Soft Tissue Tumors

Soft Tissue Tumors are those tumors that arise in the Somatic Soft Tissues, which includes skeletal muscle, fat tissue, fibrous connective tissue, blood vessels, lymph vessels, tendons, ligaments, etc. Tumors arising from the peripheral nervous system are also considered as Soft Tissue Tumors.

In a human being‚ any tissue which is not bone‚ tooth or nail is physically a soft tissue e.g. lungs‚ kidneys‚ brain‚ liver‚ skin‚ muscle‚ fat‚ etc. However the term ‘Soft Tissue ’ in ‘Tissue Tumor’ does not include all of these tissues. Soft Tissue Tumors are those tumors that arise in the ‘Somatic Soft Tissues’. This is a large group of different tissues‚ the bulk of which is made up of skeletal muscle and fat tissue. The other somatic soft tissues are fibrous connective tissue‚ blood vessels‚ lymph vessels‚ tendons‚ ligaments‚ etc. What is interesting to note is that‚ in a healthy adult individual of normal weight‚ more than 65% of the body weight is made of these somatic soft tissues! Although the embryological origin of the peripheral nervous system is different from that of the somatic soft tissues‚ tumors arising from the peripheral nervous system are also considered as Soft Tissue Tumors.

As with all tumors‚ Soft Tissue Tumors‚ based on their biologic behavior‚ can be benign (non-cancerous) or malignant (cancerous). Benign Soft Tissue Tumors are far more common than the malignant tumors.

As with all tumors‚ Soft Tissue Tumors.‚ based on their biologic behavior‚ can be benign (non-cancerous) or malignant (cancerous). Benign Soft Tissue Tumors. do not spread to other regions of the body. Malignant Soft Tissue Tumors. are cancerous tumors‚ which means they can spread to other regions of the body and can pose a serious threat to the life of the patient. Elaborating further, the W.H.O. has recognized two more categories of Soft Tissue Tumors‚ which have biologic behavior somewhere in-between these two extremes of malignant and benign tumors. They are called intermediate tumors and are recognized as ‘Locally Aggressive’ (high risk of local recurrence following surgery) or ‘Rarely Metastasizing’ (low incidence of metastasis). Soft Tissue Tumors. form a large group of tumors ranging from the very common lipoma (a benign tumor of fat tissue) to some of the rarest tumors known. Benign Soft Tissue Tumors are far more common than the malignant tumors.

‘Cancer’ is a general term used for a group of disorders, which manifest in most cases as an abnormal mass/lump/swelling formed due to abnormal cells that multiply uncontrollably‚ and which have the potential to invade other tissues‚ both local and distant. The major classes of cancer are carcinoma‚ sarcoma‚ lymphoma‚ leukaemia‚ etc.

For a more detailed explanation‚ see the section on Malignant Bone Tumors.

Sarcoma is a class of cancer arising from connective tissue, which includes bone, muscle, fat tissue, etc. The other major classes of cancer are carcinoma, lymphoma, leukaemia, etc. Malignant (cancerous) Soft Tissue Tumors arising from the somatic soft tissues are called Soft Tissue Sarcomas (STS). Depending on the exact nature of the tumor tissue, there are a variety of sarcomas, e.g. synovial sarcoma, angiosarcoma, liposarcoma, etc.

This may seem a silly question indeed as most people are aware that ‘cancer means danger’. As with any cancer, Soft Tissue Sarcoma is also dangerous and can be life threatening if not detected in time and treated appropriately. What is more important to know is, it is treatable and that, earlier the detection and treatment, better is the outcome. This is just to reinforce that information.

Most benign Soft Tissue Tumors can be cured with surgery.
Malignant Soft Tissue Tumors (STS) on the other hand, are less predictable. However, generally, if a STS is detected early and treated appropriately, there is a good chance of cure.

Most Benign Soft Tissue Tumors can be cured with surgery. There are some Benign Soft Tissue Tumors‚ which are locally aggressive, i.e. they have a high incidence of local recurrence, even after appropriate treatment. Despite this, they rarely pose a threat to the patient’s life or limb, unless they occur in a difficult location, involving vital organs, making surgery difficult or dangerous.

Malignant Soft Tissue Tumors (STS) on the other hand, are less predictable. There are various factors, which affect the outcome of treatment in a patient with Soft Tissue Sarcoma. Generally, if a STS is detected early and treated appropriately, there is a good chance of cure.

Malignant Soft Tissue Tumors are very rare. In adults, less than 1% of all cancers are soft tissue

It is a surprising fact that, although somatic soft tissues make up more than 2/3rd of the body weight in an adult human being, malignant soft tissue tumors are very rare as compared to the other malignant tumors like breast cancer, lung cancer, prostate cancer, thyroid cancer, etc. In adults, less than 1% of all cancers are Soft Tissue Sarcomas. STS are more common than bone sarcomas. STS are most common in middle aged and older adults. However, they are relatively more common in children and young adults. In children, about 7% of all cancers are STS.

In such a situation, the most important thing that you must do is, speak to your doctor and consult a specialist.

At the same time, however, it is also very important for you to know that there are some things that you must NOT do. They are as follows:

• 1.Do not massage the swelling under any circumstance. This is because, if it is a malignant tumor‚ massaging could cause some of the tumor cells to be released into the blood circulation or the lymphatic system‚ through which they could travel and lodge in some distant organ or tissue and form metastasis. Besides‚ massaging will not offer relief in the presence of a tumor; it could actually trigger or aggravate the pain.
• 2.For the same reason as above‚ do not apply any sort of compression bandage over the swelling. Also‚ avoid touching and examining the region frequently.

Definitely not. Soft Tissue Tumor is not a contagious disease or any sort of infection. There is no health risk to any other member of the family whatsoever.

Most Benign Soft Tissue Tumors do not have an identifiable cause. They seem to occur spontaneously and randomly. The risk of developing a Benign Soft Tissue Tumor is generally the same for everybody. However‚ there are some rare Benign Soft Tissue Tumor conditions which are known to run in families.

It is very unusual for sarcomas to run in families, although such occurrences are not totally unknown. Most cases of sarcoma, in general‚ have no known cause. The best explanation available now is that‚ in most cases‚ it’s merely a matter of chance (i.e. they are sporadic or of spontaneous origin). In such a situation, the risk to any member of the family is for all practical purposes similar to that of an unrelated person. However‚ there are certain very rare genetic disorders that run in some families‚ which have been associated with an increased risk of developing malignant tumors‚ including sarcomas‚ e.g. Li-Fraumeni syndrome.

Most Benign Soft Tissue Tumors do not have an identifiable cause. They seem to occur spontaneously and randomly. The risk of developing a Benign Soft Tissue Tumoris generally the same for everybody. However‚ there are some rare Benign Soft Tissue Tumor conditions which are known to run in families‚ e.g. Familial Multiple Lipomatosis (a rare autosomal dominant inherited disorder characterized by multiple discrete sub-cutaneous lipomas)‚ Neurofibromatosis Type 1 (an autosomal dominant inherited disorder where the patient develops multiple discrete cutaneous and subcutaneous masses called neurofibromas)‚ etc. These conditions are usually clinically obvious.

It is very unusual for sarcomas to run in families, although such instances are not totally unknown. Most cases of sarcoma in general, have no known cause. The best explanation available now is that, in most cases, it’s merely a matter of chance (i.e. they are sporadic or of spontaneous origin). In such a situation, the risk to any member of the family (be it, a sibling‚ offspring‚ parent or any other blood relative)‚ is for all practical purposes‚ similar to that of an unrelated person. However‚ there are certain rare genetic disorders that run in families, which have been associated with an increased risk of malignant tumors‚ e.g. Neurofibromatosis type 1‚ Li-Fraumeni syndrome‚ etc. Although Neurofibromatosis type 1, as mentioned earlier‚ is a condition that causes the formation of multiple benign nerve sheath tumors in the peripheral nervous system‚ called as neurofibromas‚ one or more of these may undergo malignant transformation into a MPNST (Malignant Peripheral Nerve Sheath Tumor).

In an adult‚ the area most commonly affected by STS is the lower extremity accounting for about 45% of the cases‚ of which about 75% occur above the level of the knee joint. About 15% of cases occur in the upper extremity. Approximately‚ 30% cases occur in the trunk and 9% in the head and neck region. The remaining 1% occurs elsewhere including within visceral organs like lung‚ kidney‚ etc.

The cause of Soft Tissue Tumors in most cases remains unknown. In effect, there is no real way of preventing a Soft Tissue Sarcoma.

The cause of Soft Tissue Tumor, in most cases‚ remains unknown‚ although certain genetic abnormalities are being found in association with some of them. As mentioned earlier, there are some rare genetic disorders that run in some families, which are associated with the formation of Soft Tissue Tumors, including sarcomas. Soft Tissue Sarcomas have been reported in some persons who have been exposed to certain chemicals, in some patients treated with radiation for some other tumor‚ etc. However‚ these represent a small number of the cases. In effect‚ there is no real way of preventing a Soft Tissue Sarcoma.

Soft Tissue Sarcomas can affect people from all classes of life, and it can occur at any age. There is no real way to identify people at risk, except those with rare familial disorders mentioned earlier. Some types of STS are more common in certain age groups.

The most common symptom of a Soft Tissue Tumor is an unexplained, visible or palpable (which can be felt) swelling, which may be painful to touch or, more often, completely painless. Some tumors may present with pain in the affected area, or a previously painless swelling may become painful. Other than these two symptoms, a Soft Tissue Tumor may cause symptoms due to its location and the related functional disruption. It is important to know that, not all soft tissue swellings are due to tumors.

The most common symptom of a Soft Tissue Tumor is an unexplained, visible or palpable (which can be felt) swelling. It may be hard or soft. It may be painful to touch or, more often, completely painless. In fact, in most cases they begin as a painless swelling and continue to remain so even when they are growing. It becomes painful when it starts to stretch the surrounding tissue or, compress or stretch a nerve. Usually they are single. However, in some benign tumor conditions, more than one such swelling may be found in the same or/and different regions of the body. The size of the swelling may increase slowly or rapidly. A rapidly growing swelling is more likely to be a sarcoma.

Some tumors may present with pain Some tumors may present with pain in the affected area, or a previously painless swelling may become painful. Some tumors may be noticed following an injury. Usually this is because the injury draws attention to the tumor; injury itself is not known to cause the formation of a tumor.

The most common symptom of a Soft Tissue Tumor is an unexplained, visible or palpable (which can be felt) swelling, which may be painful to touch or, more often, completely painless. Some tumors may present with pain in the affected area, or a previously painless swelling may become painful. Other than these two symptoms, a Soft Tissue Tumor may cause symptoms due to its location and the related functional disruption. It is important to know that, not all soft tissue swellings are due to tumors.

The most common symptom of a Soft Tissue Tumor is an unexplained, visible or palpable (which can be felt) swelling. It may be hard or soft. It may be painful to touch or, more often, completely painless. In fact, in most cases they begin as a painless swelling and continue to remain so even when they are growing. It becomes painful when it starts to stretch the surrounding tissue or, compress or stretch a nerve. Usually they are single. However, in some benign tumor conditions, more than one such swelling may be found in the same or/and different regions of the body. The size of the swelling may increase slowly or rapidly. A rapidly growing swelling is more likely to be a sarcoma. Some tumors may present with pain in the affected area, or a previously painless swelling may become painful. Some tumors may be noticed following an injury. Usually this is because the injury draws attention to the tumor; injury itself is not known to cause the formation of a tumor. Other than these two symptoms, a Soft Tissue Tumor may cause symptoms due to its location and the related functional disruption. For example: A tumor around a joint may cause limitation of the movement of that joint, either due to pain or due to the mechanical obstruction caused by the swelling. A tumor arising from a nerve, or compressing a nerve, can cause weakness of some muscles and/or tingling/numbness of the skin in some areas. In certain cases, such an involvement of a nerve in a tumor can cause severe pain. It is important to know that not all soft tissue swellings are due to tumors. For example: Some of the swelling may be due to an abscess (pus collection), often due to tuberculosis (called as a ‘cold abscess’). Sometimes, tuberculosis can cause some of the lymph nodes to swell up and appear as a lump which may be mistaken for a tumor. A collection of blood (haematoma) in the muscle or fat tissue can mimic a tumor. - What are the tests done to diagnose a Soft Tissue Tumor?

It is important to know that not all soft tissue swellings are due to tumors. For example: Some of the swelling may be due to an abscess (pus collection), often due to tuberculosis (called as a ‘cold abscess’). Sometimes, tuberculosis can cause some of the lymph nodes to swell up and appear as a lump which may be mistaken for a tumor. A collection of blood (haematoma) in the muscle or fat tissue can mimic a tumor.

The general approach to the diagnosis of a Soft Tissue Tumor is clinical evaluation, radiological investigations and biopsy.

This involves two steps. First, a detailed account is taken of the present complaint and of any other relevant medical/surgical history of the patient. The second step involves a detailed clinical examination of the affected region and also a complete physical examination of the patient.

Clinical evaluation for a Soft Tissue Tumor is the same as that for any swelling/pain in the body. It has two major components.

• 1.History: The doctor takes a detailed account of the patient’s present complaint, e.g. duration of the symptoms, location of the problem, rate of growth of any swelling, history of any past treatment for the same, etc. The doctor also enquires about any other relevant past or current medical/surgical history, family history, etc., etc.
• 2.Clinical Examination: Clinical examination: The doctor makes a detailed examination of the area with the pain and/or swelling, noting the exact anatomic location, the shape and size of any swelling, its relationship to the surrounding structures, the feel of the mass, etc., etc. This is called as ‘local examination’. Following the examination of the local area, the doctor does a complete ‘physical examination’ of the patient, to look for any other swelling or other relevant findings elsewhere in the body, and also, to assess the general health of the patient.

Clinical evaluation gives the doctor an idea of the situation and guides the line of management, which could be, observation only, further investigation, or medical/surgical treatment.

The common radiological investigations which can be done for Soft Tissue Tumors are X-ray, CT scan, ultrasonography and MRI. Of all these investigations, the most important and the most useful study is the MRI.

The common radiological investigations which can be done for Soft Tissue Tumors are X-ray‚ CT scan‚ ultrasonography and MRI. Of all these investigations‚ the most important and the most useful study is the MRI. With MRI, the size of the disease‚ areas involved by the disease‚ its relationship to the surrounding important structures‚ the presence of satellite tumors‚ etc. can be determined in fine detail. The best thing about MRI is that‚ it provides these detailed images in multiple planes, which help in understanding the three dimensional anatomy of the tumor and its relationship to the surrounding structures‚ which is crucial to the treatment planning. In addition to that, MRI gives a fair idea about the nature of the tissue within the tumor. Based on the clinical details and the information obtained with the MRI, it is often possible to narrow down the list of likely diagnosis. MRI is also very useful in planning the approach to the biopsy of the tumor‚ especially in situations where the disease is deep‚ or in a complex location.

The need for this investigation is best decided by your treating doctor. Generally, it is always a wise thing to get an MRI, if a surgery for the removal of the tumor is planned, as it is of immense help in the surgical planning. Whenever a Soft Tissue Tumor is found to be malignant, and surgical treatment is planned, an MRI is absolutely essential.

The need for this investigation is best decided by your treating doctor. Generally, it is always a wise thing to do an MRI‚ if a surgery for the removal of the tumor is planned, as it is of immense help in the surgical planning. Sometimes, when the tumor is just under the skin and is small, your doctor may decide to avoid an MRI‚ to save cost. There are some Soft Tissue Tumors/swellings where the diagnosis is clinically obvious, e.g. sub-cutaneous lipoma‚ neurofibroma‚ ganglion‚ etc.; in such situations, an MRI may be avoided. In some cases, MRI itself may provide the diagnosis. Whenever a Soft Tissue Tumor is found to be malignant‚ and surgical treatment is planned, an MRI is absolutely essential. It is advantageous to get the MRI before the biopsy of any tumor‚ as it helps in planning the biopsy. A well planned biopsy ensures ideal location of the biopsy incision and track, which in turn makes it easy for the removal of the biopsy scar and track along with the rest of the tumor‚ during the final definitive surgery.

Biopsy is a minor surgical procedure in which a small piece of the tumor tissue is sampled and sent for evaluation to a pathologist, who examines it under a microscope to establish its exact histological identity/name (diagnosis).

An improperly done biopsy can affect the future treatment of the disease, especially if it turns out to be a sarcoma. Leaving behind the biopsy scar and track, at the time of definitive surgery for removal of a sarcoma, increases the risk of local recurrence of the disease significantly. It is therefore very important that the biopsy is conducted in a planned manner, such that, it causes minimal contamination of the biopsy track with tumor tissue/cells. Also, the biopsy incision should be placed in an ideal location which would allow easy removal of its scar and track along with the tumor, during the definitive surgery for the sarcoma. Biopsy is therefore, best done by a surgeon who specializes in Soft Tissue Tumor treatment.

Biopsy is a minor surgical procedure where a small piece of the tumor tissue is sampled and sent to the laboratory for evaluation by a pathologist. The pathologist, after processing the tissue sample, examines thin slices of it under a microscope to identify what kind of tissue it is, i.e. whether it is tumor tissue or not; if it is tumor tissue, whether it is a benign tumor or a malignant tumor, and in either case, exactly what tumor it is (i.e. its exact histological identity/name). In the case of malignant tumors, the pathologist can also opine on the Grade of the tumor (whenever relevant), which is of value in ‘Staging’ the disease.

An improperly done biopsy can affect the future treatment of the Soft Tissue Tumor, especially if it turns out to be a malignant disease. It is a rule in musculoskeletal tumor surgery to remove the scar of biopsy along with the biopsy track (with a margin of surrounding normal tissue) en masse with the tumor during the definitive surgery for any sarcoma, as it is considered to be a contaminated zone (an area which has come in contact with tumor tissue and therefore, could now be seeded with tumor cells). It is well documented that, leaving behind the biopsy scar and track, at the time of the definitive surgery for the removal of a sarcoma, increases the risk of local recurrence of the disease significantly. This makes it essential that the biopsy is conducted in a well-planned and careful manner, ensuring the least possible contamination of the normal tissues forming the biopsy track (tissues through which the biopsy track cuts through), with the tumor tissue/cells. It is vital that the biopsy incision is placed in an ideal location, which would allow the biopsy scar and track to be easily included for removal, along with the tumor, during the definitive surgery. An MRI is very helpful in planning the optimal location and approach of the biopsy. An incorrect biopsy location/approach, or an improperly done biopsy, may force the surgeon to remove important tissues (which could otherwise have been saved) along with the tumor, just to include the biopsy scar and track in the resection (to achieve en bloc excision with a wide margin). This could either result in a loss of useful function, or may necessitate complex reconstruction procedures, involving plastic surgeons, vascular surgeons, etc., to attempt to restore useful function and/or save the limb. It is well documented that in some unfortunate situations, a poorly done biopsy procedure may leave no treatment optionexcept amputation, in an otherwise perfectly salvageable extremity. Biopsy is therefore, best done by a surgeon who specializes in Soft Tissue Sarcoma treatment.

Several biopsy techniques are available, including Fine Needle Aspiration Biopsy, Core Needle Biopsy, Incisional Biopsy, Excisional Biopsy, etc. Biopsy for Soft Tissue Tumors is generally done as a small open surgery (Open Biopsy) or with the help of special biopsy needles (Core Needle Biopsy). Core Needle Biopsy is generally the preferred method for doing a biopsy as it is a simple, reliable, minimally invasive, safe and quick procedure. The correct method for your situation is best decided by your doctor.

‘Grade’ of the tumor is a histological calculation of the aggressiveness of a malignant tumor (more specifically, its ability to metastasize, i.e. the ability to spread to other regions of the body). Grading of the tumor is done by the pathologist based on the histological observations made on the tumor tissue. This information tells the clinician about the extent of risk, the patient has, of developing metastasis. There are several different systems for grading a malignant tumor.

‘Grade’ of the tumor is a histological calculation of the aggressiveness of a malignant tumor (more specifically, its ability to metastasize, i.e. the ability to spread to other regions of the body). Using formal criteria, the pathologist, after observing thin slices of the disease tissue under a microscope, allots a particular grade to that tumor. This information tells the clinician about the extent of risk, the patient has, for developing metastasis. The grade for a particular tumor may differ according to what system of grading is followed by the pathologist. Musculoskeletal Tumor Society (MSTS) system classifies malignant bone tumors as either low-grade (G1) or high-grade (G2). American Joint Committee on Cancer (AJCC) System (also called the TNM System) classifies these tumors into four grades - G1 (well differentiated), G2 (moderately differentiated), G3 (poorly differentiated) and G4 (undifferentiated). G1 and G2 tumors of AJCC system is considered equivalent to the G1 tumors of MSTS system (low-grade tumors), while G3 and G4 tumors of AJCC system are considered equivalent to the G2 tumors of MSTS system (high-grade tumors). Not all tumors are graded, as some tumors invariably show consistent behavior, e.g. Ewing’s Sarcoma (Ewing’s Sarcoma is always a high grade disease. There is nothing like a low grade Ewing’s Sarcoma. Therefore, grading of Ewing’s Sarcoma is of no practical value, as just the diagnosis of Ewing’s Sarcoma implies a high grade disease.).

Core Needle Biopsy in skilled hands, offers significant advantages over Open Biopsy and therefore, it is the preferred method for obtaining tumor tissue samples.

The goal of a biopsy is to obtain a sample of tumor tissue so that it can be studied by the pathologist to identify the exact nature of the disease and establish its identity (diagnosis), as treatment of any tumor depends on its diagnosis. Generally, it is possible to obtain a larger quantity of tissue sample (which makes it easier for the pathologist to process and study the material) through an Open Biopsy. However, the material obtained by Core Needle Biopsy in most cases is adequate for a skilled pathologist. In many other ways, the advantages and benefits offered by a CNB are superior to that of an OB. As mentioned earlier, CNB is a simple, reliable, minimally-invasive, safe and quick procedure. In the following table, a comparison is made between OB and CNB.

It is quite obvious from the above comparison that Core Needle Biopsy in skilled hands‚ offers significant advantages over Open Biopsy and therefore‚ it is the preferred method for obtaining tumor tissue samples. Nevertheless‚ there may be some situations where an open biopsy procedure could be the more suitable option.

There are some soft tissue swellings where the diagnosis is obvious clinically, or on imaging. All of these are well known benign conditions. When surgical treatment for such a swelling or tumor is considered, a biopsy may be avoided. Barring these, a biopsy is a must before doing a definitive surgery for the removal of any Soft Tissue Tumors.

As we discussed in the paragraph on MRI, there are some soft tissue swellings where the diagnosis is obvious clinically, or on imaging. All of these are well known benign conditions. When surgical treatment for such a swelling or tumor is considered, a biopsy may be avoided. However, if there is even the slightest suspicion about the nature of the swelling, it is a wise thing to do a biopsy, to be certain about what it is, before proceeding with the surgical treatment. When a surgical procedure is planned for any Soft Tissue Tumors, it is very important to know what the tumor is. An unplanned surgery, where the nature of the swelling and its anatomical extent is unknown, has a high risk of resulting in an unsatisfactory procedure, either in the form of an incomplete surgery, or as a surgery with poor or contaminated margins. The consequences of these would be disastrous, if the tumor turns out to be a Soft Tissue Sarcoma. Therefore, barring a few situations, a biopsy is a must before doing a definitive surgery for the removal of any Soft Tissue Tumors. If the tumor is found to be malignant, the next step is to ‘Stage’ the cancer.

In simple words, staging is a system to determine the extent of cancer in the patient and the level of risk to the patient due to it. Stage of the cancer at diagnosis, is the best predictor of survival and is a powerful guide to the optimal treatment of the disease. One of the most important pieces of information derived from staging investigations is whether the disease is localized or metastatic. The importance of staging is that, treatment strategies can be specifically planned to the patient’s situation, to get the best outcome.

In simple words, staging is a system to determine the extent of cancer in the patient and the level of risk to the patient due to it. Stage of the cancer at diagnosis, is the best predictor of survival and is a powerful guide to the optimal treatment of the disease. In cancer management, after a diagnosis of cancer is made, further tests are carried out to ‘Stage’ the cancer. Staging may involve several investigations like histopathology, MRI, CT scan, Bone scan, PET scan, etc. The exact set of investigations varies from case to case and will be decided by your treating doctor. Staging criteria may differ slightly for different tumors. One of the most important pieces of information derived from staging investigations is whether the disease is localized or metastatic. It is possible that the patient may have the disease in other parts of the body but is not aware of it as they are small in size and therefore, are not causing any problem at the moment. Staging investigations help in locating and identifying these metastatic foci of disease early. This helps in choosing the most useful and effective treatment approach to such a patient, giving due consideration to the presence of metastasis and its treatment. The importance of staging is that, treatment strategies can be specifically planned to the patient’s situation, based on the stage of the disease, to get the best outcome. For Soft Tissue Sarcomas, there are two major staging systems. One is the MSTS (Musculoskeletal Tumor Society) staging system, which classifies these conditions into three stages, with stage ‘I&rsquo being the lowest, offering the best prognosis to the patient and stage ‘III&rsquo representing the highest, indicating metastatic disease and consequent poor prognosis. The other staging system is the AJCC (American Joint Committee on Cancer) staging system (also called the TNM System) which classifies these diseases into four stages with stage ‘I&rsquo being the lowest, offering the best prospect for recovery and stage ‘IV’ denoting the highest, indicating metastatic disease, suggesting the least chance of a favourable outcome.

‘Grade’ of the tumor tells us how aggressive the disease is; especially about its tendency to metastasize (spread to other regions of the body). However, it does not tell us anything about the extent of its presence in the patient and the actual threat it poses to the patient, at any given time.

‘Staging’, on the other hand, is a system that helps in understanding the extent of cancer in the patient and the level of risk to the patient due to it. ‘Stage of the cancer at diagnosis’, is the best predictor of survival and is a powerful guide to the optimal treatment of the disease.

As described earlier, ‘Grade’ of the tumor is a value allotted to the tumor by a pathologist based on its appearance under the microscope (sometimes, in certain borderline cases, clinical and radiological information may also be used while allotting the grade to the disease). It tells us how aggressive the disease is; especially about its tendency to metastasize (spread to other regions of the body). However it does not tell us anything about the extent of its presence in the patient and the actual threat it poses to the patient at any given time. Grade of the disease is just one of the criteria used in staging of cancer. Staging systems take into account several other details to designate the ‘Stage’ of the disease.

Whenever chemotherapy is employed in the treatment of a bone sarcoma, where the intention of treatment is to ‘Cure’ the patient, it is never the only treatment. Generally, the treatment begins with chemotherapy. This is called as neoadjuvant chemotherapy (chemotherapy given before the local management of a cancer). After a few cycles of neoadjuvant chemotherapy (the number of cycles depends on the chemotherapy regimen being used), the diseased area is surgically removed with wide margins (Wide Excision), and reconstructed appropriately (whenever indicated). A few days after the surgery, chemotherapy is resumed again and is continued until the entire regimen is completed, which may involve several more cycles of chemotherapy. This is called as adjuvant chemotherapy (chemotherapy given after the local management of a cancer). In some rare situations where surgery is impossible, or is very risky, and the sarcoma is sensitive to radiotherapy, instead of surgery, the patient may be treated with definitive radiotherapy along with the chemotherapy. This is especially true in the treatment of Ewing’s sarcoma affecting the spine, sacrum, etc. In some cases of bone sarcoma, radiotherapy may be used in addition to the chemotherapy and surgery.

For example, let us consider the situation of two patients with Soft Tissue Sarcoma; ‘Patient A’ and ‘Patient B’. A biopsy revealed that both have Ewing’s Sarcoma. (There is no grading system for Ewing’s sarcoma as it is always a high grade disease.). Thus, both patients have identical disease, i.e. Soft Tissue Ewing’s sarcoma; in which case it is logical to assume that both ‘Patient A’ and ‘Patient B’ will receive the same treatment. However, that is not correct. We still do not have the complete information necessary for formulating the right treatment plan. We know that cancer can spread to other organs and tissues. It is for this reason, staging investigations are done. Staging investigations revealed that ‘Patient A’ has no metastasis (i.e. he has localized disease only) whereas ‘Patient B’ has metastases in the lungs; which means ‘Patient A’ has ‘Stage II’(for a high grade tumor, there is no ‘Stage I’) disease while ‘Patient B’ has ‘Stage IV’ disease. Clearly, the same treatment strategy will not work for both patients as the extent of disease in both patients is different. So, despite having the same high grade disease, ‘Patient B’ with ‘Stage IV’ disease is at a higher risk as compared to ‘Patient A’ with ‘Stage II ’ disease, and therefore needs a different treatment approach. This is essentially the difference between ‘Grade’ and ‘Stage’ of the disease. Thus, staging is a system that helps in understanding the extent of the cancer in the patient and the level of risk to the patient due to it. Stage of the cancer at diagnosis, is the best predictor of survival and is a powerful guide to the optimal treatment of the disease.

Treatment strategies are different for patients in different stages of the disease, including those with localized cancer and those who have metastatic cancer. By considering various criteria, staging not only helps in identifying patients with metastatic disease, but it also helps in identifying people with non-metastatic disease who have a higher risk than some others with localized disease. Knowing that a patient has metastatic sarcoma makes it possible to employ a more aggressive treatment plan to deal with the metastases along with the primary tumor. The stage of the disease at diagnosis is one of the important details, which help in predicting the likelihood of recovering from the condition.

Treatment strategies are different for patients in different stages of the disease, including those with localized cancer and those who have metastatic cancer. By considering various criteria, staging not only helps in identifying patients with metastatic disease, but it also helps in identifying people with non-metastatic disease who have a higher risk than some others with localized disease. Knowing that a patient has metastatic sarcoma makes it possible to employ a more aggressive treatment plan to deal with the metastases along with the primary tumor. The stage of the disease at diagnosis is one of the important details, which help in predicting the likelihood of recovering from the condition.

The need for staging is obvious once you understand the behavior of malignant tumors. Malignant tumors are those tumors which have the ability to spread locally (invasion), and to distant organs and tissues (metastasis). Treatment strategies are different for patients in different stages of the disease, including those with localized cancer (non-metastatic cancer – disease, which has not spread to distant locations) and those having metastatic disease (cancer which has spread to distant organs/tissues). By considering various criteria, staging not only helps in identifying patients with metastatic disease, but also helps in identifying people with non-metastatic disease who are at a higher risk than some others with localized disease (e.g. due to a higher grade of tumor, larger size of the disease, etc.). AJCC ‘Stage I’, ‘Stage II’ and ‘Stage III’ disease, all represent localized sarcoma, but with increasing levels of risk to the patient due to higher grade of disease, larger size of disease, deeper location of disease, etc. A patient with localized disease, but with a higher stage, is at a higher risk of failure of treatment as compared to one with a lower stage, and therefore, requires more radical or aggressive treatment.

Knowing that a patient has metastatic sarcoma makes it possible to employ a more aggressive treatment plan to deal with the metastases along with the primary tumor. If staging investigations are not done, metastasis (if any) may remain unknown for a long time, until it becomes large and causes symptoms. A potentially treatable metastasis may thus become untreatable, if not detected in time and treated appropriately. Sometimes, in patients with extensive disease, staging may help avoid unnecessary unhelpful treatment. The stage of the disease at diagnosis is one of the important details, which helps in predicting the likelihood of recovering from the condition. Generally, lower the stage of the disease, better is the prognosis; higher the stage, worse is the likely outcome.

Treatment for a Benign Soft Tissue Tumor can begin as soon as the diagnosis is established, but treatment for a Soft Tissue Sarcoma should not be started until staging is over. If treatment for a malignant tumor is started without staging the disease, it may result in improper therapy as vital information regarding the extent of the disease in the patient will not have been considered in the planning of that treatment.

Treatment for a Benign Soft Tissue Tumor can begin as soon as the diagnosis is established, but treatment for a Soft Tissue Sarcoma (STS) should not be started until staging is over. Armed with the information regarding the nature of the disease, its location and size, the extent of its presence in the patient, age of the patient, the general health of the patient, etc.‚ a treatment strategy is planned, to give the maximum benefit to the patient in terms of recovering from the disease and preservation of useful function.

If treatment for a malignant tumor is started without staging the disease, it may result in improper therapy as vital information regarding the extent of the disease in the patient will not have been considered in the planning of that treatment. It is important that staging is done before beginning the treatment because treatment can lower the stage of the disease. For example, let us consider the situation of a patient with Soft Tissue Ewing’s sarcoma of the thigh, who also has lung metastasis, i.e. with ‘Stage IV’ disease. By doing the staging before starting the treatment, the most appropriate treatment strategy for this patient can be employed, which also includes the treatment of the metastasis. But if the treatment is started before staging the disease, the initial treatment with neoadjuvant chemotherapy would shrink the primary tumor and the tumor metastases. In many cases it can cause the metastasis to disappear. If this patient is staged now, no metastasis will be found, and one would erroneously consider him to be at a lower stage (e.g. ‘Stage II’),‚ which would advocate the use of a different, less aggressive, treatment strategy than what was actually needed for this patient. Thus, staging the patient after starting the treatment could completely change the disease picture and might result in sub-optimal treatment. It is therefore, important to remember that the real value of staging is only when it is at diagnosis, and not after starting treatment.

This depends on the nature, location, size, etc. of the soft tissue tumor. A small superficial Benign Soft Tissue Tumor which is not causing any symptoms and is not growing or evolving in any way, may be just observed; whereas a benign tumor causing symptoms, or is growing, may be considered for treatment. Surgery is the treatment of choice for most Benign Soft Tissue Tumor.

This depends on the nature, location, size, etc. of the soft tissue tumor. A small superficial benign Soft Tissue Tumor which is not causing any symptoms and, which has been present for a long time and is not growing or evolving in any way, may be just observed; whereas a benign tumor causing symptoms, or is growing, may be considered for treatment. Surgery is the treatment of choice for most Benign Soft Tissue Tumors. The plan for surgical treatment should take into account the exact diagnosis of the tumor, its anatomical location, its relationship to the surrounding structures, etc. All care is taken to preserve important nerves and blood vessels, even if they are in close proximity to the tumor, so that maximum function is retained and the limb is not endangered in any way. In general, with benign tumors, a small risk of recurrence of the disease is acceptable if that risk allows the preservation of useful function. This is because, in benign tumors, the risk of disease recurrence is only local (i.e., in the area of previous disease), which, if it happens, can be surgically removed again without posing a risk to the life or limb of the patient. In most Benign Soft Tissue Tumors, there is no role of chemotherapy or radiotherapy. However, there are some rare situations where chemotherapy and/or radiotherapy may be used in the treatment of certain benign soft tissue tumors.

Surgery‚ in the form of ‘Wide Excision’ of the tumor is the primary treatment for most Soft Tissue Sarcomas. Generally (not always), in cases of STS‚ radiotherapy is a part of the treatment and is usually administered to the affected area after the surgical excision. In some STS‚ chemotherapy also plays an important role in the treatment. Rarely‚ all three modalities may be employed in the treatment of some STS.

In any cancer treatment,‚ the first thing to be decided, is the ‘Goal’ of treatment; i.e. whether the goal is to achieve ‘Cure’ or ‘Palliation’. This depends on various factors like‚ the extent of the disease‚ the stage of the tumor‚ the general health of the patient‚ etc. Asisevident to all, the goal of ‘Cure’ implies that the treatment is directed at complete eradication of the disease from the patient and ensuring the lowest possible risk of relapse of the cancer. On the other hand, ‘Palliation’ implies partial treatment of the condition‚ the goal being‚ to control the disease (not cure)‚ so as to ease the symptoms and discomfort caused by it, or‚ to slow the progression of the disease‚ so as to avoid or delay the adverse complications due to it; thus offering the patient better survival and better quality of life.

Surgery is the primary treatment for most Soft Tissue Sarcomas. Surgery for a STS always includes a margin of normal tissues in the excision, along with the biopsy scar and biopsy tract; the whole thing is removed as one single piece of tissue (en bloc). This is called as Wide Excision of the tumor. ‘Wide Excision’ means surgical removal of the tumor tissue (with the scar and track of biopsy)‚ along with a good margin of surrounding healthy normal tissue, in one single piece. This is done with the intention of ensuring the complete removal of the cancer tissue. Cancer begins with just one abnormal cell that eventually forms the tumor, which is made of billions of copies of this original parent cell. Therefore‚ hypothetically speaking‚ just leaving one cancer cell behind is enough for this tumor to grow back again. Thus‚ in the surgery for a Soft Tissue Sarcoma, there is no justification for removing the tumor in pieces, as doing so defeats the very purpose of the surgery. (Even when the surgery is done with a palliative intention, the tumor is removed with a wide margin, unless such a surgery is likely to cause more morbidity‚ disability, and discomfort to that patient.). When important structures like blood vessels‚ nerves‚ bone‚ joint‚ skin‚ etc. are in close proximity to the tumor‚ the operating surgeon has to take crucial decisions on whether to preserve them or sacrifice them, based on various details like‚ the nature of the tumor‚ its grade‚ size‚ location‚ etc. These decisions are usually taken before surgery‚ based on the clinical findings‚ MRI images‚ pathology findings‚ etc.‚ and appropriate plans are made to repair or reconstruct the damaged/sacrificed vital structure/s. This may involve the role of other specialists, like plastic surgeons, vascular surgeons, etc.‚ in the surgery. In some situations, where such repair or reconstruction options are not available/possible/feasible‚ amputation of the limb may be considered.

Generally (not always), in cases of STS‚ radiotherapy is a part of the treatment.

Radiotherapy is a form of cancer treatment where a certain type of energy called as ‘Ionizing radiation’ is used to kill/damage cancer cells and shrink tumors. ‘Ionizing radiation’ is made of either powerful electromagnetic rays (X-rays/gamma rays)‚ or high energy sub-atomic particles like atomic nuclei‚ protons‚ neutrons‚ etc. Radiation therapy injures or destroys cells in the area being treated by damaging their genetic material (DNA)‚ either killing them or affecting their ability to grow and multiply. Although radiation damages both cancer cells and normal cells‚ cancer cells are much more vulnerable to the damaging effects of radiation, while most normal cells can recover from its effects and function properly. This is one of the reasons why radiation in most cases is given in divided doses over a number of days‚ which gives normal cells/tissues time to repair and recover. This is called as ‘fractionation’ of the radiation dose. The goal of radiation therapy is to damage or destroy as many cancer cells as possible‚ while limiting harm to surrounding healthy tissue. In STS‚ radiation is usually given after the surgery to the region of surgical treatment with the intention of killing any stray cancer cell or cells‚ which may have been inadvertently left behind‚ thus providing additional security against the possibility of local recurrence of the disease. This is called as adjuvant radiotherapy. Adjuvant radiotherapy may be given as ‘External Beam Radiotherapy’ or as ‘Brachytherapy’, or sometimes, both. In some cases radiotherapy may be given before surgery to shrink the size of the tumor, to make surgery safer and easier. This is called as NeoadjuvantRadiotherapy.

In some STS‚ chemotherapy has an important role. Whenever chemotherapy is a part of the treatment‚ it is usually started first. This is called as NeoadjuvantChemotherapy. This shrinks the tumor‚ which becomes smaller in size and bulk. It also reduces the vascularity of the mass, i.e. it reduces the amount of blood circulating through the mass at any given time. This reduces the blood loss during surgery. All of this makes surgery simpler and safer. Wide Excision surgery is done after a few cycles of chemotherapy. Chemotherapy is again resumed‚ a few days after the surgery. This is now called as Adjuvant Chemotherapy.

Sometimes all three modalities of treatment (i.e. surgery‚ radiotherapy and chemotherapy) may be used to treat a particular sarcoma.

When the intention of care is ‘palliative’‚ although surgery certainly has an important role in such a treatment‚ often the treatment may be limited to just radiotherapy‚ or in some cases‚ radiotherapy with chemotherapy.

The decision regarding the treatment plan is taken by your treating doctor based on all the available information.

Soft Tissue Tumor can recur after successful completion of treatment. Benign Soft Tissue Tumors recur only locally (i.e., in the region where it was previously located)‚ and this risk is generally very low with most of them. Soft Tissue Sarcomas‚ on the other hand, carry a significant risk of recurrence of disease in the operated area (local recurrence) and/or metastatic disease in other parts of the body, at some time in the future. This risk of relapse of Soft Tissue Sarcoma is highest in the first 2 to 3 years following the treatment. After this duration‚ the risk falls considerably.

Soft Tissue Tumor can recur after successful completion of treatment. The recurrence could be local, regional or distant.

Benign tumors only have a risk of local recurrence. This risk is generally very low with most Benign Soft Tissue Tumor. However, there are some‚ which have a high rate of local recurrence‚ e.g. Fibromatosis. Some of these locally aggressive tumors may be surgically removed with a margin of normal tissue to reduce this risk. In some situations‚ treatment of such benign aggressive tumors may also involve the use of radiotherapy or/and chemotherapy.

Risk of recurrence of disease is much higher with malignant tumors. Despite receiving the best of care and completing the treatment successfully‚ there still remains a possibility of recurrence of the sarcoma at some time in the future. Recurrence is also called as ‘relapse’ of the cancer. This could be‚ as mentioned earlier‚ in the form of:

Local recurrence: Disease recurs in the region of its previous presence‚ i.e.‚ in the operated/treated area.

Regional recurrence: Disease recurs in the regional draining lymph nodes (also called as lymph node metastasis or nodal metastasis) or‚ in the tissues close to the region of the previous surgery‚ but not in the previously operated area. Lymph node metastasis is very rare in sarcomas. Some sarcomas like Epithelioid sarcoma‚ rhabdomyosarcoma‚ etc. show a slightly higher incidence of lymph node metastasis as compared to the other sarcomas.

Distant recurrence: The disease recurs in distant organs or tissues. This is also called as systemic metastasis. Most of the metastases in sarcomas occur in the lungs. It can also metastasize to the bones or to other organs like the liver‚ brain‚ etc.

The relapse of sarcoma may appear singly‚ either as a local recurrence or metastasis‚ or it may appear in any possible combination of the above mentioned recurrence patterns‚ either at the same time or at different times. For example‚ a person with local recurrence of disease may also have metastasis at the same time or at some time in the future. Conversely‚ some patients‚ who developed metastasis, may eventually show local recurrence of the disease. Thus there is not fixed pattern of the relapse in cancer. A person with metastatic disease may have a single metastasis‚ or have multiple metastases in many tissues/organs. The number of metastasis may remain unchanged‚ or it may increase‚ with time.

The benefit of optimal treatment is that it reduces this risk of relapse of the disease to a large degree; however, this risk still remains significant. It is, therefore‚ important to keep a regular follow-up with your doctor as per his/her advice and undergo regular clinical and radiological examinations as advised. Recurrence of disease‚ if any‚ if detected in time‚ may be treatable.

This risk of relapse in Soft Tissue Sarcoma is highest in the first 2 to 3 years following the treatment. After this duration‚ the risk falls considerably.

Relapse of sarcoma cannot be prevented with 100% certainty. However‚ by following the proper method for diagnosing‚ staging and treating the tumor‚ the risk of recurrence of the disease can be reduced to a large extent.

Relapse of sarcoma cannot be prevented with 100% certainty as there are several factors‚ which contribute to this risk that cannot be controlled. However‚ by following the proper method for diagnosing‚ staging and treating the tumor‚ the risk of recurrence of the disease can be reduced to a large extent. Excellent local treatment of a non-metastatic malignant tumor‚ in addition to reducing the risk of local recurrence‚ also lowers the risk of developing metastasis. In Soft Tissue Sarcomas‚ this is usually achieved with surgery and radiotherapy. In cases where the tumor is sensitive to chemotherapy‚ addition of chemotherapy to the treatment also helps in reducing the risk of local recurrence and metastasis.

Limb salvage surgery means saving the limb while effectively treating the malignant tumor affecting it. Though this is a term more appropriately used with the treatment of bone sarcomas, it is also relevant to the management of Soft Tissue Sarcomas affecting the extremities. Situations where earlier amputation was the only option of treatment (e.g., in cases with the tumor involving the major blood vessels or adjacent bone and joint‚ etc.)‚ can now be seriously considered for limb salvage‚ with the help of a multidisciplinary surgical team‚ which may include specialists like plastic surgeons‚ vascular surgeons‚ etc.

Although this is a term more appropriately used with the management of bone sarcomas‚ it is also relevant to the management of Soft Tissue Sarcomas affecting the extremities. Earlier‚ for large Soft Tissue Sarcomas involving the blood vessels‚ major nerves‚ or bone‚ amputation was often the only safe surgical treatment possible. However‚ the availability of excellent imaging technologies like MRI‚ adjuvant treatment like radiotherapy (and sometimes chemotherapy)‚ and the advances in orthopaedic‚ vascular and plastic surgery‚ etc.‚ has now made it possible to do Limb Salvage Surgery in many situations where‚ earlier‚ amputation was considered as the only appropriate treatment. In cases where blood vessels are involved by the disease‚ entire segments of the blood vessels can be removed with the tumor and reconstructed using synthetic vascular grafts. In situations where nerves are involved by the disease‚ nerve grafts can be placed to bridge the gap in the nerve following the resection‚ which may eventually result in recovery of useful muscle function. Besides this‚ procedures like tendon transfers‚ etc., in suitable cases‚ can restore useful voluntary range of joint function. In patients where the Soft Tissue Sarcoma has invaded the bone‚ the affected bone (most often‚ only a part of the bone) can be removed with the tumor‚ followed by the skeletal reconstruction with appropriate metallic implants, etc. In situations where there is an extensive loss of skin following the removal of tumor, plastic surgeons can provide wound cover using a variety of techniques ranging from local flaps‚ skin grafts‚ to microvascular free flaps.

Limb Salvage Surgery has come a long way in the management of extremity malignant tumors and has now reached a stage where it is the norm, representing one of the finest achievements of modern technology and medicine‚ offering the possibility of functional limb preservation in more than 80% of these patients without compromising on the overall treatment of their disease.

A successful completion of treatment for cancer does not mean that the patient is cured of the disease. The patient is said to be in remission. It just means there is no detectable cancer in the body. In a significant number of patients‚ cancer can come back. It is important to detect any recurrence of the disease early. The importance of follow-up is that early detection and timely treatment of the recurrent disease (whenever indicated)‚ can improve survival. Benign Soft Tissue Tumors generally do not require intensive follow-up except in cases with a known high risk for local recurrence‚ e.g. Fibromatosis.

A successful completion of treatment for cancer does not mean that the patient is cured of the disease. The patient is said to be in remission‚ which means there is no sign or symptom of cancer in the patient’s body. It does not necessarily mean there is no cancer in the body. It just means there is no detectable cancer in the patient. In a significant number of patients‚ cancer can come back (this is called as a ‘relapse’ of the cancer). It is important to detect any recurrence of the disease early. Following completion of treatment for any sarcoma‚ patients are expected to visit the treating doctor at regular intervals (even when they are feeling fine and have no complaints)‚ during which they undergo a thorough clinical examination and certain investigations to look for any evidence of local, regional or distant recurrence of the disease. These investigations could be blood tests‚ X-ray‚ CT scan‚ MRI‚ sonogram‚ bone scan‚ PET scan‚ etc. These investigations are not done at every visit to the doctor. Generally, in soft tissue sarcomas, follow-up is advised every three months for the first two years, followed by every six months until five years (next three years), and then yearly until ten years (next five years). The importance of follow-up is that early detection and timely treatment of the recurrent disease (whenever indicated), can improve survival. Benign Soft Tissue Tumors generally do not require intensive follow-up except in cases with a known high risk for local recurrence, e.g. Fibromatosis.

Benign:

Locally Aggressive tumors:

Rarely metastasizing tumors:

Malignant: