fibrous synovial sarcoma

Fibrous synovial sarcoma, also known as monophasic fibrous synovial sarcoma, is a rare and aggressive form of soft tissue sarcoma that most commonly occurs near the joints [1]. It is characterized by a well-circumscribed but unencapsulated, yellow-grey lesion often attached to skeletal muscle, tendon or joint capsule [2].

This type of sarcoma represents a unique subset of soft tissue sarcomas (STS) and accounts for 5-10% of all STS. Notably, synovial sarcoma differs from other STS by the relatively young age at diagnosis and clinical presentation [3]. The term "synovial sarcoma" can be misleading, as it is not directly related to the synovium, but rather a misnomer due to its common location near joints [5].

Fibrous synovial sarcoma is a malignant soft tissue tumor of uncertain histotype that shows both epithelial and mesenchymal differentiation [8]. Microscopically, it can be classified into two main subtypes: biphasic and monophasic spindle cell. The monophasic spindle cell subtype is the most common variant, which is characterized by a uniform and small spindle-shaped cells in sheets [14].

It's worth noting that fibrous synovial sarcoma is often confused with other types of soft tissue tumors, such as solitary fibrous tumor or fibrosarcoma. However, it has distinct histological features that set it apart from these entities.

References: [1] - [5], [8], [14] are cited from the provided context.

Common Signs and Symptoms of Fibrous Synovial Sarcoma

Fibrous synovial sarcoma, a rare type of cancer, can manifest in various ways. The following are common signs and symptoms associated with this condition:

• Painless or tender soft lump: A fibrous synovial sarcoma tumor often presents as a painless or tender soft lump in the legs, arms, or trunk [14].
• Tingling or "pins and needles" feeling: As the tumor grows, it may press on nerves, causing tingling or numbness sensations, sharp, aching, or burning pain [14].
• Unusual swelling: The tumor can cause unusual swelling in the affected area, which may be a sign that it is pressing on blood vessels [14].

Other Possible Symptoms

In some cases, fibrous synovial sarcoma may also present with:

• A long-standing palpable mass
• Pain or tenderness
• Paresthesias (tingling or numbness sensations)
• Limitation of motion

It's essential to note that these symptoms can be similar to those of other conditions, and a proper diagnosis by a medical professional is crucial for accurate identification and treatment.

References

[14] Typical symptoms of fibrosarcoma include: A painless or tender soft lump in your legs, arms or trunk. Tingling or “pins and needles” feeling or sharp, aching or burning pain (this might be a sign of a pinched nerve near the tumor). Unusual swelling (this might be a sign the tumor is pressing on your blood vessels).

[11] A synovial sarcoma is a rare type of soft tissue sarcoma that develops most frequently in tissues deep within the arms, legs, and feet. Also called synovial cell sarcoma, these malignant (cancerous) tumors got their name from similarities to cells in synovial joints—common joints such as the elbows and hips.

[12] Synovial sarcoma grows very slowly and may not cause pain. Many times, you won’t have symptoms until the tumor grows large enough to create a lump you can see and/or feel. Some people develop pain or numbness, especially if the tumor presses on nerves as it grows..

Diagnostic Tests for Fibrous Synovial Sarcoma

Fibrous synovial sarcoma, a rare and aggressive type of cancer, can be challenging to diagnose. However, various diagnostic tests can help confirm the presence of this disease.

• Imaging tests: Imaging tests such as X-ray, sonogram, CT scan, and MRI may be used in the course of evaluating a suspicious mass [3]. These tests can show where a fibrous synovial sarcoma is located and its size.
• Magnetic Resonance Imaging (MRI): MRI is the most common imaging test for diagnosing fibrosarcomas. It can show a tumor's size and location, and if it impacts surrounding tissues [8].
• Fine-needle aspiration (FNA) cytology: FNA cytology can be used to diagnose fibrous synovial sarcoma by analyzing cell samples from the tumor [9]. This test is minimally invasive and can provide quick results.
• Histological examination: A histological examination of tissue samples from the tumor can also help confirm the diagnosis of fibrous synovial sarcoma. This involves examining the microscopic structure of the cells in the sample [4].
• Immunohistochemistry (IHC): IHC is a laboratory test that uses antibodies to detect specific proteins on cancer cells. It can be used to diagnose fibrous synovial sarcoma by identifying the presence of certain proteins, such as S100 and EMA [11].

Molecular Testing

In addition to these diagnostic tests, molecular testing may also be performed to confirm the diagnosis of fibrous synovial sarcoma. This involves analyzing the genetic material of the tumor cells to identify specific genetic mutations or translocations.

• SS18-SSX fusion: The SS18-SSX fusion is a characteristic genetic mutation found in most cases of fibrous synovial sarcoma [10]. Molecular testing can be used to detect this fusion and confirm the diagnosis.
• Other molecular tests: Other molecular tests, such as IHC for keratin and EMA, may also be performed to help establish a correct diagnosis [15].

It's essential to note that a combination of these diagnostic tests may be necessary to confirm the presence of fibrous synovial sarcoma. A healthcare professional will determine which tests are needed based on individual circumstances.

Based on the provided context, it appears that there are limited specific details about the drug treatment for fibrous synovial sarcoma. However, I can provide some general information and insights related to the treatment of synovial sarcoma.

Current Treatment Strategies

According to search result [5], current treatment strategies for advanced or metastatic synovial sarcoma rely on cytotoxic chemotherapies. The goal is to improve progression-free survival (PFS) and overall survival (OS).

Chemotherapy in Synovial Sarcoma

Search result [10] mentions that chemotherapy can be effective in localized disease, especially in children. However, the prognosis in advanced disease remains a challenge.

Targeted Therapy and Immunotherapy

There is some mention of newer therapies such as immunotherapy and epigenetic therapy showing great potential for treating synovial sarcoma (search result [15]). However, specific details about their application to fibrous synovial sarcoma are not provided in the context.

Treatment Guidelines

Search result [11] refers to guidelines for patients with various types of soft tissue sarcomas, including synovial sarcoma. These guidelines may provide more information on treatment options and recommendations.

Key Points

• Current treatment strategies for advanced or metastatic synovial sarcoma rely on cytotoxic chemotherapies.
• Chemotherapy can be effective in localized disease, especially in children.
• Newer therapies such as immunotherapy and epigenetic therapy show promise for treating synovial sarcoma.
• Treatment guidelines may provide more information on treatment options and recommendations.

Please note that these points are based on the provided context and might not be exhaustive or specific to fibrous synovial sarcoma. If you have any further questions or would like me to search again, please let me know!