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obsolete adult synovial sarcoma
Description
Obsolete Adult Synovial Sarcoma
Synovial sarcoma, also known as malignant synovioma, is a rare and aggressive type of cancer that affects the soft tissues around joints. In adults, it was previously referred to as an "obsolete" term, but it still represents a serious health concern.
- Definition: Synovial sarcoma is a type of soft tissue tumor that can occur anywhere in the body, but it typically affects the areas around large joints such as the knee, ankle, and elbow.
- Age and Sex: While synovial sarcoma can affect people of all ages, it is more common in young adults and children. In adults, it tends to occur in those between 20-40 years old, with a slight male predominance (M:F = 1.2:1).
- Incidence: Synovial sarcoma accounts for approximately 5-9% of all soft tissue sarcomas (STS), making it a relatively rare malignancy.
Key Facts
- Synovial sarcoma is a high-grade malignant tumor that can be aggressive and invasive.
- It can occur anywhere in the body, but it typically affects areas around large joints.
- The peak incidence is observed in young adults and children, with a slight male predominance.
- Synovial sarcoma accounts for approximately 5-9% of all soft tissue sarcomas (STS).
References
[3], [4]
Signs and Symptoms
Common Signs and Symptoms of Synovial Sarcoma in Adults
Synovial sarcoma, a rare type of soft tissue cancer, can exhibit various signs and symptoms in adults. While it's essential to note that not all cases present with noticeable symptoms, being aware of these indicators can aid in early detection and treatment.
- Swelling or Lump: The first symptom is often the appearance of a swelling or lump under the skin, usually near large joints such as the knees, elbows, or shoulders. This lump may be painless or painful, depending on its size and location [10].
- Limited Mobility: As the tumor grows, it can cause limited mobility in the affected area, making it difficult to move the joint or surrounding muscles [4].
- Pain or Numbness: In some cases, synovial sarcoma can press on nerves as it grows, leading to pain or numbness in the affected area [11].
- Weight Loss: Some patients may experience unexplained weight loss due to the tumor's impact on their overall health [2].
Important Considerations
It's crucial to remember that these symptoms can be similar to those of other conditions. If you're experiencing any unusual lumps, swelling, or pain, it's essential to consult a medical professional for proper evaluation and diagnosis.
References:
[1] - Not applicable (no relevant information in the context)
[2] - Context #2
[4] - Context #4
[10] - Context #10
[11] - Context #11
Additional Symptoms
- Limited Mobility
- Swelling or Lump
- weight loss
- pain
Diagnostic Tests
Diagnostic Tests for Obsolete Adult Synovial Sarcoma
Synovial sarcoma, a rare type of cancer, can be challenging to diagnose. The following diagnostic tests are used to confirm the presence of this disease in adults:
- Imaging tests: These include MRI scans, X-rays, and CT scans, which can show the size and location of the tumor, as well as any potential spread to other areas of the body [1].
- Biopsy: A biopsy is a procedure where a sample of tissue is removed from the affected area for further examination under a microscope. This is often performed using an image-guided needle biopsy [8].
- Molecular diagnostic tests: These tests can detect specific genetic mutations associated with synovial sarcoma, such as the SS18-SSX fusion oncogenes [10][12].
It's worth noting that aside from molecular diagnostic tests, no laboratory studies are specific for this diagnosis [4]. Early diagnosis and treatment are crucial in improving outcomes for patients with synovial sarcoma.
References:
[1] Tests and procedures used to diagnose synovial sarcoma include: Imaging tests. Imaging tests take pictures of the body. They can show where a synovial sarcoma is, how large it is and if it has spread to other areas of the body. [4] Aside from molecular diagnostic tests, no laboratory studies are specific for this diagnosis. [8] To confirm a diagnosis, we will perform an image-guided needle biopsy. A pathologist with expertise diagnosing sarcomas looks at the sample under a microscope ... [10] Synovial sarcoma (SS) is a mesenchymal tumor with partial epithelial differentiation. It is commonly seen in older children and younger adults. The presence of t(X;18)(p11.2;q11.2) is a pathognomonic feature of synovial sarcoma. [12] Synovial sarcoma is a malignant soft tissue tumor of uncertain histogenesis with variable epithelial differentiation. ... Diagnosis confirmed by molecular or cytogenetic testing for SS18-SSX fusion; commonly used methods include FISH, RT-PCR and NGS (Cancer ...
Treatment
Treatment Options for Adult Synovial Sarcoma
Synovial sarcoma, a rare and aggressive form of soft tissue cancer, can be challenging to treat. While there are no specific treatment options that have improved significantly in recent years, various drug therapies have been explored to manage this condition.
- Chemotherapy: Chemotherapy uses strong medicines to kill cancer cells. For synovial sarcoma, chemotherapy might be used before or after surgery (see 9 for more information). Common chemotherapeutic agents include doxorubicin and ifosfamide, which are often the first choice (see 8).
- Targeted Therapy: Targeted therapy uses medications to target specific cancer cells. Pazopanib, a targeted therapy taken as a pill, is FDA-approved for use by itself in adults with soft tissue sarcoma who have received chemotherapy (see 7 for more information).
- Gene Therapy: Gene therapy involves modifying genes to prevent or treat disease. Tecelra (afamitresgene autoleucel), a gene therapy approved by the FDA, is indicated for the treatment of adults with unresectable or metastatic synovial sarcoma who have received prior chemotherapy and meet specific criteria (see 13 and 14).
Second-Line Treatment Options
For patients who have not responded to first-line treatments, several drugs are available for second-line and further treatment of advanced and recurrent synovial sarcoma. These include gemcitabine and docetaxel, which may be used in combination with other chemotherapeutic agents (see 8).
Comparing Children and Adults
Research has shown that children and adults with synovial sarcoma have different outcomes when treated with the same therapies. A study published in Cancer compared the survival rates of 1268 patients, including both children and adults, and found significant differences between the two groups (see 10).
Conclusion
While there are no specific treatment options that have improved significantly in recent years, various drug therapies have been explored to manage adult synovial sarcoma. Chemotherapy, targeted therapy, and gene therapy are among the treatment options available for this condition. Further research is needed to improve outcomes for patients with synovial sarcoma.
References:
Differential Diagnosis
Additional Information
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- https://w3id.org/def/predibionto#has_symptom_11596
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- t437121
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- disease_ontology
- oboInOwl#id
- DOID:5489
- core#notation
- DOID:5489
- IAO_0000115
- An adult sarcoma of soft tissue and synovial sarcoma that affects non-epithelial, extraskeletal tissue of the body, including the muscle, fat, fibrous tissue, vessels and peripheral nervous system.
- rdf-schema#label
- obsolete adult synovial sarcoma
- owl#deprecated
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- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
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