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Synovial Sarcoma Treatment Pune
We at Sancheti Hospital offer expert care for Synovial Sarcoma using advanced diagnostics, limb-sparing surgery, and personalized cancer management plans.
Overview
Synovial sarcoma is a rare and aggressive form of cancer that develops in the body’s soft tissues. Despite what its name suggests, this cancer doesn’t actually start in the synovium (the lining around joints). The name is misleading because doctors once thought the tumor cells looked like early joint-lining cells.
This soft tissue cancer typically grows near large joints like knees and ankles, but it can also appear in arms, feet, abdomen, chest, head, neck, and even internal organs like the lungs.
The disease most commonly affects teenagers and young adults, usually before age 30, with an average diagnosis age of around 30 years. While advanced or metastatic synovial sarcoma historically had a poor prognosis, new treatment approaches including targeted therapy and immunotherapy are offering hope for better outcomes.
Symptoms
Synovial sarcoma often grows slowly, which means symptoms may not appear for up to two years. When symptoms do develop, they can easily be mistaken for common conditions like arthritis or injuries from sports.
The most common signs include:
- A lump or bump you can feel: This is usually the first symptom. The lump sits deep under the skin and may or may not cause pain
- Pain: While initially painless, discomfort can develop as the tumor grows and presses on nearby nerves
- Swelling: The area around the tumor often becomes swollen
- Numbness: If the tumor affects nerves, you may feel numbness in that area
- Joint stiffness: When the tumor is near a joint, it can make movement difficult or cause stiffness
When synovial sarcoma develops in unusual locations, it can cause specific symptoms:
- Head and neck tumors: May cause hoarseness, trouble swallowing, or breathing problems
- Lung tumors: Can lead to shortness of breath or chest pain
Causes
The main cause of synovial sarcoma involves specific genetic changes within cells. In over 90% of cases, the cancer is caused by a unique chromosomal translocation – essentially, genetic material switches places between the X chromosome and chromosome 18.
This genetic switch creates what’s called the SS18::SSX gene fusion. There are three main types of this fusion: SS18::SSX1, SS18::SSX2, and rarely, SS18::SSX4. These abnormal gene fusion proteins interfere with normal cell function by disrupting important genetic controls that prevent cancer growth.
The gene fusion acts like a broken switch that turns on cancer-promoting genes while turning off protective ones. This disruption includes affecting the Sox2 gene, which is crucial for synovial sarcoma cell growth.
It’s important to understand that this gene fusion is not inherited from parents. It’s a somatic mutation, meaning it happens in the body’s cells after birth. Scientists don’t yet know exactly what triggers this specific genetic change.
Risk Factors
While researchers don’t fully understand what causes the chromosomal translocation that leads to synovial sarcoma, several risk factors have been identified:
Age is the most significant risk factor. Synovial sarcoma primarily affects adolescents and young adults, with most cases occurring before age 30. Younger patients, particularly those under 15-20 years old, tend to have better long-term outcomes than older individuals.
Gender may play a role, though studies show mixed results. Some research suggests a slight male predominance, while other studies indicate equal distribution between males and females. There appears to be some connection between gender and the specific type of SS18::SSX gene fusion.
Other rare risk factors mentioned in medical literature include:
- Previous radiation therapy: Though extremely uncommon, radiation-induced synovial sarcoma has been reported
- Certain genetic conditions: Inherited conditions like Li-Fraumeni syndrome or neurofibromatosis type 1 may increase risk for soft tissue sarcomas in general
Remember that having risk factors doesn’t mean you’ll develop synovial sarcoma. The underlying gene fusion is what defines this cancer, and currently, there’s no way to prevent it.
Diagnosis
Diagnosing synovial sarcoma can be challenging because it’s rare, grows slowly, and causes symptoms similar to common, non-cancerous conditions. This often leads to delayed diagnosis, making it crucial for healthcare providers to maintain a high level of suspicion, especially in young patients with deep masses.
The diagnostic process typically involves several steps:
Clinical Evaluation: A healthcare provider will examine you physically, review your symptoms, and take a detailed medical history.
Imaging Tests: These are essential for visualizing the tumor and determining its characteristics:
- X-rays: Often the first test performed, these can show soft tissue masses and detect calcifications within the tumor, which occur in up to 30% of synovial sarcomas
- CT scans: Provide detailed images showing the tumor’s complex nature and are excellent for detecting subtle calcifications. Chest CT scans routinely check for lung metastasis
- MRI: The preferred method for determining tumor extent and planning surgery. Advanced MRI can reveal distinctive features in larger tumors, including the “triple sign” (different signal intensities) and “bowl of grapes” appearance
- PET scans: Often used to evaluate for metastatic disease
Biopsy: This is the definitive diagnostic step. A tissue sample is obtained and examined by a pathologist. Core needle biopsy with imaging guidance is generally preferred as it provides adequate samples while minimizing tissue removal.
Pathological and Molecular Confirmation: Specialists identify the unique features of synovial sarcoma and use molecular techniques to detect the characteristic SS18::SSX fusion gene, which is critical for definitive diagnosis.
Given the complexity of synovial sarcoma, early referral to a specialist sarcoma center is highly recommended. This ensures diagnosis and management by a multidisciplinary team of experts, significantly improving outcomes.
Types
Synovial sarcoma is classified into three main types based on how the tumor cells look under a microscope:
Monophasic Synovial Sarcoma: The most common type, composed mainly of uniform, small, spindle-shaped cells that are tightly packed together. Despite being primarily spindle cells, these tumors still show epithelial characteristics when examined with special stains.
Biphasic Synovial Sarcoma: Contains two distinct components – spindle cells (like the monophasic type) and epithelial cells. The epithelial cells can form gland-like spaces or grow in groups, typically showing stronger staining for epithelial markers.
Poorly Differentiated Synovial Sarcoma: Considered a high-grade variant consisting of sheets of uniform, closely packed, small round cells. These tumors have many dividing cells and areas of dead tissue, making them more aggressive with a higher rate of metastasis.
While these subtypes look different under the microscope, all three are characterized by the SS18::SSX gene fusion. The type can correlate with the fusion variant, but the fusion type itself isn’t typically a significant factor in prognosis – tumor grade and disease stage are more important.
Stages
Staging helps determine how far the cancer has spread and guides treatment decisions. The TNM (Tumor, Node, Metastasis) system is most commonly used:
- T (Tumor): Describes the size and local extent of the primary tumor
- N (Nodes): Indicates whether cancer has spread to nearby lymph nodes. Synovial sarcoma spreads to lymph nodes more often than other soft tissue sarcomas (8-27% of patients)
- M (Metastasis): Shows whether cancer has spread to distant body parts, most commonly the lungs
Tumor Grading assigns scores based on how abnormal the cells look, how many are dividing, and how much dead tissue is present. Synovial sarcoma is typically classified as high-grade due to its aggressive nature.
For treatment purposes, synovial sarcoma is often grouped into:
- Localized Disease (Stages 1-3): Cancer is confined to its original site or nearby tissues/lymph nodes
- Metastatic Disease (Stage 4): Cancer has spread to distant organs, indicating poorer prognosis
Treatment
Managing synovial sarcoma requires a comprehensive approach involving a multidisciplinary team of specialists including medical oncologists, surgical oncologists, radiation oncologists, pathologists, and radiologists. Treatment strategies are tailored to each patient based on tumor characteristics and overall health.
Surgery is the cornerstone of treatment for localized synovial sarcoma. The goal is to achieve wide surgical margins – completely removing the tumor with surrounding healthy tissue to minimize recurrence risk. Limb-sparing surgery is preferred whenever possible, with amputation rarely necessary.
Radiation Therapy uses high-energy beams to destroy cancer cells. It’s frequently combined with surgery to improve local control, especially for larger tumors (>5 cm) or those with narrow surgical margins. This can be given before surgery (to shrink the tumor) or after surgery (to target remaining cancer cells).
Chemotherapy is particularly important because synovial sarcoma responds better to chemotherapy than many other soft tissue sarcomas. Anthracycline-based regimens, often combined with Ifosfamide, are standard first-line treatment for advanced or metastatic synovial sarcoma, achieving response rates around 60%.
Targeted Therapy attacks specific pathways within cancer cells. Pazopanib is the only FDA-approved targeted therapy for synovial sarcoma, demonstrating improved progression-free survival. Other agents like Regorafenib and Anlotinib are being studied in clinical trials.
Immunotherapy and Cell Therapy represent exciting new approaches:
- Adoptive Cell Transfer using genetically engineered T-cells shows promising results by targeting Cancer Testis Antigens highly expressed in synovial sarcoma
- Key targets include NY-ESO-1 (expressed in 80% of cases), MAGE-A4 (50-80% of cases), and PRAME
- These therapies have shown objective clinical responses in trials
Rehabilitation
Recovery from synovial sarcoma treatment often requires comprehensive rehabilitation to help patients regain function and quality of life. This typically includes:
Physical Therapy: Essential for regaining strength, mobility, and function after surgery, especially when tumors affect limbs or areas near joints.
Pain Management: Addresses chronic pain that may result from the tumor itself or treatment effects.
A holistic approach during and after treatment maximizes patient recovery and enhances overall well-being.
Complications
Synovial sarcoma is associated with several serious complications due to its aggressive nature:
Local Recurrence: Despite aggressive treatment, there’s a high risk of the cancer returning at the original site. Recurrences can happen years or even decades after initial treatment, highlighting the need for long-term follow-up.
Metastasis: The cancer spreads to distant sites in 40-68% of patients. Common metastasis sites include:
- Lungs (80% of distant spread)
- Lymph nodes (8-27% of cases)
- Bones (typically appearing as destructive lesions)
- Brain and other organs (rarely)
Compression Effects: Growing tumors can press on nearby nerves, blood vessels, or organs, causing pain, numbness, or organ dysfunction.
Treatment-Related Complications: Medical therapies can cause significant side effects:
- Chemotherapy: Blood cell reduction, nausea, hair loss, heart problems (anthracyclines), nerve and kidney damage (ifosfamide)
- Radiation therapy: Wound healing problems, lymphedema, tissue scarring, fractures
- Targeted therapy: Heart or liver toxicity
- Immunotherapy: Cytokine release syndrome, nervous system effects
Close monitoring is essential even after successful treatment due to the cancer’s aggressive nature.
Prevention
Unfortunately, synovial sarcoma cannot be prevented. The underlying cause is a specific chromosomal translocation that results in the SS18::SSX gene fusion, which occurs spontaneously during a person’s lifetime and cannot be inherited from parents.
Since the primary cause is genetic and beyond individual control, lifestyle changes or dietary modifications are not effective prevention strategies.
However, while primary prevention isn’t possible, outcomes can be improved through:
- Early detection: Being aware of body changes and promptly seeking medical attention for persistent or growing lumps
- Optimal treatment: Receiving care at specialized sarcoma centers with multidisciplinary teams ensures the most effective treatment strategies
Living With Synovial Sarcoma
A synovial sarcoma diagnosis can be overwhelming, but advances in treatment and supportive care allow many individuals to achieve long-term survival. Successfully living with this condition requires a proactive approach to health management and strong support systems.
Regular Follow-ups: Even after successful treatment, synovial sarcoma can recur or spread years later. Regular check-ups with your oncology team, including imaging scans like chest X-rays and CT scans, are crucial for early detection of any new or recurring disease.
Managing Treatment Side Effects: Your healthcare team can help you effectively manage side effects from chemotherapy, radiation therapy, and other treatments to maintain quality of life.
Physical and Emotional Support: Physical therapy helps regain strength and mobility, while occupational therapy assists with daily activities. Emotional support from family, friends, or cancer support groups provides crucial psychological benefits.
Nutrition and Rest: Maintaining good nutrition and adequate rest supports your immune system and overall health during and after treatment.
Open Communication: Don’t hesitate to ask questions about your condition, treatment options, and potential side effects. Report any new or worsening symptoms promptly to your healthcare provider.
While the journey can be challenging, a dedicated multidisciplinary team and strong support systems help patients navigate the complexities of synovial sarcoma and improve overall well-being.
Key Takeaways
- Synovial sarcoma is a rare, aggressive soft tissue cancer primarily affecting young adults, characterized by the SS18::SSX gene fusion
- Symptoms include slow-growing lumps, pain, swelling, and numbness, often mistaken for benign conditions
- Diagnosis requires imaging studies and biopsy with molecular confirmation of the gene fusion
- Treatment involves a multidisciplinary team approach with surgery, radiation therapy, and chemotherapy
- Advanced treatments include targeted therapy like Pazopanib and immunotherapy targeting NY-ESO-1 and MAGE-A4
- The cancer has high risks for recurrence and metastasis, requiring long-term monitoring
- Prevention isn’t possible due to genetic causes, but early detection and expert care improve prognosis
At Sancheti Hospital, we understand the complexities of managing rare cancers like synovial sarcoma. Our comprehensive cancer care program brings together a dedicated multidisciplinary team of oncologists, surgeons, radiologists, and pathologists who specialize in soft tissue sarcomas.
We provide state-of-the-art diagnostic facilities including advanced imaging technologies and molecular testing capabilities essential for accurate synovial sarcoma diagnosis. Our treatment approach encompasses all modern therapeutic options, from precision surgery and advanced radiation therapy to the latest chemotherapy protocols and targeted therapy options.
We also offer comprehensive rehabilitation services, pain management programs, and psychological support to help patients and families navigate their cancer journey. At Sancheti Hospital, we are committed to providing personalized, evidence-based care that gives our patients the best possible outcomes while maintaining their quality of life throughout treatment and recovery.
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Patient Stories & Experiences
The pain in my left knee left me feeling helpless for years. After my treatment here, I can finally say I'm pain-free.
Vinita Singh
The nerves were swollen, and the body went numb. But thanks to Sancheti Hospital, I got a second life!
Parvati
I finally could walk again, a relief I've only felt after the hip pain surgery. I thank the doctors at Sancheti Hospital for their help.
Balaji Kharat
I'm a police officer, and I'm extremely thankful to Sancheti Hospital for treating my fracture without surgery.
Shantilal
My life has completely changed after the knee replacement surgery at Sancheti Hospital. It's like I can finally live again!
Kalpana Lepcha
The knee pain I've carried for years finally went away with the help of Sancheti Hospital.
Karuna
I can't believe that I get to finally live a normal and happy life, all thanks to the knee surgery I had at Sancheti Hospital.
Kishore Bhosle
Frequently Asked Questions
How long does it take for synovial sarcoma to spread to other parts of the body?
Synovial sarcoma spreads at different rates in different people. Some patients may develop metastasis within months, while others may remain stable for years. The cancer’s tendency to spread depends on factors like tumor size, grade, and location. Regular monitoring is crucial since spread can occur even years after initial treatment.
Will I need to have my arm or leg amputated?
Amputation is rarely necessary today. Modern surgical techniques allow doctors to remove the tumor while preserving the limb in over 90% of cases. Limb-sparing surgery combined with radiation therapy is now the standard approach, maintaining function while effectively treating the cancer.
How often do I need scans after treatment, and for how long?
Most patients need chest X-rays or CT scans every 3-4 months for the first 2-3 years, then every 6 months for years 4-5, and annually thereafter. Since synovial sarcoma can recur decades later, lifelong monitoring is recommended, though scans become less frequent over time.
Can I still have children after chemotherapy treatment?
Chemotherapy can affect fertility in both men and women. Before starting treatment, discuss fertility preservation options with your doctor, such as sperm banking or egg freezing. Many patients do successfully have children after treatment, but it’s important to plan ahead and get specialist advice.
