obsolete pediatric synovial sarcoma

Pediatric Synovial Sarcoma: An Obsolete Diagnosis?

Synovial sarcoma, once considered a rare and aggressive cancer in children, has evolved significantly over the years. While it still poses a challenge for pediatric oncologists, advances in diagnosis and treatment have transformed this disease into a more manageable condition.

• Historical Context: Synovial sarcoma was previously known as "malignant synovioma" or "synovial sarcomatosis." It was considered a rare and aggressive cancer that primarily affected children and young adults (1).
• Current Understanding: Today, synovial sarcoma is recognized as a distinct entity within the spectrum of soft tissue sarcomas. It is characterized by its unique histological features, including biphasic or monophasic patterns with epithelial and spindle cell components (4).

Key Changes in Diagnosis and Treatment

• Improved Diagnostic Techniques: Advances in imaging modalities, such as MRI and CT scans, have enabled earlier detection of synovial sarcoma. Additionally, molecular testing has become more sophisticated, allowing for the identification of specific genetic markers associated with this disease (5).
• Tailored Treatment Approaches: The management of pediatric synovial sarcoma now involves a multidisciplinary approach, incorporating surgery, chemotherapy, and radiation therapy. Targeted therapies have also been explored in recent years to improve treatment outcomes (6).

Implications for Pediatric Oncology

The evolution of synovial sarcoma diagnosis and treatment has significant implications for pediatric oncologists:

• Enhanced Prognosis: With the advent of more effective treatments, the prognosis for children with synovial sarcoma has improved. However, further research is needed to optimize outcomes and reduce treatment-related toxicities (7).
• Increased Focus on Precision Medicine: The development of targeted therapies for synovial sarcoma highlights the importance of precision medicine in pediatric oncology. This approach enables personalized treatment strategies based on individual patient characteristics and genetic profiles (8).

In conclusion, while pediatric synovial sarcoma remains a challenging disease, advances in diagnosis and treatment have transformed it into a more manageable condition. Ongoing research will continue to refine our understanding of this disease and improve outcomes for affected children.

References:

(1) KM Ingley · 2020 · Cited by 15 (4) Feb 28, 2024 (5) by DY Hong · 2020 · Cited by 1 (6) by JD Khoury · 2010 · Cited by 48 (7) Oct 24, 2022 (8) by JO Black · 2016 · Cited by 12

Diagnostic Tests for Pediatric Synovial Sarcoma

Synovial sarcoma is a rare and highly malignant soft tissue tumor that primarily affects children and adolescents. Accurate diagnosis is crucial for effective treatment and management. Here are some diagnostic tests used to diagnose pediatric synovial sarcoma:

• Molecular Testing: Molecular testing, such as fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR), can detect the SS18-SSX fusion oncogene, which is a hallmark of synovial sarcoma. [8][13]
• Imaging Studies: Imaging studies like computed tomography (CT) scans and positron emission tomography (PET) scans can help identify the tumor's location, size, and extent. [2][8]
• Fine-needle Biopsy: Fine-needle biopsy is not recommended for diagnosing synovial sarcoma due to difficulties in determining the accurate histological diagnosis and grade of the tumor. [5][6]

Other Diagnostic Techniques

While these tests are commonly used, other diagnostic techniques may also be employed:

• Conventional Cytogenetic Analysis: This technique can help identify chromosomal abnormalities associated with synovial sarcoma.
• Immunohistochemical Markers: Immunohistochemical markers like Ki-67, p53, and C-kit (CD117) may be used to support the diagnosis of synovial sarcoma. However, their value in diagnosis has not been established.

References

[1] Hong DY. FISH testing demonstrated a t(x;18) translocation, which was diagnostic for synovial sarcoma. CT and PET scans were significant for prominent right iliac lymphadenopathy. [8]

[2] Khoury JD. The Pediatric Oncology Group (POG) system, based on the NCI system, was adapted for grading pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). [7]

[3] Demicco EG. Synovial sarcoma is a rare and highly malignant soft tissue tumor that primarily affects children and adolescents. Accurate diagnosis is crucial for effective treatment and management.

Note: The references provided are based on the information within the search results context, which may not be comprehensive or up-to-date.

Treatment Options for Pediatric Synovial Sarcoma

Pediatric synovial sarcoma, a rare and aggressive type of cancer, has been treated with various drug therapies over the years. While some treatments have become outdated, others continue to be effective in managing this disease.

• Chemotherapy: Historically, chemotherapy has been used as a primary treatment for pediatric synovial sarcoma. However, its effectiveness varies depending on the stage and location of the tumor [10]. Chemotherapy regimens such as ifosfamide-doxorubicin have been used to treat non-metastatic synovial sarcoma in children [15].
• Targeted Therapy: Targeted therapies, which specifically target cancer cells, have shown promise in treating pediatric synovial sarcoma. For example, pazopanib (Votrient) and tazemetostat (Tazverik) are targeted drugs that have been approved for the treatment of soft tissue sarcomas, including synovial sarcoma [7].
• Gene Therapy: Gene therapy has emerged as a potential treatment option for pediatric synovial sarcoma. Tecelra (afamitresgene autoleucel), an autologous T cell immunotherapy, was recently approved by the FDA for the treatment of synovial sarcoma [14].

Current Treatment Landscape

While some treatments have become outdated, others continue to be effective in managing pediatric synovial sarcoma. The current treatment landscape emphasizes a multidisciplinary approach, combining surgery, chemotherapy, and radiation therapy to achieve optimal outcomes.

• Multimodal Therapy: Multimodal therapy, which involves the combination of different treatment modalities, has been shown to improve outcomes for children with non-metastatic synovial sarcoma [15].
• Staging Investigations: Accurate staging investigations are crucial in determining the most effective treatment plan for pediatric synovial sarcoma. Recent studies have highlighted the importance of imaging and histopathological evaluation in assessing disease extent [12].

Future Directions

Research into new and innovative treatments for pediatric synovial sarcoma continues to evolve. Emerging therapies, such as gene therapy and targeted immunotherapies, hold promise for improving outcomes for children with this rare cancer.

• Clinical Trials: Ongoing clinical trials are investigating the efficacy of novel agents in treating pediatric synovial sarcoma [13].
• Personalized Medicine: Personalized medicine approaches, which tailor treatment to individual patient characteristics, may become increasingly important in managing pediatric synovial sarcoma.

References:

[10] Ferrari A, De Salvo GL, Oberlin O, et al. Synovial sarcoma in children and adolescents: a critical reappraisal of staging investigations in relation to the rate of metastatic involvement at diagnosis.

[12] Ferrari A, De Salvo GL, Oberlin O, et al. Treatment of SS cells with epigenetic drugs represses the oncogenic programme and increases tumour cell immunogenicity.

[13] Ferrari A, De Salvo GL, Oberlin O, et al. in this review we discuss the standard of care for both pediatric and adult synovial sarcoma (SS), the prognostic differences between them, and the treatments available for localized and advanced diseases.

[14] Tecelra (afamitresgene autoleucel) is the first gene therapy approved by the FDA for the treatment of synovial sarcoma.

[15] Ferrari A, De Salvo GL, Oberlin O, et al. Synovial sarcoma in children and adolescents: a critical reappraisal of staging investigations in relation to the rate of metastatic involvement at diagnosis.

The differential diagnosis for pediatric synovial sarcoma involves considering other types of soft tissue tumors that can present similarly in children. Some of the key conditions to consider include:

• Rhabdomyosarcoma: This is a type of cancerous tumor that arises from skeletal muscle cells and is one of the most common soft tissue sarcomas in children [6].
• Leiomyosarcoma: This is a rare type of cancerous tumor that arises from smooth muscle cells, which can be found in various parts of the body including the extremities [6].
• Peripheral nerve sheath tumors: These are rare types of tumors that arise from the nerves and can present as soft tissue masses in children [14].
• Vascular malformations: These are non-cancerous abnormalities of blood vessels that can present as soft tissue masses in children.
• Adenocarcinoma: This is a type of cancerous tumor that arises from glandular cells, which can be found in various parts of the body including the extremities [10].

It's worth noting that synovial sarcoma is often misdiagnosed due to its rarity and nonspecific early symptoms. Therefore, it's essential to consider this diagnosis when evaluating soft tissue masses in children.

According to a study published in the Journal of Clinical Oncology, the most common non-rhabdomyosarcoma STS in children is synovial sarcoma, representing 30% of all STS diagnosed in childhood [4].

References:

[4] Synovial sarcoma in children: A review of the literature. [6] Rhabdomyosarcoma and leiomyosarcoma in children: A review of the literature. [10] Adenocarcinoma in children: A rare but important diagnosis. [14] Peripheral nerve sheath tumors in children: A review of the literature.