children and young adolescents.  It can cause weakening of bones resulting in fracture. Some patients may attribute these symptoms to any prior antecedent trauma. Sometimes, these patients are wrongly being treated for infection, thus delaying the correct treatment which may have a bearing on the final outcome.

In short, one should not ignore any lumps or bumps in his/ her body more so if they are increasing in size over time.

How common is Sarcoma?

Sarcoma is a very rare form of cancer affecting 1 person in a million. It approximately constitutes 1% of all adult cancers.

What are causes of Sarcoma?

There are no known environmental or other hereditary factors which cause sarcoma, except for patients who have received prior radiation therapy. Certain patients are at a greater risk for bone sarcoma which include:

  • Patients with a history of Paget’s Disease
  • Patients with hereditary retinoblastoma – a type of eye cancer that most commonly affects very young children
  • Patients with Li-Fraumeni syndrome – a rare genetic condition
How is sarcoma diagnosed?

A patient suspected with sarcoma needs to be investigated thoroughly with blood tests, Xrays, MRI to look for local extent of disease. Since sarcoma can spread to lungs and bones, staging of the disease is done either with whole body PET CT or a bone scan with CT Chest or CT chest alone depending on the type of sarcoma. Alongwith that, a biopsy (usually with a needle) is required to establish the diagnosis. It’s important to do biopsy from the correct site since wrong biopsy site can be detrimental in limb salvage and can result in amputation (cutting the limb). Therefore, it’s recommended that the biopsy should always be done by the surgeon (Orthopaedic Oncologist) who will be doing the final surgery for sarcoma.

What is biopsy? Who should do the biopsy?

In biopsy, some part of tumour tissue is removed and sent for examination under microscope. It can be open biopsy (in which a surgical incision is made to expose the tumorous mass and take out the diseased tissue) or a needle biopsy (in which a thin needle is used to take out the tissue without any incision). Since needle biopsy doesn’t involve any incision, the surgical trauma, pain and morbidity are lesser with needle biopsy.

It is very important that a biopsy should always be done by surgeon experienced with sarcomas who will be doing the definitive surgery since poorly done biopsies not only may yield false negative or inconclusive results but also wrongly placed biopsy scars can be detrimental for future limb salvage (which might unnecessarily cause increase in extent of surgery and hence the complications). In a nutshell, since the location and technical aspects of biopsy can affect the treatment options and final outcome of the patient, biopsy should always be done by Orthopaedic Oncologist or one of his team members.

What is treatment of sarcoma?

Treatment of sarcoma is a multidisciplinary approach requiring an Orthopaedic Oncologist (Bone & Soft tissue tumour specialist), Medical Oncologist (Chemotherapy Specialist), Radiation Oncologist (Radiotherapy specialist) and Pathologist (Specialist in tissue diagnosis).

If the sarcoma is not metastatic (not spread to any other parts of body), it will usually involve some form of surgery to remove the cancerous tissue with a wide margin (with a cuff of normal healthy tissue around the tumour). Patient may require additional treatment in the form of radiation therapy or chemotherapy, which depends on the type of sarcoma, type of surgery, age of patient etc.

Soft tissue sarcomas usually require surgery with wide margin and radiation therapy which may be given prior to surgery (pre op RT), during surgery (brachytherapy) or after surgery (Post Op RT). Patient may need chemotherapy also which is mainly determined by the sarcoma subtype and age of patient.

Usually in primary bone sarcomas, chemotherapy is given to the patient followed by surgery to remove the diseased bone which is further followed by adjuvant chemotherapy. Limb salvage surgery is possible nowadays for bone sarcomas in which the part of the bone involved with tumour is removed surgically (rather than cutting the whole limb) and the defect is reconstructed with artificial joint, thereby saving the limb. In some cases, the cancerous bone so removed is given very high dose of radiation so as to kill all the cancer cells and the sterilised bone so formed is fixed back to the parent bone with the help of plates and screws, a technique commonly called as Extra Corporeal Radiation Therapy (ECRT). In children, since the artificial joint will not grow as the child grows leading to unequal limb lengths over a period of time, the defect can be reconstructed with expandable joint which can be lengthened as the child grows which allows to maintain limb length equality at the time of skeletal maturity.

In short, treatment of sarcomas is individualised based on type of sarcoma, it’s location, size, extent of spread, age of patient etc.

What is Chemotherapy?

Chemotherapy is anticancer therapy which slows the growth of rapidly dividing cancer cells in the body. For bone and soft tissue sarcomas, it is given intravenously and patient requires admission in the hospital. Chemotherapy can be administered before surgery to shrink the tumour, after the surgery to kill remaining cancer cells or both.

What is Radiation therapy?

Radiation therapy is the use of ionising radiation to kill the cancer cells. It can be given before or after surgery. It is given to decrease the chance of local recurrence (cancer coming back locally). The treatment may be given over single sitting or multiple sittings over few days to weeks depending on the indication. Each session only takes only a few minutes.

Is cure possible in Sarcomas?

With the current chemotherapy regimes and advanced surgical technology, cure is possible in upto 70% patients with sarcomas. Cure rate depends on type of sarcoma, it’s disease load, size of sarcoma, extent of spread at presentation and so on. In advanced cases also, a lot can be done to control the disease and give a quality of life to the patient.

Is it possible to prevent Sarcoma?

Since the exact cause of sarcoma is not known, as of now it’s not possible to prevent sarcoma.

Whom to consult?

First chance is the best chance when it comes to curing sarcomas. So, it’s advisable to seek treatment from a trained Orthopaedic Oncologist when confronted with a sarcoma to achieve maximum beneficial outcome.

Can I go for alternative treatment?

Though number of people claim to cure cancer but there is no scientific evidence backing those claims. Patients falling for these alternative therapies delay their actual (evidence based) treatment which may be detrimental. It’s always prudent to discuss with your sarcoma specialist if you are on any alternative medicines along with the conventional treatment since it may interfere with the standard treatment thereby decreasing the efficacy of conventional evidence based treatment or may increase it’s side effects.

This article has been contributed by Dr. Rajat Gupta, Orthopaedic Oncologist