Kamil Dolecki was 17 years old when he heard the diagnosis: bone sarcoma. He was then a high school student, a young athletic boy. One day he felt pain under the knee in his right lower leg. He didn’t particularly care about it, nor did the doctor he turned to. After all, it could usually be soreness after exercise or the effects of being hit by a car while riding a bicycle. However, when the night pains got stronger and the results in sports got worse, Kamil asked to be referred for an X-ray of his leg. He went to the sports doctor with the photo. Soon all the doctors from the clinic were looking over the photo. It was a rare occasion for them to see a rare tumor, bone sarcoma.
– Sarcomas, i.e. primary malignant neoplasms of soft tissues and bones, are very rare neoplasms – explains Prof. dr hab. n. med. Piotr Rutkowski, head of the Department of Tumors of Soft Tissues, Bones and Melanomas at the Oncology Center in Warsaw. – They constitute about 1 percent. all malignant neoplasms in adults and about 15 percent. in children. They mainly affect the elderly, therefore, in terms of numbers, the number of children is much smaller. Annually, we have approx. 1 thousand morbidity (including 80 in children). It is a very democratic cancer, because you can get sick at any age, regardless of gender. My oldest patient fell ill at the age of 91, but it could also be a one-year-old child. In the European Union countries, all rare cancers affect 6 people per 100 thousand. residents. There are 190 types, of which about 100 are sarcomas. About 3 million people suffer from it in the EU countries. Untreated sarcoma leads to death because it metastasizes mainly to the lungs. We live longer and longer and therefore will have more and more sarcomas.
What should arouse vigilance?
Sarcomas are a very diverse group of cancers. More than half of soft tissue sarcomas (STS) are located on the extremities, and about 15 percent each. in the retroperitoneal and intraperitoneal space (GIST). In general, the only manifestation of STS is a painless tumor, and sometimes also painless distortion of the limb outlines near the joints, lasting more than 6 weeks. A lump of several millimeters on the arm or leg, especially if it is located just under the skin, will usually attract attention. It is worse with tumors that grow deep in the muscle tissue or in the retroperitoneal space. In this case, it may take many months for the patient to see the doctor. Particular attention should be paid to a lesion that quickly (several months) grows larger than 10 cm or begins to grow rapidly. Patients often associate STS with recent trauma, but trauma is not a causal factor of this neoplasm. Occasionally, congestive swelling of the distal parts of the legs is observed. Sarcomas located on the extremities or on the integuments of the body rarely cause pain. If they do occur, they are usually in the late stage of the locally advanced disease. This is sometimes accompanied by muscle atrophy. On the other hand, sarcomas located inside the abdominal cavity can give pain symptoms related to pressure on the organs located there or lead to acute complications in the form of: obstruction, bleeding, perforation of the gastrointestinal tract.
– If the tumor located under the skin begins to enlarge, if it exceeds 5 cm, it is already an alarm signal – points out Prof. Piotr Rutkowski. – As a rule, the patient notices that the circumference of the leg or arm muscles is enlarging, or that something strange is happening in his body. A serious signal should be to locate the tumor not just under the skin, but deeper in the muscles. Such a change should first be diagnosed, not removed immediately and then examined. So you need to do an ultrasound, computed tomography or magnetic resonance imaging. If there is a suspicion that it is a tumor, a biopsy should be performed. For 100 mild lesions, this one will be a sarcoma. And if it is located in the muscles, one in 10 mild lesions could be sarcoma. A patient with suspected sarcoma should immediately be referred by a family doctor to an appropriate oncology facility.
Diagnostic difficulties
Patients with STS report late for oncological treatment because the observed tumor does not cause pain and therefore seems to be a trivial disease, and its presence is not burdensome in professional work and personal life. In addition, the doctors who are first contacted by patients make an incorrect diagnosis. The most common errors in the diagnosis of STS are the diagnosis of non-existent disease entities: stretching or rupture of the muscle belly, chronic hematoma or post-traumatic soft tissue edema, and spontaneous muscle hernia. Patients with such diagnoses are treated in many different ways for many months. Unfortunately, this is a priceless time wasted on the wrong therapy.
– There are very few people who are able to diagnose sarcomas not only in Poland, but all over the world – informs prof. Piotr Rutkowski. – 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 the Mother of Child, the Children’s Health Center in Warsaw and the Medical University in Wrocław. When it comes to adults, the situation is worse. Sarcomas are treated in several oncology centers, but the only one focused on this type of cancer is the clinic I am honored to run. Unfortunately, about 50-60 percent of patients come to us. Others are treated haphazardly, which means that they are not always treated properly. As I mentioned, most soft tissue tumors are benign tumors with a very high recovery rate after surgery; sarcomas as malignant tumors account for a very small percentage. In recent years, there has been a significant development in the knowledge of soft tissue sarcomas, both in terms of histopathology and genetics. The close cooperation of pathologists, surgeons and oncologists led to an increase in the survival time of patients who previously had a very poor prognosis.
Bone sarcomas
The situation is different for bone sarcomas. Annually, about 300 of them are detected in Poland, equally often in adults and children. This tumor relatively often develops in the still growing bones, and the bone tumor hurts. So if a young person or child complains of pain in an arm or a leg, this should not be taken lightly. At least an ordinary x-ray must be done – it could save your life.
The story of Kamil Dolecki is the best example of this. When he showed the photo to the sports doctor, he was immediately referred to the Oncology Center in Warsaw, to a clinic treating sarcomas. After an accurate diagnosis, he underwent difficult but effective treatment. It consisted of surgery, several cycles of chemotherapy and radiotherapy. There were hard moments, moments of breakdown, but he persevered. He managed to keep his leg, and this is perhaps the greatest success of this therapy. Fifteen years have passed since those difficult months, but he still reports for checkups and is always accompanied by enormous stress. Kamil’s recovery was due to the fact that he was quickly in the right place under the care of the right specialists. The stay at the clinic was happy for him in every way. Here he met his future wife. Here he met, he claims, excellent doctors who dealt with him in a professional manner. He also expresses his superlatives about the qualifications of the nurses from this ward. They are graduated from higher education and psychological courses, so they can provide the patient with comprehensive help. They know what to ask the patient, what to advise him, and how to mentally support him and his family. Patients can also count on the care of a psycho-oncologist.
Prof. Piotr Rutkowski, speaking of the treatment of sarcomas, emphasizes that surgery remains the most important method, but in most cases it is combined with radiotherapy or chemotherapy based on the so-called cytostatics or molecularly targeted drugs. These drugs have caused a breakthrough in oncology in recent years. They work for a specific purpose, i.e. a disorder in the tumor cell. Unfortunately, there are no drugs that are molecularly adapted to all types of sarcomas. – The progress in the treatment of sarcomas is incredible – admits prof. Piotr Rutkowski. – we can heal 70-80 percent patients with these tumors. In this respect, in specialized centers, we achieve results similar to those of the EU and the USA. We have great doctors in Poland – the problem, however, is the organization of access to them. Not every patient enters a specialist center immediately. Meanwhile, the therapy should be selected individually for the patient and the type of tumor, which can only be provided by reference centers.
Patients for patients
In 1996, on the initiative of the then head of the Department of Tumors of Soft Tissues, Bones and Melanomas, prof. Włodzimierz Ruka, the Sarcoma Sarcoma Help Association was founded. Kamil Dolecki is the president of this organization and one of its founders. Other people who live many years after recovery are also active in it. The organization provides comprehensive assistance to the sick and their families. It is psychological, informative, legal and financial help. Together with doctors from the Oncology Center in Warsaw, it also conducts an educational campaign aimed at general practitioners. On September 2, the fifth on-run around the buildings of the Oncology Center will take place. Every year there are more competitors at the starting line. They are cancer patients, their doctors and a group of sympathizers. Last year, the run lasted 60 minutes. 834 runners registered, who ran 5850 km in total. The income from the event was allocated to the various needs of the charges, including the costs of travel to the Oncology Center, medical visits and orthopedic supplies.
– It is not a poorly prognostic cancer if it is treated correctly – says prof. Piotr Rutkowski. – Further progress in the treatment of sarcomas will take place when patients are referred to reference centers in the early stages of the disease, if the diagnostic process is shortened, and therefore there will be pathologists who can diagnose sarcomas and the financing of reference centers will improve.
For those interested in the problem of sarcomas, we recommend the website of the patient organization: www.sarcoma.pl
Text: Barbara Skrzypińska