Journal of Orthopedic Oncology
Open Access

Pediatric patients; Pathologists; Radiologists; Tumor characteristics

Introduction

Clinical trials play a crucial role in expanding treatment options and improving outcomes for children with STS. Participation in clinical trials provides access to novel therapies and contributes to the advancement of medical knowledge in this challenging field. A multidisciplinary approach is essential for the accurate diagnosis and effective treatment of soft tissue sarcoma in children. Early detection, precise characterization of tumor subtypes, and the use of innovative therapies are crucial in providing improved outcomes and a brighter future for young patients battling this rare and complex group of malignancies.

Soft tissue sarcoma (STS) is a rare type of cancer that can affect people of all ages, including children. While STS accounts for a small proportion of childhood cancers, its diagnosis and treatment present unique challenges due to the rarity and diverse nature of the disease. In this article, we will explore the diagnosis and treatment approaches for soft tissue sarcoma in children, shedding light on the importance of early detection and multidisciplinary care. Rhabdomyosarcoma is the most common childhood soft tissue sarcoma in children 14 years and younger. See the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information [1-4].

Diagnosis of soft tissue sarcoma in children

Diagnosing soft tissue sarcoma in children requires a thorough evaluation by a team of specialists, including pediatric oncologists, pathologists, and radiologists. The process typically begins with a physical examination and medical history review, followed by imaging studies such as X-rays, ultrasound, CT scans, and MRI to assess the tumor’s size, location, and extent of invasion. To confirm the diagnosis and identify the specific subtype of soft tissue sarcoma, a biopsy is performed. This involves removing a small sample of the tumor tissue for examination under a microscope by a pathologist. Accurate diagnosis is critical as the treatment approach can vary significantly depending on the tumor type.

Treatment approaches for soft tissue sarcoma in children

Surgery to remove the tumor, such as Mohs microsurgery for small sarcomas of the skin, wide local excision, or limb-sparing surgery and Radiation therapy before and/or after surgery. High-dose radiation therapy, for tumors that cannot be removed by surgery.

Surgery

Surgery is the primary treatment for localized soft tissue sarcoma in children. The goal of surgery is to remove the tumor entirely while preserving nearby healthy tissues and organs. In some cases, surgical resection may be followed by reconstructive procedures to restore function and appearance. The surgical approach and extent of resection depend on the tumor’s size, location, and aggressiveness. Surgery is the standard treatment for all patients with adult-type, localized soft tissue sarcomas, and it should be performed by an appropriately trained surgeon [5-8].

Chemotherapy

Chemotherapy plays a significant role in treating certain types of soft tissue sarcoma in children, especially those with high-risk disease or tumors that have spread to other parts of the body. Chemotherapy involves the use of powerful drugs to kill cancer cells or shrink tumors before surgery or radiation. In some cases, chemotherapy may be used after surgery to eliminate any remaining cancer cells.

Radiation therapy

Radiation therapy uses high-energy X-rays or other types of radiation to target and destroy cancer cells. It is often used in combination with surgery to improve local tumor control and reduce the risk of recurrence. Radiation therapy is carefully planned to minimize exposure to healthy tissues and organs, ensuring the best possible outcomes with minimal long-term side effects.

Targeted Therapies

Recent advancements in targeted therapies have shown promising results in certain types of soft tissue sarcoma. Targeted therapies specifically aim at molecular alterations within cancer cells, providing a more focused and personalized treatment approach. While targeted therapies are still under investigation for pediatric soft tissue sarcoma, ongoing research holds the potential to expand treatment options for children with specific genetic mutations or biomarkers [9,10].

Clinical trials

Clinical trials play a crucial role in advancing the understanding and treatment of soft tissue sarcoma in children. These trials evaluate new treatment approaches, combination therapies, and novel drugs that may offer improved outcomes for young patients. Participation in clinical trials provides access to cutting-edge treatments and contributes to the progress of medical science. Treatment for pediatric STS consists of a multimodal approach, including surgery, chemotherapy, radiation therapy, and targeted therapies. Surgery remains the primary treatment option for localized tumors, aiming to achieve complete resection while preserving healthy tissues. Chemotherapy is employed for high-risk cases or metastatic disease, and radiation therapy complements surgery to enhance local tumor control. Recent advancements in targeted therapies offer a more personalized treatment approach by focusing on specific molecular alterations within cancer cells.

Conclusion

Soft tissue sarcoma in children represents a complex and challenging group of cancers, requiring specialized expertise and a multidisciplinary approach for accurate diagnosis and effective treatment. Early detection, precise diagnosis, and tailored treatment strategies are vital to improving outcomes and quality of life for young patients. Advancements in surgery, chemotherapy, radiation therapy, and targeted therapies offer new hope for children with soft tissue sarcoma. As research continues to expand our understanding of the disease and its underlying mechanisms, we move closer to providing more effective and personalized treatments for these young warriors, striving to ensure brighter futures for every child affected by this rare form of cancer. Soft tissue sarcoma in children represents a complex and challenging group of cancers, requiring specialized expertise and a multidisciplinary approach for accurate diagnosis and effective treatment. Early detection, precise diagnosis, and tailored treatment strategies are vital to improving outcomes and quality of life for young patients.

1. Imagawa K, de Andrés MC, Hashimoto K, Goldring MB, Roach HI, et al. (2011) The epigenetic effect of glucosamine and a nuclear factor-kappa B (NF-kB) inhibitor on primary human chondrocytes-implications for osteoarthritis. Biochem Biophys Res Commun 405: 362-7.

Indexed at, Google Scholar, Crossref

2. Eneroth M, Apelqvist J, Stenström A (1997) Clinical characteristics and outcome in 223 diabetic patients with deep foot infections. Foot Ankle Int 18: 716-722. 

Indexed at, Google Scholar, Crossref

3. Lipsky BA, Pecoraro RE, Larson SA, Hanley ME, Ahroni JH (1990) Outpatient management of uncomplicated lower-extremity infections in diabetic patients. Arch Intern Med 150: 790-797. 

Indexed at, Google Scholar       

4. Breen JD, Karchmer AW (1995) Staphylococcus aureus infections in diabetic patients. Infect Dis Clin North Am 9: 11-24. 

Indexed at, Google Scholar       

5. Lipsky BA, Berendt AR, Cornia PB, Pile JC, Peters EJ, et al. (2012) 2012 Infectious Diseases Society of America clinical practice guideline for the diagnosis and treatment of diabetic foot infections. Clin Infect Dis 54: 132-173. 

Indexed at, Google Scholar, Crossref

6. Jeffcoate WJ, Harding KG (2003) Diabetic foot ulcers. Lancet 361(9368): 1545-1551. 

Indexed at, Google Scholar, Crossref

7. Flint A, Weiss SW (1995) CD-34 and keratin expression distinguishes solitary fibrous tumor (fibrous mesothelioma) of the pleura from desmoplastic mesothelioma. Hum Pathol 26: 428-431.

Indexed at, Google Scholar, Crossref

8. Witkin GB, Rosai J (1989) Solitary fibrous tumor of the mediastinum: a report of 14 cases. Am J Surg Pathol 13: 547-557?

Indexed at, Google Scholar, Crossref

9. Babar Kayani, Aadhar Sharma, Mathew D Sewell, Johnson Platinum, Andre Olivier, et al. (2018) A Review of the Surgical Management of Extrathoracic Solitary Fibrous Tumors. Am J Clin Oncol (7): 687-694?

Indexed at, Google Scholar, Crossref

10. Little PJ, Drennon KD, Tannock LR (2008) Glucosamine inhibits the synthesis of glycosaminoglycan chains on vascular smooth muscle cell proteoglycans by depletion of ATP. Arch Physiol Biochem 114: 120-6.

Indexed at, Google Scholar, Crossref