Risk factors
The causes of soft tissue sarcoma are not precisely known, but some risk factors include:
- undergoing radiotherapy;
- certain genetic diseases, for example: neurofibromatosis;
- Gardner's syndrome;
- Li-Fraumeni syndrome;
- retinoblastoma
- insecticides or herbicides (substances used to kill insects or plants);
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Types of soft tissue sarcomas
Soft tissue sarcomas can be divided into groups depending on how they look under a microscope and what abnormal genes they contain. The most common types of sarcomas are:
- Liposarcomas, which look like fatty tissue.
- Leiomyosarcoma, which looks like smooth muscle: uterus;
- walls of the digestive tract;
- blood vessel walls
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Prognosis for bone sarcoma
The prognosis largely depends on the type of disease, tumor location, stage of the disease, general condition of the patient and his age. With early diagnosis and comprehensive treatment, the survival rate is quite high: 80% in the first stage, 60% in the second, 50% in the third. The lowest survival rate for the fourth stage is no more than 10%.
Prevention of disease. A medical examination every six months, a balanced diet, and feasible physical activity will help you maintain your health for a long time. Avoid injury and do not self-medicate!
Signs and symptoms
Signs and symptoms of soft tissue sarcomas depend on the location of the tumor.
Soft tissue sarcomas that are found in the extremities usually appear as a painless lump. Some symptoms of soft tissue sarcomas:
- numbness or tingling in the arm or leg where the tumor has formed;
- swelling of the arm or leg where the tumor formed;
- pain at the site of the tumor;
- nausea or vomiting;
- a feeling of bloating from the pressure of the tumor on other tissues or nerve endings, if such a tumor is large.
In some cases, a person may notice a lump after injury to a particular area and think that the injury was the cause of the lump. Doctors believe that the injury simply draws attention to the tumor that has already formed.
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How and why do soft tissue sarcomas occur?
The cause of the development of soft tissue sarcomas is unknown, but there is evidence of the unfavorable carcinogenic effect of long-term radiation exposure several decades ago and long-term profound immunodeficiency. During the course of their lives, 99% of people with sarcoma did not experience either one or the other factor.
Slightly less than two people out of every 100 thousand Russians are affected. Some types of neoplasms have unexpressed gender preferences, but there are no clear statistics on occurrence in men and women.
Diagnostics
Your doctor will determine whether it is necessary to biopsy (take a sample) of the tumor. Depending on the location of the tumor, you will have a computed tomography (CT) or magnetic resonance imaging (MRI) scan to determine the size of the tumor and its distance from nearby blood vessels, muscles, or other organs.
If you need a biopsy, this procedure may be done in your doctor's office or Interventional Radiology. The tissue sample will be sent to a pathologist, who will examine it under a microscope.
Unlike other types of cancer, sarcomas do not have different stages. Low-grade sarcomas are associated with a low (less than 5%) risk of metastasis (spread to other parts of the body). High-grade sarcomas have a higher risk of metastasis, most often to the lungs. Sarcomas usually do not spread to the lymph nodes.
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Bone sarcoma - signs of the disease, types of pathology
Osteosarcoma is considered the most aggressive form of the disease and is diagnosed in 50% of cases. The main site of injury is the tubular bones. Bone tumors of the lower extremities most often affect the knee joint.
Ewing's sarcoma is diagnosed between the ages of 5 and 15 years. The disease is accompanied by severe pain and fever. The pathology is characterized by rapid development and metastasis to the liver, lymph nodes and lungs.
Chondrosarcoma is a malignant tumor that affects the femur and pelvic bones. The disease is characterized by late metastases.
Reticulosarcoma is the rarest type, in which bone destruction occurs in small areas. Tubular, spongy and flat bones are susceptible to tumors. The disease is accompanied by enlarged lymph nodes and relatively good health.
Characteristic signs of bone sarcoma disease:
- swelling of the joints;
- sharp pain that intensifies at night (painful sensations are difficult to relieve and tend to progress);
- strengthening of the venous network around the lesion;
- pain on palpation;
- temperature increase;
- limb dysfunction.
Bone sarcoma - stages:
- The first stage is a tumor no more than 8 cm, there is no damage to the lymph nodes and internal organs.
- The second is a tumor larger than 8 cm, no metastases.
- Stage three – the tumor is widespread throughout the bone, there is no damage to internal organs or lymph nodes.
- The fourth is extensive damage to the lymph nodes and internal organs.
Treatment
Your surgeon, oncologist (cancer specialist), and radiation oncologist will work together to develop the best treatment plan for you. They will meet with you and discuss possible treatment options. These specialists will analyze the cell type, grade and location of the tumor before recommending the most appropriate treatment.
Operation
Surgery is the main treatment for soft tissue sarcoma. The purpose of the operation is to excise the tumor, ensuring that all cancerous tissue is removed. Your doctor or nurse will tell you in detail about your upcoming surgery. Your nurse will help you prepare for your surgery and also give you Preparing for Surgery material.
Radiotherapy
Radiotherapy uses powerful x-rays to kill cancer cells. Radiotherapy can be used alone or as an additional treatment before or after surgery.
During a radiotherapy session, the device directs a beam of radiation to the place where the tumor is located. This beam passes through the patient's body and destroys cancer cells in its path.
If radiation therapy is included in your treatment plan, you will receive doses of radiation every weekday for several weeks. Your doctor or nurse will discuss the side effects of radiation with you and teach you how to care for yourself during treatment.
Chemotherapy
Chemotherapy is a treatment with drugs that destroy cancer cells throughout the body. Chemotherapy drugs may be given into a vein (intravenously) or taken by mouth (orally) as tablets. You may be given chemotherapy before surgery to shrink the size of a large tumor and make it easier to remove. Chemotherapy may also be prescribed after surgery.
Chemotherapy can be given using 1 or more drugs. You can undergo it on an outpatient or inpatient basis. Your doctor and nurse will discuss your treatment plan with you. They will also explain possible side effects to you. You will be given additional information about chemotherapy and the National Cancer Institute's brochure, “Chemotherapy and You.”
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Forecasts
The prognosis and chances of cure depend on a number of factors:
- the size of the tumor and its location;
- stage of development of pathology and degree of prevalence of sarcoma, presence of metastatic foci;
- the possibility and feasibility of the operation;
- effectiveness of chemotherapy, radiation therapy;
- age, general health of the patient and the presence of dangerous concomitant or chronic pathologies.
If a localized tumor is detected and there are no metastases, the five-year survival rate of patients is 70%. If malignant cells show susceptibility to cytostatic drugs, this figure increases to 80-90%. If metastases are detected in the bones and bone marrow, the prognosis worsens and, subject to adequate treatment, the five-year survival rate is about 30%. At the same time, we must not forget that any prognosis is very individual, and in each specific medical case this indicator may differ significantly. Source: Survival rate for metastases of sarcoma of unknown origin. Abbasov F.A. Biomedicine No. 4, 2004.
In some cases, after treatment, the malignant tumor returns as a relapse. A new lump can appear either in the same place where the tumor was previously, or in another part of the body, in the area of metastases. Therefore, even after complete recovery, patients must regularly undergo preventive diagnostics for the timely detection of neoplastic processes in the body.
The highest risk of disease recurrence is present in the first 2-3 years after treatment of the primary tumor. It is also possible that the cancer will develop again in the future, but the likelihood of its occurrence is significantly reduced.
Long Term Care
Your health care experience at Memorial Sloan Kettering (MSK) is a long process. You will see your doctor every 4 to 6 months for the first 3 years after treatment ends. At each of these follow-up visits, you will be examined by a doctor and have an X-ray or CT scan.
After the early follow-up period, you will see your doctor every 6 to 12 months for the next 5 years. After this, you will only see your doctor once a year or once every two years.
Your doctors and nurses are committed to your long-term health care. Please contact them at any time during the period of such service if you have any questions or concerns.
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About sarcoma of the hand and medical negligence
Recently I had the rare chance to attend an operation to remove sarcoma of the hand.
A completely wild case, not least because of the narrow-mindedness of some “specialists.” Honestly, there's a whole chain of events here.
A young man, let's call him Maxim, 32 years old. In 2021, I discovered that my hand was swollen. I decided that this was after a recent fall from a bicycle. For some time he waited for it to “go away on its own,” then he finally turned to traumatologists, who began to treat him for tendinitis, tendovaginitis: inflammation of the tendons and synovial membranes, popularly known as “inflammation of the ligaments.” He treated them for a year and changed several hospitals.
When the traumatologists realized that nothing was helping, they suspected phlegmon (extensive purulent inflammation) of the hand, made an incision, “cleaned” it, and bandaged it. The treatment continued, Maxim did bandages - both himself and in various clinics. And only in the second or third medical institution, they finally thought of sending a piece of tissue for histology.
Unfortunately, histology was carried out in the local morphology department, and the hospital was not an oncology one. The histologist had never seen a sarcoma in his life, maybe only at the institute, so he concluded that it was either a hygroma or a chondroblastoma.
The doctors did not try to convey to the patient that this was not a simple purulent boil, but a serious tumor.
Maxim, most likely, was scared; he began to have a denial reaction. And instead of turning to a competent oncologist, he was stuck in this denial. He went to other doctors looking for a second opinion (one that would reassure him).
For a whole year, Maxim was treated for something unknown, because he went to traumatologists - and they don’t often deal with sarcoma, so they decided that this was another patient with trauma. Nobody thought about MRI.
The tumor at this time entered the stage of rapid growth. But for another year, until his hand became the size of a basketball, Maxim convinced himself that it was just inflammation.
Until he finally ended up in the international clinic Medica24, where he was given the correct diagnosis and underwent surgery.
Oncologist Pyotr Sergeevich Sergeev, who invited me, is a specialist in sarcomas, including bone ones. Maxim’s relatives approached him with the words: “You know, the case here is very neglected. And the point is not that there is a tumor, but that Maxim is no longer completely normal. He doesn’t want to believe that this is something terrible, he came up with some kind of legend for himself.”
This happens a lot with young patients - it is very difficult for them to accept that something so bad could happen to their health. But the danger is that sarcoma is a malignant tumor that often occurs in young people. Half of bone sarcomas and 20% of soft tissue sarcomas occur before age 35.
And so we met at the clinic. In this CT image you can see that the bones of the hand are almost destroyed.
According to the testimony, Maxim needed to disarticulate the shoulder - that is, remove the entire arm along with the shoulder blade, leaving, in fact, only the chest wall. Maxim did not agree to such a radical operation - he believed that it was just inflammation.
Therefore, Pyotr Sergeevich carried out amputation of the forearm.
The amputation went quickly. Dr. Sergeev has a dissertation on working with bone sarcomas and 10 years of surgical practice. The tissues were again sent for histology, but this time to a good laboratory, where oncology is the main area of work. The result of the study was epithelioid sarcoma.
All this suggests an important conclusion: in unclear cases, overexamination is better than underexamination. If you are young and your limb hurts, but the extent of the injury does not correspond to the pain syndrome, be wary. Simply put, a bruise should hurt for 2-3 days, but you’ve been hurting for three months now? Get an MRI immediately. It's not even an x-ray - consider it harmless.
Traumatologists very often miss sarcomas at an early stage, mistaking them for bruises. And 90% of sarcomas grow to impressive sizes using ointments, heating and electrophoresis - it would help with a bruise, but it stimulates the growth of sarcoma. But patients do not want to believe the worst, and reach critical conditions.
In addition, it may be beneficial for the traumatologist not to notice the sarcoma - one dressing costs 500–1,000 rubles. Maxim, for example, after “treatment of phlegmon” went to dressings 2 times a week.
Some of the doctors are greedy, some had excellent grades, many don’t care what happens to the patient, as long as the endless queue at the emergency room ends. Their work is really hard, it’s hard to blame them, but the result is a five-year survival rate for patients with sarcomas from 60 to 80%. In 5 years, we will no longer see a third of them. And the maximum 10-year survival rate is up to 40%. More than half of people with sarcomas die within 10 years.
Therefore, remember: your health is your responsibility, and it’s better to be on the safe side!
Resources
The Kristen Ann Carr Fund www.sarcoma.com The Kristen Ann Carr Fund is a resource for people diagnosed with soft tissue sarcoma. Every year the foundation prepares a newsletter “Up-to-date information on the problem of sarcoma.” You can find more information about soft tissue sarcoma on the foundation's website.
Cancer.Net www.cancer.net/cancer-types/sarcoma-soft-tissue/introduction Cancer.Net provides information about soft tissue sarcoma, including videos and blog posts.
This website also has a Spanish version. Visit the website for more information. to come back to the beginning
