What is a sarcoma?

sarcome

Sarcomas are malignant tumors that develop from the body’s supporting tissues or “soft tissues” such as muscles, adipose tissue, cartilage, blood vessels or lymphatics, nerves, or bones. The type of sarcoma depends on the type of cells from which it originated.

Sarcomas can be classified into three major categories:

  • Soft tissue sarcomas
  • Visceral sarcomas
  • Bone sarcomas or osteosarcomas

Ewing’s sarcoma is the second most common malignant bone tumor after osteosarcoma in adolescents and young adults. Ewing’s sarcoma affects about 80 to 100 new patients per year in France. The majority of cases (70%) occur in young people aged 5 to 25 years. However, these tumors can be diagnosed before 5 years and after 30 years.

In this pathology, the term “primitive tumor” refers to the location in the body where Ewing’s sarcoma originated. Ewing tumors are considered “localized” when the tumor is limited to this primitive tumor, localized in the bone in the majority of cases (in 15% of cases they can develop elsewhere).

The main symptoms of Ewing’s sarcoma

The most common symptom is pain. Other symptoms depend on the neighboring organs that may be affected by the presence of the sarcoma: motor, sphincter, or respiratory problems, for example. Most often, the tumor initially develops in a bone, in the pelvis (30%), thorax (20%), femur (16%), tibia (9%), vertebra (8%), and humerus (5%). Unfortunately, the disease has a strong potential to generate metastases that can be located in the lungs, bones, or bone marrow. At the slightest doubt, do not hesitate to consult your doctor, who will prescribe appropriate tests for the child and refer them, if necessary, to the relevant specialists.

Diagnosis

A biopsy and imaging tests are necessary to diagnose a sarcoma located in the bone:

  • A radiograph of the entire bone is used to look for “ignored” lesions, meaning that the osteosarcoma has spread from the primitive tumor to other parts of the bone;
  • A chest scan is used to see if the osteosarcoma has spread to the lungs;
  • An MRI is used to evaluate the tumor and facilitate surgical planning. For Ewing’s sarcoma, the diagnosis is confirmed after a genetic analysis of the tumor cells that were sampled. This will identify the genetic abnormalities frequently found in these tumors and confirm the diagnosis.

Treatments

The treatment of Ewing’s sarcoma includes three phases, all of which are important to control the disease, achieve remission, minimize the risk of recurrence, and maximize the chances of cure with the least possible sequelae. The choice of treatment should be discussed in a multidisciplinary consultation meeting (RCP) “sarcoma” for adult patients or “pediatric or AYA” for patients under 18 years old.

Initial “neoadjuvant” chemotherapy

This pre-operative chemotherapy has many advantages:

  • Stop the progression of the disease wherever it is located
  • Early attack on metastases
  • Eliminate pain and facilitate the surgical procedure The current treatment, called VDC-IE (combining vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide), includes 14 cycles of pre-operative chemotherapy over 9 months. Patients with bone or bone marrow metastases have more prolonged chemotherapy that may include a combination of Temodal/Irinotecan (TEMIRI cycles). The response to these treatments will be considered good if there are fewer than 10% of live tumor cells remaining.

Local treatment: surgery

Surgery aims to remove the entire portion of the bone affected by the sarcoma and then reconstruct it. This is known as “conservative” surgery with resection/reconstruction during the same operation. More rarely, an amputation may be necessary when local invasion is significant. Radiotherapy is often used in addition to surgery to reduce the risk of local recurrence. In cases considered inoperable, exclusive radiotherapy may be the only local treatment.

Post-operative “adjuvant” chemotherapy

This chemotherapy is similar to that used pre-operatively and is adapted in the case of risk factors for recurrence (presence of metastases at diagnosis, initial tumor volume, and poor response to initial chemotherapy). For metastatic forms or in case of poor histological response, other treatments are discussed:

  • Irradiation of isolated pulmonary metastases
  • High-dose chemotherapy (combination of busulfan and melphalan) with hematopoietic stem cell transplantation
  • Maintenance treatment with oral chemotherapy

Long-term complications of radiotherapy

Unfortunately, the irradiated child’s bone will develop less well than normal. Fractures and consolidation defects are generally found following radiotherapy, as is the occurrence of a secondary tumor, which remains rare. Knowledge of these complications has led to limiting the indications for radiotherapy in operated children.

RESEARCH AGAINST EWING’S SARCOMA WITH IMAGINE FOR MARGO

Since 2011, Imagine for Margo has made research against cancer in children, adolescents, and young adults a priority. Indeed, given the current situation, the association funds numerous research programs to cure more and better the young affected by cancer. Among these, there are programs concerning Ewing’s sarcoma:

Current research on childhood Ewing’s sarcoma should further improve the management of this disease by increasing treatment effectiveness and reducing side effects.

If you wish to help research and build a world with cancer-free children, please consider donating by clicking here. Every gesture is essential in our fight for life.

Together, we will defeat childhood and adolescent cancer.