Sarcomas – types, symptoms, diagnosis, treatment, prognosis

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Bone and soft tissue sarcomas are not very common. Even less than forty years ago, the diagnosis of sarcoma was treated almost like a death sentence. It is now known that they can also be effectively treated and destroyed with remarkable precision.

Sarcomas – characteristics and types

Sarcomas, a group of malignant tumors that originate from connective tissue, are divided into two large subgroups: soft tissue sarcomas (formed in adipose, muscle, and fibrous tissue) and bone sarcomas. Although there are over 100 subtypes of soft tissue sarcomas, it can be concluded that most of them are very aggressive. Although they rarely metastasize to the lymph nodes, they often cause distant metastases (mainly to the lungs). The likelihood of metastasis increases in proportion to the size of the tumor.

Sarcomas are rare, as it is estimated that they constitute only 1% of all malignant neoplasms in adults, and they are more often diagnosed in children and adolescents (7-10% of malignant neoplasms). The percentage of new cases is estimated at two cases per 100, which means that in Poland about 000 people will get sick each year.

Find out more: Benign and malignant neoplasms – typology

Soft tissue sarcomas – symptoms and diagnosis

Half of the cases of soft tissue sarcomas are diagnosed in the limbs, slightly less in the trunk, and in every tenth patient, the lesions are located in the head, neck or retroperitoneal space. In children, striated sarcoma is most often detected.

Soft tissue sarcomas for a long time may not be accompanied by any disturbing symptoms, and patients usually do not realize that their body is developing cancer. A sarcoma may be the cause of alleged joint swelling or refractory anemia. Meanwhile, these symptoms are apparently treated as a result of an injury or inadequate diet.

The long-term lack of clear or disturbing symptoms from the organism causes the diagnosed neoplasm to become very large. Then it most often causes metastases to the lungs. Fortunately, sarcomas as malignant tumors account for a very small percentage of all soft tissue cancers.

GIST sarcomas – symptoms and diagnosis

GIST sarcomas are tumors of the gastrointestinal stromal tissue. Changes that were considered poor prognosis a few years ago are often curable today, and the earlier they are detected, the better the prognosis. It has been known for several years that the formation of GIST sarcomas is associated with the malfunction of one of the cell receptors.

These tumors usually develop in the stomach or intestines and usually do not cause any symptoms for a long time. This is bad, because before they are diagnosed, they grow to a considerable size and often metastasize to the liver. Treatment is then much more difficult and may not always be successful. But there is also good news. Patients with early diagnosis of GIST sarcoma have a good chance of recovery.

Bone sarcomas – symptoms and diagnosis

Pain is the main symptom of bone sarcomas early in the development of the cancer. It may be accompanied by a tumor or swelling of the joint or bone area, distorting the outline of the limb, which patients sometimes associate with accidental trauma. In the more locally advanced stage of small cell bone sarcoma, general symptoms may occur: weakness, anemia and fever.

Bone sarcomas are located in various locations of the bone tissue: at the epiphyses or in the shafts of long bones, or in short bones of different shapes. Tumors that affect long and flat bones most often appear in young people – in their twenties and thirties. On the other hand, changes in bones of various shapes are relatively often observed in people who are definitely older.

Initially, nothing disturbing happens in the sick organism, only bone pains appearing at night are troubling. You should see a doctor for pain lasting more than 1,5 months. It is too long to consider complaints merely related to the trauma. Often, these ailments are mistakenly associated with the growth process of a young organism or with degenerative conditions occurring in older people. A proper diagnosis can be made only when, during rehabilitation attempts, there is a fracture or damage to the pathologically changed bone and it is necessary to perform tests.

Also check: Bone sarcomas can be cured

Other types of sarcomas

There are over 100 types of sarcomas. They include the following types.

Types of soft tissue sarcomas

The types of soft tissue sarcomas depend on the specific tissue or location that is affected. There are, for example:

1. Angiosarcoma – this type attacks blood or lymph vessels.
2. Gastrointestinal stromal tumor – affects the specialized neuromuscular cells of the intestine.
3. Liposarcoma – This is a fatty tissue sarcoma. It often starts in the thigh, behind the knee, or behind the abdomen.
4. Leiomyosarcoma – affects the smooth muscles of the walls of organs, often in the abdominal cavity.
5. Synovial sarcoma – This could be a stem cell tumor. Cancerous tissue may develop around the joints.
6. Neurofibrosarcoma – This type affects the protective lining of the nerves.
7. Rhabdomyosarcoma – forms in skeletal muscles.
8. Fibrosarcomas – affect fibroblasts, which are cells of connective tissue.
9. Mesenchymal sarcoma – They are rare and combine elements of other sarcomas. They can occur in any part of the body.
10. Vascular sarcoma – this type occurs in blood vessels.
11. Schwannoma – affects the tissues that cover the nerves.
12. Kaposi’s sarcoma – This type mainly affects the skin but can occur in other tissues. It stems from the human herpesvirus 8.

Types of bone sarcomas

The types of bone sarcomas include:

1. Osteosarcoma – affects the bones.
2. Ewing’s sarcoma – can occur in bone or soft tissue.
3. Chondrosarcoma – starts in cartilage.

Find out more: Inconspicuous symptoms of a tumor

Sarcomas – symptoms

Symptoms depend on the type of sarcoma, although it is assumed that the first symptom is a tumor located under the skin or within the muscles (most often on the limbs, but also on the abdomen or around the head or neck). The tumor gradually grows and begins to infiltrate, damaging the surrounding healthy tissue and compressing the nearby nerves, causing pain. In addition to pain, there can also be swelling and problems with moving the limb. When we are dealing with sarcoma located in deep tissues, its diagnosis is much more difficult.

It is assumed that the symptoms that are the reason for the diagnosis of sarcoma (regardless of the type of sarcoma) are: tumors enlarging, painful tumors, tumors over 5 cm in size, tumors located in deep tissue (usually found on imaging tests).

In the case of tumors smaller than 5 cm and located superficially, it is possible to excise them completely and submit them for histopathological examination. In the case of tumors larger than 5 cm and those located in deep tissues, the material to be examined is collected by biopsy or a tumor sample is taken during surgery.

It should be mentioned here that it is very important to carry out histopathological examinations, because only thanks to them it is possible to precisely determine what type of cancer we are dealing with, what stage it is, what the prognosis is and what treatment to choose.

Also read: Alarming symptoms of cancer in children

Sarcomas – causes

It is not known what causes sarcomas. In the past, it was thought that soft tissue injuries could increase the likelihood of sarcomas. However, ongoing research has shown that injuries – along with lifestyle factors such as diet and exercise – are not correlated with an increased risk of sarcoma.

Specialists suspect that it is determined by genetic factors, HIV infection (they cause Kaposi’s sarcoma). To this day, a large group of patients associate the sarcoma with trauma that occurred many years ago. It has been suggested that radiological irradiation or contact with carcinogenic chemicals may influence the development of these malignant lesions.

Regarding radiographs, patients may develop sarcomas after radiation used to treat other cancers, such as breast cancer or lymphoma. Sarcoma often starts in the part of the body that has been treated with radiation. The average time between radiotherapy and sarcoma diagnosis is around 10 years.

In the case of chemicals, experts say exposure to vinyl chloride (a chemical used in the manufacture of plastics) is a risk factor for the development of liver sarcomas, but it has not been proven that it causes soft tissue sarcomas. Arsenic is also associated with a type of liver sarcoma, but not with soft tissue sarcoma. Exposure to dioxins and herbicides that contain phenoxyacetic acid in high doses (such as in farm workers) may also be risk factors, but this is not certain.

However, there is no evidence that herbicides, (herbicides) or insecticides in the amounts encountered by the general public cause sarcomas.

The final causes of sarcomas, however, remain an unsolved mystery. The genetic examination of tumor cells can provide some explanation – in some cases chromosomal mutations are found, and on the basis of these, the type of tumor can be determined.

It has been noticed that sarcomas tend to appear in the course of hereditary diseases, e.g.

1. Neurofibromatosis Syndrome – also known as von Recklinghausen disease, causes many benign (non-cancerous) tumors that form in the nerves under the skin and in other parts of the body (neurofibromas). It is caused by a defect (mutation) of the NF1 and NF2 genes. About 5% of people with neurofibromatosis will develop the sarcoma into neurofibromatosis.
2. Werner syndrome – is caused by defects in the RECQL2 gene. Children with this syndrome have problems similar to those seen in the elderly. These include cataracts, skin changes, and clogged arteries in the heart (atherosclerosis) that can lead to a heart attack. They also have an increased risk of cancer, including soft tissue sarcomas.
3. Gardner’s syndrome – is a disease caused by defects in the APC gene. This syndrome is a type of familial adenomatous polyposis (FAP) and people with it have multiple polyps in the colon (and gut) and have a high risk of developing colon cancer. The syndrome also causes problems outside of the colon, including desmoid tumors.
4. Li Fraumeni syndrome – is caused by inherited defects of the TP53 gene. People affected by this syndrome have a high risk of developing cancer such as breast cancer, brain tumors, leukemia, and sarcomas. However, only 10 to 20 out of 100 people with Li-Fraumeni syndrome will develop soft tissue sarcoma. People with this syndrome are sensitive to the carcinogenic effects of radiation. So if they have cancer that is treated with radiation therapy, they have a very good chance of developing new cancer in the part of the body that has been treated.
5. Retinoblastoma – is an eye cancer in children that can be caused by defects in the RB1 gene. Children with this gene defect are also at greater risk of developing bone sarcomas or soft tissue sarcoma, especially if retinoblastoma has been treated with radiation therapy.
6. Gorlin Syndrome – This is also known as the nevus epithelioma syndrome. It is caused by defects in the PTCH1 gene. People with this syndrome have a high risk of developing many basal cell skin cancers. They also have an increased risk of developing fibrosarcoma and rhabdomyosarcoma.
7. Tuberous sclerosis – may be caused by a defect in the TSC1 and / or TSC2 gene. People with this syndrome often have seizures and learning difficulties. Benign (non-cancerous) tumors appear in many different organs. They also have kidney problems, often along with a kidney tumor called an angiomyolipoma. 

Read also: Atypical neoplasms

Sarcomas – diagnosis

In the diagnosis of sarcomas, information collected during general examinations and data obtained using:

1. x-rays (the lungs are a common site of soft tissue sarcoma metastases, so a chest X-ray may be taken to see if the cancer has spread to the lungs)
2. computed tomography (useful for the initial diagnosis of soft tissue sarcoma and to see if the cancer has spread to other areas of the body; can also be used to guide a biopsy needle)
3. magnetic resonance imaging (can help outline the location of the tumor in the soft tissues, as well as help determine if cancer cells have spread)
4. endoscopic examination along with taking a sample.

PET is also used to diagnose sarcomas (it is useful when the doctor thinks the cancer has spread but does not know where; it can be used instead of many different X-rays as it scans the entire body; PET-CT is often used. It helps is to decide if the lesions seen with CT scan, i.e. CT, are cancer or something else; PET is not often used in sarcomas but can be helpful in some cases) and ultrasound (can be used to obtain information about the tumor or surrounding tissues and organs, as well as to precisely locate the tumor for needle biopsy).

Performing a biopsy (necessary to assess the degree of tumor malignancy) requires special care to prevent the spread of neoplastic cells throughout the body. The two most common biopsy techniques used to perform a soft tissue sarcoma biopsy are needle biopsy and open surgical biopsy.

Needle biopsy – With this procedure, your doctor will numb the area of ​​the planned biopsy with a local anesthetic before inserting the needle into the tumor to collect a cell sample. Computed tomography or ultrasound may be used to facilitate guidance of the biopsy needle, especially if the nodule is not close to the body surface. Needle biopsy may be particularly useful for tumors that are difficult to access, such as a retroperitoneal sarcoma (a sarcoma deep in the abdominal or pelvic cavity) or a sarcoma deep in the thigh.

Surgical biopsy – Due to the nature of the treatment of sarcomas, an open surgical biopsy is only performed by an experienced oncologist surgeon who has previously worked with sarcomas. This surgery is usually performed under general anesthesia as the surgeon will remove a relatively large sample of the tumor. In the case of soft tissue sarcomas, the surgeon must be careful not to destroy the tumor as this can spread the cancer cells.

Sarcomas – methods of treatment

In recent years, significant progress has been made in the treatment of soft tissue and bone sarcomas, not only of the primary tumor, but also of local recurrences and metastases. Surgery plays a key role in the treatment of these cancers, and radiotherapy and chemotherapy are supportive treatments that increase the chance of a full recovery or long-term survival, as well as limiting the scope of surgery (limb-sparing surgery instead of amputation). Thanks to the close cooperation of pathologists, surgeons and oncologists, an increase in the length of survival of patients who previously had a very poor prognosis has now been achieved.

The treatment of sarcomas, due to their malignant nature, follows certain rules. The therapy must be radical, at the same time as economical for the body as possible, and at the same time effective and well-aimed.

When performing surgical procedures, the most important thing is radical excision of the lesion. Sometimes, after opening the lesion, the surgeon deems the limb to be amputated. However, a life-saving mutilation can be an effective weapon against the sarcoma reoccurring. Not so long ago, amputation of a limb affected by sarcoma was a standard, now it is possible to save as much as 80% of limbs. It must be remembered that sarcomas are also often responsible for recurrences of the disease in a place that has already been affected and cleared of cancerous tissue.

The use of surgery, chemotherapy and radiotherapy does not always bring the expected effect in terms of stopping the tumor growth, reducing its size or eliminating the lesion. High hopes are raised by the therapy with the use of molecular factors used in systemic treatment, i.e. systemic treatment – they hit the tumor like arrows at the target.

Read also: Teratomas – the strangest of cancers

Prognosis for sarcoma

The prognosis depends on factors such as: the type and size of the sarcoma, the response to treatment, and whether the tumor was radically resected (whether it was completely removed with an adequate margin of healthy tissue). It should be noted, however, that the results in treating sarcomas are improving and complete recovery is relatively common.

This is due to the fact that sarcomas are often found on the surface of the body, subcutaneously, where they can be easily seen, so you can quickly consult a doctor and start effective treatment.

See also: Hereditary neoplasms

Life after sarcoma is cured

It is very important to attend all check-ups. During these visits, doctors will ask questions about any problems the treated person has, and may perform tests and lab tests to look for signs of cancer or side effects from treatment.

Almost any cancer treatment can have side effects. Some may last from weeks to months, but others may last for life. Check-ups may be very frequent at first, and will decrease over time with good results. Some of these visits may include chest x-rays and other scans of the tumor site. This helps the doctor watch for any signs of a recurrent sarcoma.

During this time, it is very important that you report any new symptoms to your doctor immediately so that any problems can be detected early when they are easier to treat.

Depending on the type of treatment, physical therapy and rehabilitation can be a very important part of recovery.

It is also worth remembering to adopt healthy behaviors in our lives, such as not smoking, eating well, exercising regularly and maintaining a healthy weight, as this can help (although this has not been confirmed by researchers). However, we know that such changes can have positive health effects beyond the risk of soft tissue sarcoma or other cancers.

If the cancer recurs at some point, your treatment options will depend on the location of the cancer, previous treatments, and your health.

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