Sarcomas is a diverse group of malignant neoplasms originating in the mesenchymal tissue. These tumors affect many different tissues, but they share common features - very aggressive growth, infiltrating and destroying adjacent tissues, frequent recurrence and a tendency to form distant metastases. Which sarcomas occur in children and which in adults? How to recognize a sarcoma? And can it be cured?
What are sarcomas?
Sarcomasare cancers that affect both children and adults.Sarcomas in adultsare rare neoplasms, accounting for 1% of all cancers in adults. According to research, there are about 2 cases / 100,000 people / year in Poland. The risk of developing sarcoma in adults is believed to increase with age.
The statistics concerning the youngest are completely different, wheresarcomas in childrenconstitute approx. 15% of all childhood cancers. Soft tissue neoplasms are much more common, about 10% of cases are bone tumors.
The most common sarcoma in adults is leiomyosarcoma, while in children it is rhabdomyosarcoma.
Types of sarcomas
The classification and nomenclature of sarcomas is based on the differentiation of the tissues from which they originate.
- liposarcoma (liposarcoma)- originating from adipose tissue
- leiomyosarcoma (leiomyosarcoma)- originating from the tissue of smooth muscles, i.e. those found in the wall of internal organs
- rhabdomyosarcoma- originating from the tissue of striated muscles, i.e. skeletal muscles
- fibrosarcoma- originating from fibrous tissue found in the tendons, pleura, peritoneum and nerve sheaths
- Osteosarcoma (osteosarcoma)- originating from bone tissue
- chondrosarcoma- originating from cartilage
- vascular sarcoma (haemangiosarcoma)- originating from the tissue that forms the blood vessels
Within each of the groups listed, there are several or even a dozen or so subtypes,Therefore, it is considered that over 60 different sarcomas occurring in humans can be identified.
The above-mentioned sarcomas differ from each other in their sensitivity to treatment as well as in clinical symptoms.
Causes of sarcomas
The causes of sarcomas are not fully understood.
It is believed that viruses (e.g. HIV), exposure to chemicals, ionizing radiation, mechanical injuries and thermal injuries (burns) may be the causative agents.
So far, the genetic determinant of sarcomas has not been proven, but they tend to occur in the course of hereditary diseases, such as:
- neurofibromatosis syndrome
- Li Fraumeni band
- Werner's team
- Gardner's syndrome
In addition, some sarcomas are characterized by the presence of specific genetic mutations on the basis of which the final diagnosis of sarcoma is made.
Sarcoma - clinical symptoms
The sarcomas are most often located within the limbs, the next place is the abdominal cavity, and the head and neck area.
The symptoms of sarcoma vary depending on its type, but in general it can be assumed that the first symptom is the appearance of a tumor most often located under the skin or within the muscles.
The tumor is painless at first, but it does not disappear either, and even grows with time.
Pain occurs due to infiltration and damage to the surrounding he althy tissue by the tumor, as well as compression of the nerves adjacent to the tumor. Pain may be accompanied by swelling and problems with moving the limb.
The diagnosis of deep tissue sarcoma is usually difficult, therefore such tumors are diagnosed late. The alarming symptoms that require the initiation of diagnostics for sarcoma, regardless of its type, include:
- tumors larger than 5 cm
- tumors growing
- painful tumors
- tumors located in deep tissues, usually found in imaging tests
Diagnosis of sarcoma
In order to start the diagnosis of sarcoma, it is necessary to find a suspicious change during a medical examination.
The next step is to perform imaging tests, which allows to determine the exact location of the tumor and its size. For this purpose, an ultrasound or X-ray examination is used.
If the tumor is located within the limbs or abdominal wall, an MRI is performed, and if the tumor is located near the pelvis or the abdominal cavity, computed tomography is useful.
If suspicion existsIf metastases occur, a CT scan of the chest is performed at the time of diagnosis, because the sarcoma most often forms metastases in the lungs.
The result of a histopathological examination is necessary to confirm the diagnosis. The material for such an examination can be obtained in two ways, depending on the location of the tumor and its size.
If the tumor size does not exceed 5 cm and it is located superficially, it can be completely excised and sent for histopathological examination. If, on the other hand, the tumor is large (>5 cm) or located in deep tissues, then the material for examination is taken by biopsy or a tumor sample is taken during surgery.
Only the histopathological examination allows for the precise determination of the type of neoplasm, stage and prognosis. It is also possible to select an appropriate treatment based on the histopathological examination.
Sarcoma treatment
When a sarcoma is diagnosed, treatment varies depending on the type and stage of sarcoma.
If no distant metastases are found, the primary method of treatment is surgical removal of the tumor along with a margin of he althy tissue. In some cases, postoperative radiotherapy is also necessary.
In the case of large tumors, preoperative radio- or chemotherapy is used to reduce the mass of the tumor, which helps to avoid large, mutilating surgeries and improves the prognosis.
In patients with metastases, chemotherapy is the first method of treatment.
Surgical removal of metastases is sometimes possible, as long as they are not numerous and are located in a place that allows surgery, e.g. in the lungs.
Sarcoma prognosis
The prognosis depends on the type of sarcoma, its size and response to treatment, as well as whether the tumor resection was performed in a radical manner, i.e. whether the tumor was completely removed with an appropriate margin of he althy tissue.
Currently, however, the results of treating sarcomas are very satisfactory and very often it is possible to get a complete recovery.
Sarcomas are a very diverse group of malignant neoplasms, but most of them are located on the surface of the body, subcutaneously, where they are easy to observe.
If you notice a disturbing, enlarging tumor, you should consult a doctor, because a quick diagnosis allows for the implementation of effective treatment.
According to an expertProf. dr hab. n. med. Piotr Rutkowski, head of the Department of Tumors of Soft Tissues, Bones and Melanomas
People who arethere is very little to diagnose sarcoma, not only in Poland, but also in the world. In order to recognize and treat this neoplasm, many specialists are needed, so the patient should be referred to a reference center, and a multidisciplinary one at that.
In Poland, we have a network of pediatric oncology centers that deal with sarcomas. The best are the Institute of Mother and Child and the Children's Memorial He alth Institute in Warsaw, as well as the Medical University in Wrocław.
When it comes to adults, the situation is worse, because sarcomas are treated in several oncology centers, but the only clinic targeted at this type of cancer is the clinic I am honored to run.
For adult patients to be properly treated, 3-4 such facilities are needed. On the other hand, Poland is lucky to be such a large country that there are specialists in treating sarcomas! There is no center in Lithuania, as only 70 people suffer from these cancers.
Early diagnosis and proper diagnosis are very important. Since we have 1 thousand sarcomas annually and over 20 thousand. family doctors, statistically speaking, one such doctor will encounter a sarcoma every twenty years. The point is not to make a mistake at this stage.
Statement by prof. Rutkowski comes from the press materials of the Sarcoma Aid Society
About the authorBow. Agnieszka MichalakA graduate of the First Faculty of Medicine at the Medical University of Lublin. Currently a doctor during postgraduate internship. In the future, she plans to start a specialization in pediatric hematooncology. She is particularly interested in paediatrics, hematology and oncology.