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Sarcomas are a relatively rare malignant tumor that is not widely known. Its treatment is a challenge for the medical community not only because of its diversity and specificity but also sometimes because of its late and inaccurate diagnosis.

Today, medical science provides significant opportunities for early diagnosis and effective treatment of sarcomas, especially within the framework of an organized oncology center, specialized in the treatment of such tumors.

Prof. Dr. Nikolaos Vassos, Director of the Department of Surgical Oncology and the Center for Sarcoma, Melanoma & Rare Tumors at the Athens Medical Center explains everything we need to know about soft tissue sarcomas and when we should consult a specialist:

• Sarcoma, as a term, is relatively unknown. What exactly are sarcomas and what should we know about them?

A sarcoma is practically what the word itself implies, a tumor of the flesh. It is a malignant tumor that occurs in the so-called soft tissues of the body, that is, in anatomical structures that support other organs, and specifically in structures such as fat, muscles, tendons, vessels, nerves, etc. These tumors can develop anywhere in the body, which differentiates them from cancers that develop in specific organs, while they most often appear in the legs and arms, as well as in the trunk of the body (thorax, abdomen, retroperitoneum). Depending on the tissue of origin, sarcomas are divided into several subtypes (over 100 have been identified), with the most common being liposarcoma, leiomyosarcoma, pleomorphic sarcoma, rhabdomyosarcoma, angiosarcoma and gastrointestinal stromal tumors.

• How often do sarcomas occur and at what ages?

Sarcomas are a relatively rare type of cancer and can occur at any age. Overall, they account for 2% of all adult cancers and approximately 20% of cancers that develop in children and adolescents. However, soft tissue sarcomas occur more frequently in adults than in children, and the most common age of onset is between 40 and 60 years. The incidence in Europe is 8 cases per 100,000 people, with an increasing trend in recent years. In Greece, the actual incidence of sarcomas in the general population is not easy to determine, due to frequent misdiagnosis, lack of experience and specialization, and failure to record cases.

• What are the most suspicious symptoms?

Soft tissue sarcomas are often asymptomatic in the early stages and for a long time, while symptoms appear when the tumor increases in size and their nature depends on the part of the body where they develop. Many times, the appearance of a mass in the leg, arm or trunk raises suspicion, which is why any mass or formation in our body should be investigated immediately, if it: has a size of more than 4cm and a progressive tendency to increase in size, is located under the subcutaneous tissue and muscle fascia, and causes pain. In the abdomen, sarcomas cause atypical symptoms (abdominal pain, weakness, weight loss, gastrointestinal disorders) due to the tumor pushing neighboring organs.

• What tests are included in the diagnosis and staging of sarcoma?

The diagnosis is usually suspected clinically with the discovery of a painless mass, usually large, in the extremities or trunk, unless it is located within the abdomen, where the symptoms are atypical or repulsive. CT and especially MRI are essential for effective tumor imaging and disease staging, however, the most decisive role is played by fine-needle biopsy, i.e., the collection of a sample from the tumor for histopathological examination. The biopsy must be performed by an experienced and specialized surgeon or interventional radiologist guided by CT or ultrasound, so that the sample is representative of the tumor, without the risk of spread.

It is extremely important to say that biopsy is necessary before any therapeutic intervention, and in every oncological case. Only after a valid diagnosis and completion of the staging of the disease, can a proper treatment plan be formulated, and indeed within the framework of an interdisciplinary oncology council in the presence of all relevant specialties (specialized surgeon, pathologist – oncologist, radiation therapist, pathologist and radiologist).

• How are sarcomas treated? Is a cure possible?

The treatment of sarcoma is basically surgical and consists of total removal of the tumor within healthy margins, thus reducing the risk of local recurrence. Surgical treatment is sometimes accompanied by other forms of treatment, such as radiotherapy and systemic therapy (chemotherapy, targeted therapy, immunotherapy).

The development of science and the progress of technology now enable the experienced Sarcoma Surgeon to perform surgical procedures safely, achieving an excellent oncological result and ensuring the patient’s quality of life. This is because in the past it was not uncommon for patients with sarcoma in the hand or foot to be amputated. Today, this can clearly be avoided, with the holistic and individualized treatment of each case as well as the application of modern therapeutic approaches, such as isolated hyperthermic limb diffusion, intraoperative radiotherapy, etc.

It is extremely important to turn to experienced doctors and centers that specialize in the treatment of sarcoma. That is why at the Athens Medical Center we created the first specialized Center for Sarcoma, Melanoma & Rare Tumors. Our goal is the personalized care and holistic treatment of patients with sarcoma by specialized surgeons and doctors of all relevant specialties, with the state-of-the-art infrastructure, innovative equipment and highly trained staff of the Athens Medical Group.