Bone and Soft Tissue Cancers: Symptoms, Causes and Treatment

Introduction:

Bone and soft tissue cancer is also known as musculoskeletal cancer. These are cancers affecting the musculoskeletal system which includes bones, cartilage, muscles, tendons, and ligaments. Besides these, cancers involving the adipose tissue (fat tissue), blood vessels and peripheral nerves are also included in this group of cancers. The primary cancers arising in these tissues are collectively called as Sarcomas. These are cancers which originate in these tissues. In the bones, we also encounter another group of cancers which are collectively called as secondary cancers or metastasis. These are cancers of other organs/tissues that has spread to the bone.

Primary musculoskeletal cancers:

They are broadly divided into two groups. Those that affect bone and those that affect the soft tissues. This group of primary cancers are collectively called as sarcomas.
This is one of the rarest family of cancers and accounts for less than 1% of all cancers in adults. However, in children, around 15 to 20% of cancers are sarcomas. There are more than 100 types of sarcomas. These are identified based on their tissue of origin, appearance on imaging and findings on histology under the microscope. Treatment is planned based on the exact diagnosis and stage of the disease. Depending on the type of sarcoma, treatment may involve chemotherapy, surgery, and radiotherapy; either as a single modality or more often as a combination of these different treatment modalities. As these diseases involve the musculoskeletal system, they most commonly affect the extremities – the upper limbs and lower limbs. The rarity of these cancers along with the diversity of tissues they arise from, their varied locations in the body, the huge number of types of these cancers and the different age groups they affect, makes their treatment very challenging. Moreover, surgery for these cancers involve extensive loss of bone, muscle, and important joints which needs very complex preparation and planning for limb and function preservation. These are called as limb salvage surgeries for bone and soft tissue cancer. Hence, it needs very dedicated and highly experienced specialist team of doctors to manage these conditions well. With timely and proper treatment many of the sarcoma patients can be cured. Also, in almost 90% of these patients, safe surgical removal of the malignant tumour with optimal limb preservation, maintaining good function in the affected limb, is possible.

Secondary bone cancers:

 These are also called as bone metastasis. Unlike primary bone cancers, which originate in the bone, secondary bone cancers are cancers of other organs which has spread to the bone. These represent an advanced stage of that primary cancer. Almost all cancers can spread to bone. In fact, along with liver and lung, bone is one of the most common locations for spread of cancer. Some of the cancers which very commonly spread to bone are breast cancer, lung cancer, prostate cancer, kidney cancer and thyroid cancer. Cure is a very rare possibility in this situation and may be attempted in some well selected cases only.  In most of these cases, treatment is aimed at controlling the disease and managing the symptoms of the metastasis. This is called palliative care. The most important symptom of bone metastasis is pain. Besides that, in a significant number of patients, the disease in the bone weakens it; making it vulnerable to fracture with minimal trauma or sometimes without any obvious injury or cause. This is called as pathological fracture. This can very badly affect the quality of life of the patient, often rendering them bedridden or seriously disabled. If the bone metastasis has affected the spine, it can cause severe back pain and sometimes can even cause paralysis of extremities – in most such cases – of the lower limbs, along with loss of control of passing stools and urine. This can be a very distressing experience for the patient and family. With proper patient work-up and well-planned treatment, majority of these complications and consequences of bone metastasis can be avoided or can be managed before it becomes severe. In those where such events do occur, optimal management by a team of experts can make a huge difference to the patient in treating the damaged bone, restoring their independence, and maintaining quality of life.

At Hinduja Hospital, we have the best team of specialists for sarcoma and musculoskeletal cancer treatment along with state-of-the-art equipment and facilities that enables us to provide world class care to sarcoma patients and patients with bone metastasis.

What are the Symptoms of Bone and Soft Tissue Cancer?

The four major symptoms of bone and soft tissue cancers are pain, swelling, disability and pathological fracture.

● Pain:

Unexplained pain in a particular area of the body is often a presenting complaint. These start for no apparent reason and progressively worsen over time which may be in a matter of days, weeks, or months. Rarely, it may be noticed after a minor injury which draws attention to the region of pain. The important feature is the progressive and worsening nature of the pain, both in duration and intensity. This pain eventually becomes constant and is then felt even at rest. In some it may even affect sleep. It is however important to know that not all such pains are caused by cancer. There are other conditions like benign (non-cancerous) bone and soft tissue tumours, tumour like conditions, infections, etc., which may present with similar complaints. The important thing is, when you have any unexplained pain, especially which is not resolving, you should see your doctor. As a rule of thumb, any pain of more than 3 weeks duration should be investigated.

● Swelling:

Any unexplained swelling should be brought to the attention of your doctor. Any swelling which is of recent onset or noticed recently, and any swelling which is growing, irrespective of its size, should be viewed with a high degree of suspicion. A common misconception is that a painless swelling is harmless. That is not true. Many malignant tumours begin as painless swellings. If you have a small long-standing stable swelling (of many years duration), you may not worry much about it. However, if that swelling shows signs of activity in the form of growth or new symptoms, it should be viewed with concern and brought to the attention of your doctor. Long standing benign tumours can rarely change their behaviour into malignant disease. Another commonly mismanaged situation is a small and superficial soft tissue swelling. Often these are treated based on a clinical assessment or a sonography. Although most of these are benign conditions, some of these can turn out to be soft tissue sarcomas. It is very important that any swelling that is subject to any form of treatment is thoroughly worked-up, first. This is especially important for any swelling of recent onset, any swelling that is growing, and any long-standing swelling that has recently changed its behaviour.

● Disability:

This is generally noted in bone tumours. Sometimes, large soft tissue tumours can also cause disability. In majority of the cases this disability is due to pain which limits the ability to use the affected extremity. In the lower limbs it can affect the mobility of the patient, making walking difficult or even impossible. In the upper limbs, it may result in difficulty in doing routine activities like writing, eating food, using a computer, brushing hair, etc.  Rarely, the disability is caused due to the swelling resulting from the growth of the tumour which restricts the range of movement of the adjacent joint.

● Pathological fracture:

Pathological fracture is a fracture of bone which is weakened by disease. The distinguishing feature of pathological fracture is that, unlike a regular fracture, it happens with minor trauma/injury or sometimes during routine activity. Bone is structurally a strong tissue. It gives shape and structure to the body and it takes up the load of our body. It takes considerable amount of force to break any bone. This happens in high velocity injuries and falls from considerable heights. However, when bone is weakened due to any condition, either physiological (e.g., osteoporosis) or pathological (sarcoma, metastasis, benign tumour, infection, etc.), it can break with minor injury or sometimes while simply using the extremity – for standing/walking or lifting a weight. This can cause severe pain and disability. If it occurs in the lower limbs, it can make it very difficult or even impossible for the patient to get out of bed. In the upper extremity, it can disable the affected limb. In the spine, such incidents can cause excruciating back pain and even paralysis of the lower extremities. Any pathological fracture is a distressing experience for the patient and family. However, with the right care, most of these patients can be treated and their function restored. It is always very helpful if the condition is detected before the actual fracture, as it can help avoid such painful and disabling incidents. Many of these patients have progressively worsening pain in the region for days or weeks prior to the fracture. This pain is typically triggered or aggravated by movement and weight bearing or lifting of heavy objects. Any such symptom is an important clue to a possible pathology in the bone with a risk of pathological fracture. This is even more important in patients who have been diagnosed with cancer or have been treated for cancer in the past. Any such pain which is of recent onset and is experienced on weight bearing or is worsened with movement should be investigated thoroughly.

What are the Causes of Bone and Soft Tissue Cancer?

In most of the primary bone and soft tissue cancers, there is no known cause. They are of spontaneous origin and there is no way to prevent them from happening, or lowering the risk. The best way to manage these conditions is to detect them early and treat them appropriately. Extremely rarely, sarcomas can be associated with certain genetic conditions. Rarely, some benign bone tumours may carry a small risk of change into a malignant tumour.

How is Bone and Soft Tissue Cancer Diagnosed at Hinduja Hospital?

As is the protocol for these tumours, at Hinduja Hospital, we diagnose bone and soft tissue cancers in a systematic and scientific manner. It is very important to thoroughly investigate every patient to get the diagnosis right as the treatment for every tumour is different. Diagnosing these tumours involve a thorough clinical evaluation, radiological/imaging studies, histopathological tests, and staging investigations.

Clinical evaluation:

This involves a meticulous clinical assessment of the patient which includes a detailed history of the patient’s complaints and medical condition along with a thorough physical examination. Following this, the doctor will advise appropriate imaging studies.

Imaging Tests:

Medical imaging modalities include X-Ray, MRI, CT scan, and PET (Positron Emission Tomography) scan. A lot of information related to the disease in the bone is obtained through these imaging studies. X-ray is one of the most important primary radiological investigations for the understanding and diagnosis of bone cancers. With these studies, we also get to know the exact location, size and extent of the disease.

Histopathological tests:

This involves sampling of the tumour tissue which is then studied under a microscope by specialists called pathologists. This is the most reliable way of identifying most cancers. The two common ways this is done is called FNAC and biopsy.

FNAC is short for fine needle aspiration cytology. In this, cells of the disease are sampled from the tumour using a fine needle and syringe, which is then studied under a microscope. Although it is a simple procedure and can sometimes be sufficient for identifying a tumour, it has several limitations, and is therefore not the preferred procedure to identify a primary bone and soft tissue tumour. It is however, extremely helpful in identifying any relapse of a known cancer.
 

Biopsy – In this, actual cores or macroscopic pieces of the disease tissue is sampled which is then processed, embedded in wax, sliced thinly, stained, and studied by the pathologist under a microscope.

There are two ways in which the biopsy procedure for tissue sampling can be done.

1) Needle Biopsy – Here, multiple cores of tumour tissue are sampled using a special thick bore hollow biopsy needle or a tru-cut biopsy needle. This is the preferred method for biopsy of most musculoskeletal tumours. These procedures are often done with image guidance (CT scan, USG or X-ray guided) for precise localisation of the tumour and accurate needle placement, which ensures high degree of success with these procedures.

2) Surgical Biopsy or open biopsy – In this, small pieces of the disease tissue is collected through a small surgical incision made directly over the diseased area. This involves a larger surgical incision as compare to needle biopsy. For this, and many other reasons, open biopsy is not the preferred method of biopsy of musculoskeletal tumours. However, in situations where needle biopsy is difficult, unsafe, or has failed, open biopsy may be considered.

An important point to be careful with regarding biopsy of any bone and soft tissue cancer is the site of biopsy – the point of entry on the skin. This must be chosen well, taking into consideration the location of the disease, so that it can be removed with the tumour at the time of surgical treatment of the cancer.

Keeping these considerations in mind, biopsy procedures are performed with utmost care after thorough planning by our specialist doctors at Hinduja Hospital. All care is taken to ensure the procedure is conducted in a manner which ensures that the actual treatment of the condition is not compromised in any way.

Staging investigations:

Whenever a diagnosis of cancer is made, it is very important to stage the disease. This is to find out whether the disease is confined to one place (primary site) or whether it has spread to other areas/organs of the body (secondary sites). This helps in deciding the plan of treatment of the patient along with the treatment goals. It helps in deciding whether the treatment should be conducted with the intention of curing the patient or whether the patient should be treated with a palliative intent – aiming at controlling the disease and treating any troubling symptoms caused by it. In bone cancers this is done using a bone scan to screen the skeletal system for any disease spread (skeletal metastasis) and a HRCT of the chest to screen the lungs for any pulmonary metastasis (spread of disease to the lungs). Nowadays, a head-to-toe PET-CT scan can be done, instead. PET scan also gives information about the level of metabolic activity in the tumour which can be helpful in assessing treatment response when compared with follow-up PET scans in patients who receive chemotherapy.

How is Bone and Soft Tissue Cancer Treated?

Treatment of bone and soft tissue cancer is tailored depending on the type of cancer, stage of the disease, the age of the patient, location of the cancer in the body, extent of the disease in that location, length of bone affected, condition of the adjacent joint, health condition of the patient, and many other factors. This individualised approach is essential as different cancers respond to different treatments. Moreover, even with the same disease, when it comes to surgery, every patient’s treatment is different; especially when it comes to bone cancer surgery. The major modalities of cancer treatment are medical management, surgery, and radiation therapy.

Medical management:

In this modality of cancer treatment, the disease is treated with powerful medicines which are injected through an intravenous (IV) line or consumed through the mouth. The specialists who treat cancer with medicines are called medical oncologists. One of the important forms of medical treatment of bone and soft tissue cancer is called chemotherapy. These are powerful medicines which are injected into the body through an intravenous line. Often special intravenous lines are inserted into the body for administering chemotherapy. One important type of such IV line is called a chemotherapy port. Another similar IV line for chemotherapy is called PICC (peripherally inserted central catheter) line. There are many benefits of such dedicated chemotherapy IV lines as the chemotherapy treatment is given in several cycles and can go on for many weeks or months.

A chemotherapy cycle is the duration between the start of one chemotherapy session to the beginning of the next. In any given cycle, the actual treatment with IV chemotherapy drugs happens in the first few days and the remaining days of the cycle is for the normal healthy cells of the body to recover from the powerful effects of the treatment. Generally, a cycle is of 3 weeks duration. However, it may be variable depending on what chemotherapy protocol is being used on the patient. This will be decided by your medical oncologist. Osteosarcoma and Ewing’s Sarcoma are primary bone cancers where treatment starts with chemotherapy. After the initial few cycles of chemotherapy, the patient is evaluated for local management of the primary cancer. In most of the patients, this is managed with surgery. This part of the chemotherapy which is administered before the local management of the primary cancer is called Neo-adjuvant chemotherapy (NACT). After the local management of the tumour, the patient completes the remaining cycles of chemotherapy. Some primary bone sarcomas do not need chemotherapy and are treated directly with surgery. An important example of this type of primary bone cancer is conventional chondrosarcoma. Besides chemotherapy, there are several other ways in which a medical oncologist treats cancer. This includes immunotherapy, targeted therapy, etc. At present, chemotherapy is still the mainstay of primary bone cancer treatment. At Hinduja Hospital, we have a robust medical oncology department who have vast experience in treating bone and soft tissue cancers.

Surgery:

Surgery is another important treatment modality in the management of bone and soft tissue cancer. Doctors who treat bone and soft tissue cancer surgically are called orthopaedic oncologists or more appropriately, orthopaedic oncosurgeons. These are highly specialised doctors who have trained in orthopaedic surgery and then further specialized in bone and soft tissue cancer surgery. The challenge with bone and soft tissue cancer surgery is that it needs the surgeon to be an expert both in the principles of cancer surgery and in the methods of orthopaedic surgery. Unlike other forms of orthopaedic surgery, the surgical management of musculoskeletal cancers require the treating doctor to have a very sound knowledge, training and experience of bone and soft tissue cancer surgery. This is because cancer surgery requires the surgeon to plan and execute the surgical removal of the malignant tumour with safe margins. This is the most important part of the surgery, to ensure safe and complete removal of the cancerous tumour in a way that ensures that it does not come back in the same region again. The procedure is called wide excision of the tumour. There are well established surgical principles and guidelines that are followed by the specialist surgeon to ensure oncologic safety of the procedure.

A few decades ago, the only way this was possible was with an amputation surgery at an appropriate level. However, since then a lot has changed in the field of bone and soft tissue cancer surgery. We are fortunate to be in the era of limb saving surgery for these cancers. The challenge of these cancers is that they affect the extremities most often and it needs the surgeon to be aware of the anatomy of these regions and also be proficient in the techniques of orthopaedic reconstruction following the safe surgical removal of bone and soft tissue cancers. This is where a specialist orthopaedic oncosurgeon makes all the difference. With the development of principles and techniques of safe wide excision of bone and soft tissue cancer, various limb saving procedures and techniques, and availability of complex limb reconstruction implants, a specialist orthopaedic oncosurgeon can now save the limbs of more than 90% of patients affected with these cancers. Many limbs are saved with the use of metal implants called megaprosthesis which not only help reconstruct major joints but also replace long segments of diseased bone which have been removed.

 Another important role of an orthopaedic oncosurgeon is the treatment of bone metastasis. Many patients with advanced cancer of other organs develop bone metastasis – spread of cancer to the bone. This can be a very distressing experience for the patient as it can cause severe pain leading to difficulty in using the affected extremity and sometimes resulting in fractures of the affected bone – called pathological fracture. These are very unlike the routine fractures that happen following serious injury. In pathological fractures, the affected bone is diseased and therefore needs a very different approach to its treatment. An orthopaedic oncologist, who understands the nature of these diseases that has led to the fracture of the bone, is the right specialist to manage pathological fractures.

 At Hinduja Hospital, we have a team of specialist cancer surgeons, orthopaedic surgeons and very experienced orthopaedic oncosurgeon dedicated to the treatment of bone and soft tissue cancer.

Radiation Therapy:

Radiation therapy is another important modality of treatment for bone and soft tissue cancer. This treatment is generally used as an adjuvant treatment to surgical management of bone and soft tissue cancer. In fact, the most important role of radiotherapy is in the treatment of soft tissue sarcoma. It may be used in some specific cases of bone sarcoma as well. In most cases, radiotherapy treatment starts after the surgical wound of a soft tissue sarcoma excision has healed well. Nowadays, in some cases of soft tissue sarcoma with specific concerns, radiotherapy treatment may be given before the surgical excision of the tumour. Radiotherapy treatment is done by specialists called radiation oncologists who are also called as radiotherapists.

Many are confused between a radiologist and a radiation oncologist. A radiologist is a specialist who uses various imaging modalities like X-ray, MRI, ultrasound, and CT scan to locate and diagnose medical conditions. They may also do certain image guided procedures for treatment or diagnosis of these conditions. On the other hand, a radiation oncologist is a highly specialised doctor who treats cancer with powerful rays of photons (X-rays/gamma rays) or particles (electrons, protons, etc.). They use highly sophisticated machines, materials, and techniques to treat cancer patients. They have a role in treating many different types of cancer. We will restrict our discussion here to their role in treating bone and soft tissue cancer.

Radiation oncologist treats cancer using powerful X-rays or sub-atomic particles which are delivered in a very controlled manner to treat the cancer and cancer affected areas of the body. The important thing is to ensure that the radiation treatment is delivered very precisely to the area of interest while minimising or completely avoiding exposure to the rest of the body. Areas of the body in the immediate vicinity of the target region will receive some spill of radiation. With careful planning, the radiation oncologist ensures that the target area receives the maximum planned dose of radiotherapy while the normal surrounding organs and tissues are protected and any unavoidable radiation received by these regions is as low as possible.

There are many ways in which radiotherapy is delivered to the patient. One of the most important ways of delivering radiotherapy is called external beam radiotherapy (EBRT). As the name suggests, here the source of radiation is outside the body. In this, powerful X-rays generated by machines called as linear accelerators (linac) are focused on the target area to kill cancer cells. These are very sophisticated machines that allow the radiation oncologist to precisely shape the X-ray beam to hit the target area while limiting the exposure to surrounding normal tissue. This treatment is given in multiple fractions over many sessions. Generally, the treatment lasts around 6 weeks with one session a day, 5 days a week. Depending on the type of disease being treated, the location of the target area, and considering other factors, treatment is highly individualized and can therefore differ from patient to patient.

Another form of radiotherapy that is used in well selected cases of soft tissue sarcoma is called as brachytherapy. In this, radiotherapy is delivered through special catheters that are placed in the surgical bed at the time of surgical removal of the tumour. The radiotherapy treatment starts a few days later. Here, using a special machine, a tiny radiotherapy source is passed through the catheters to deliver radiotherapy directly to the bed of the tumour from within the body. This allows the delivery of high dose of radiotherapy directly to the target area while significantly minimising the radiation exposure of normal tissues. The other benefit of brachytherapy is the treatment can start early after the surgery and it also gets over very soon – in a matter of days – typically around 5 days. The radiation oncologist will decide about the suitability of the patient for brachytherapy at the time of surgical planning. In cancer patients with bone metastasis, radiotherapy is very helpful in managing bone pain, and sometimes in also avoiding pathological fractures. At Hinduja Hospital, we have a very well-equipped radiation oncology department with a team of radiation oncologists who are very experienced in the treatment of bone and soft tissue cancer.

Conclusion and message:

Like any other form of cancer, primary bone and soft tissue cancer (sarcoma) can be treated very well when detected early, diagnosed correctly and managed appropriately. It is very important to detect these conditions before they spread to other locations in the body. This is one of the most crucial factors which decide the overall prognosis of the patient. This is something where you can make a big difference.

Do not ignore any unexplained pain or swelling; especially if it is persistent and worsening. Any pain which is bothering you for more than 3 weeks should be brought to the attention of your doctor. Any persistent pain which is felt even at rest should be investigated. Any unexplained swelling is a cause for concern and should be brought to the attention of your doctor. Commonly misunderstood swellings are those which are painless and those which are small. It is important to know that most malignant tumours are painless to begin with and they all start out small. In fact, it is the best time to manage these conditions. Of course, it is important to know that most of these small and painless swellings are benign conditions like lipoma, fibroma, schwannoma, sebaceous cyst, inclusion dermoid, etc. However, it is very often difficult to make out a malignant condition from these benign ones, only on the basis of size and symptoms. Your doctor should be able to help you out with this. Special attention should be paid to any swelling (of any size) of recent onset, any swelling which is growing, or any pre-existing long standing swelling which is changing its behaviour – these should be investigated in detail as soon as possible.

At Hinduja Hospital, we have a very well-developed bone and soft tissue tumour department which includes all the diagnostic and treatment facilities. We have a robust team of medical oncologists, surgical oncologists, radiation oncologists and a highly experienced orthopaedic oncologist. We deal with all forms of bone and soft tissue cancers in all age groups. We are also very well equipped to treat bone metastasis, and all forms of benign bone and soft tissue tumours. In case of any musculoskeletal discomfort, pain, or swelling, visit Hinduja Hospital for comprehensive care and treatment. We are confident that you will receive the best advice and care for your health condition.