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Paper Abstract

Current treatment option for chest wall Ewing Sarcoma

What is chest wall Ewing sarcoma?

Chest wall Ewing sarcomas, historically known as skin tumors, are tumors formed by a particular type of bone or soft tissue cell. It is often found in children, adolescents, and young adults with a female preponderance. The cause of Ewing sarcoma is not known. However, it is believed to be associated with a change in a specific gene. This gene change can be inherited or acquired during an individual’s lifetime. Most commonly, they develop on the ribs, but they can also occur on the paravertebral, sternal, and scapular areas.

A chest wall mass, chest pain, or respiratory distress are common symptoms of chest wall Ewing sarcoma. Sometimes, patients do present with pathological fractures or metastases-related symptoms, as well as unspecific symptoms such as fever, enlarged superficial lymph nodes, decreased general condition, and nocturnal sweating.

Chest wall Ewing sarcoma is usually found during a routine X-ray or CT scan. A biopsy, which is a procedure to remove a small sample of tissue for examination under a microscope, is usually needed to confirm the diagnosis[1].

Chest wall Ewing Sarcoma.jpg

New development in the efforts to cure Chest wall Ewing sarcomas

The recent advances in the management of Ewing sarcoma have led to a significant improvement in the overall survival of patients with this disease. However, the prognosis for patients with chest wall involvement remains poor, with a 5-year survival rate of less than 50%.

The current treatment for chest wall Ewing sarcoma includes:

  • Surgery

  • Radiation therapy

  • Chemotherapy

 

The main treatment for chest wall Ewing sarcoma is surgery, followed by adjuvant chemotherapy and radiation therapy. The type of surgery depends on the location and volume of the tumor as well as the possibility of infiltration into the lung, pericardium, diaphragm, esophagus, spine, or major vessels. However, it has a limited success rate because of the high rate of local recurrence, which often leads to poor outcomes.

A new surgical technique called En-bloc resection and reconstruction has been developed to improve the outcome of patients with chest wall Ewing sarcoma. It involves the complete removal of the tumor, along with a margin of healthy tissue. However, it can be a challenge due to the infiltration into other parts of the body.  Healthy tissue is then reconstructed to recreate a normal appearance. To prevent instability, flailing, and infection, large defects are often covered with viable tissue and prosthetic materials (muscle flaps)[2].

 

The goal of the reconstruction is to restore the function and appearance of the chest wall. There are a variety of different muscle flaps that can be used for reconstruction including the pectoralis flap, the latissimus dorsi flap, and the rectus abdominis flap. The choice of these muscle flaps depends on the size and location of the tumor, as well as the overall health of the patient. Radical En-bloc resection and reconstruction can be very effective in treating chest wall Ewing sarcoma, but it is also very complex and requires a highly skilled surgeon. The surgery is often performed at a specialized center[3].

Neoadjuvant therapy

Prior to the initiation of the main treatment (preferably surgery), neoadjuvant therapy is given to reduce the tumor size in the chest wall sarcoma. This is called neoadjuvant therapy.  Chemotherapy, radiotherapy, and hormone therapy are a few examples of neoadjuvant therapy. 

 

The standard chemotherapy treatment for Ewing sarcoma is a combination of two drugs, doxorubicin, and vincristine. This can be given as neoadjuvant therapy. Doxorubicin is an anthracycline drug. Vincristine is a vinca alkaloid drug. These drugs are given through an IV or intravenous line. They are usually given every three weeks.

Neoadjuvant therapy is performed before surgery (main treatment) due to the following reasons:

  • To facilitate tumor shrinkage

  • To aid in the complete removal of the tumor during surgery

  • To minimize the need for additional large-field radiotherapy, which has a high risk of secondary morbidities, such as growth impairment, cardiomyopathy, and secondary cancers

 

Preoperative radiotherapy can also be given as neoadjuvant therapy, in cases where the tumor grows rapidly or when the margins are expected to be tight. Neoadjuvant therapy(either chemotherapy or radiation or both) led to a statistically significant reduction in the tumor volume[4].

When a new approach promises to be useful it ought to be tried!

Chest wall Ewing sarcoma is a very rare and one of the most aggressive malignancies, with 5-year survival rates ranging between 40 and 60%. To ensure long-term survival, patients must undergo multiple treatments following specific protocols, including radical resections as well as chest wall repair.

References

  1. A. Akhtar et al., “Sarcoma Arising from the Chest Wall : A Case Report,” Cureus, vol. 9, no. 8, p. e1604, doi: 10.7759/cureus.1604.

  2. En-bloc resection of soft-tissue sarcoma of anterior chest wall and reconstruction with titanium bars and free anterolateral thigh flap - PubMed.” https://pubmed.ncbi.nlm.nih.gov/34266816/ (accessed Dec. 20, 2022).

  3. A. Burt et al., “Treatment of chest wall sarcomas: a single-institution experience over 20 years,” Am J Clin Oncol, vol. 38, no. 1, pp. 80–86, Feb. 2015, DOI: 10.1097/COC.0b013e31828aac22.

  4. S. Pasquali and A. Gronchi, “Neoadjuvant chemotherapy in soft tissue sarcomas: latest evidence and clinical implications,” Ther Adv Med Oncol, vol. 9, no. 6, pp. 415–429, Jun. 2017, DOI: 10.1177/1758834017705588.

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