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Juvenile Fibrosarcoma Treatment Pune
Sancheti Hospital’s Ortho Onco Department provides specialized juvenile fibrosarcoma treatment in Pune, ensuring precise care and long-term recovery.
Overview
Symptoms
Recognizing the signs of childhood fibrosarcoma early is crucial for prompt diagnosis and treatment. The symptoms can vary based on the tumor’s size, location, and whether it has spread to other areas.
Parents should watch for these common warning signs:
- Painless or tender mass: The most frequent symptom is a lump that appears on an arm, leg, or trunk. This mass may or may not cause pain
- Persistent pain or soreness: Discomfort can occur when the tumor presses against nearby nerves and muscles
- Movement difficulties: Children may experience limping or trouble using their arms, hands, legs, or feet depending on where the tumor is located
- Rapidly growing lump: Particularly in infants, parents may notice a mass that grows quickly over time
Causes
The exact cause of childhood fibrosarcoma remains unclear to medical researchers. However, scientific studies have identified several factors that may contribute to its development.
Genetic alterations appear to play a significant role in tumor formation. Research has shown that some families have multiple members across different generations who develop soft tissue sarcomas, suggesting a possible hereditary component.
A major breakthrough in understanding infantile fibrosarcoma came with the discovery of a specific genetic change called the ETV6-NTRK3 fusion. This chromosomal rearrangement occurs in approximately 70% of congenital-infantile fibrosarcoma cases. This genetic marker has become an important diagnostic tool, helping doctors distinguish this type of tumor from other similar conditions in children.
Scientists continue to study the connection between fibrosarcoma and other cancer types, as some research suggests links between soft tissue sarcomas and the development of additional cancers.
Risk Factors
While specific risk factors for childhood fibrosarcoma are not extensively documented, age plays a crucial role in determining both the type of tumor and its likely outcome.
Age-related risk factors include:
- Infants under one year: Most likely to develop infantile fibrosarcoma, which has a better prognosis
- Children aged 10-15 years: More likely to develop the adult form, which is more aggressive
- Tumor location: For infantile fibrosarcoma, children with tumors in central body areas (chest, abdomen) tend to have higher mortality rates
- Age at diagnosis: Children diagnosed after age one with infantile fibrosarcoma may face increased risks
Research shows that children under five years old have only a 7.3% chance of the cancer spreading, while children 10 years and older face a 50% risk of metastasis at five-year follow-up.
Diagnosis
Diagnosing childhood fibrosarcoma involves a comprehensive evaluation to determine the tumor type and whether it has spread. The process typically begins with a detailed medical history and thorough physical examination.
Diagnostic procedures may include:
- Imaging studies: Multiple scans help visualize the tumor and check for spread
- X-rays to see bone involvement
- MRI scans for detailed soft tissue images
- CT scans to assess tumor extent
- Bone scans to check for bone metastasis
- Blood tests: Complete blood count and chemistry panels provide important health information
- Biopsy: This critical procedure involves taking a tissue sample from the tumor for microscopic examination. The biopsy confirms the diagnosis and helps determine the specific type of fibrosarcoma
Once fibrosarcoma is confirmed, doctors perform staging to determine how far the tumor has spread. This information is essential for creating the most effective treatment plan and predicting outcomes.
Types
Childhood fibrosarcoma primarily occurs in two distinct forms, each with different characteristics and treatment approaches.
Infantile or Congenital Fibrosarcoma: This type affects approximately 80% of children diagnosed in their first year of life. It typically appears as a rapidly growing mass at birth or shortly after. Despite its fast growth, this form is generally less aggressive than the adult type. The tumor has a low tendency to spread to other body parts, with metastasis occurring in less than 10% of cases. The ETV6-NTRK3 gene fusion is commonly found in this type, making it an important diagnostic marker.
Adult Form Fibrosarcoma: This more aggressive variant affects older children and adolescents, typically between ages 10 and 15. It requires more intensive treatment approaches due to its aggressive nature. Children with this form face a higher risk of metastasis, with studies showing a 50% spread rate at five-year follow-up, similar to rates seen in adults.
Stages
Childhood fibrosarcoma staging helps doctors understand the extent of disease spread and plan appropriate treatment strategies.
Localized Fibrosarcoma: The tumor remains confined to its original location without spreading to distant body parts. Most infantile fibrosarcomas are localized at diagnosis and typically don’t spread. Treatment for localized disease often combines local control measures (surgery, radiation) with systemic therapy (chemotherapy) to achieve the best outcomes.
Metastatic Fibrosarcoma: This stage indicates the tumor has spread from its primary site to other body areas. The lungs are the most common site for metastasis in infantile fibrosarcoma. While distant spread is less common in the infantile form (less than 10%), it can occur, particularly in older children. Survival rates are generally better when only local recurrence occurs compared to distant or combined local and distant recurrence.
Treatment
Modern treatment for childhood fibrosarcoma has significantly improved outcomes, especially for localized disease. The approach typically involves multiple treatment methods tailored to each child’s specific situation.
Surgery : Surgery remains the primary treatment for fibrosarcoma, with the goal of completely removing the tumor while preserving as much normal function as possible. Modern surgical techniques emphasize limb-saving procedures over amputation whenever feasible.
Surgical options include:
- Wide local excision: Removing the tumor with surrounding healthy tissue margins
- Limb-salvage surgery: Preserving limb function while removing the tumor
- Rotationplasty: A specialized procedure where the cancer-free lower leg is reattached to the thigh
- Amputation: Reserved for cases where the tumor extensively involves major nerves and blood vessels
Chemotherapy: Chemotherapy plays a crucial role in treating fibrosarcoma by targeting cancer cells throughout the body. Treatment typically begins with chemotherapy to shrink the tumor before surgery, followed by additional chemotherapy to eliminate remaining cancer cells.
Common chemotherapy regimens include the VAC protocol (vincristine, actinomycin-D, and cyclophosphamide), sometimes combined with other medications. Treatment cycles are often given every two weeks in a schedule called “interval compression,” which studies suggest improves outcomes.
Radiation Therapy: Radiation therapy uses high-energy rays to destroy cancer cells and may be used alongside surgery. It’s particularly helpful for treating residual disease after surgery or for tumors in central body locations where complete surgical removal is challenging. However, radiation use in infants requires careful consideration due to potential growth impairment.
Rehabilitation
Recovery from childhood fibrosarcoma often requires comprehensive rehabilitation and supportive care to help children regain their abilities and manage treatment side effects.
Rehabilitation services may include:
- Physical therapy: Helps restore strength, mobility, and function, especially after limb surgeries
- Occupational therapy: Assists with daily activities and adaptive techniques
- Patient and family education: Provides information about long-term care needs and expectations
- Psychosocial support: Addresses emotional and psychological well-being for both child and family
- Genetic counseling: Offers guidance about potential hereditary factors
Complications
While childhood fibrosarcoma treatment has improved dramatically, certain complications can arise from either the disease itself or its intensive treatments.
Disease-related complications:
- Local recurrence: Can occur in up to 43% of cases, usually within the first year but sometimes much later
- Metastasis: More common in older children, with lungs being the most frequent site
Treatment-related complications:
- Surgical complications: May include loss of nerves, blood vessels, or muscles depending on tumor location
- Chemotherapy side effects: Can affect healthy cells and cause various adverse reactions
- Radiation effects: May cause growth impairment, bone deformities, or other late effects, particularly concerning in very young children
Prevention
Currently, there are no known prevention strategies for childhood fibrosarcoma. Since the exact causes remain unclear and genetic factors appear to play a role, medical science has not yet identified specific measures to prevent this type of cancer from developing.
Living With Childhood Fibrosarcoma
The outlook for children with childhood fibrosarcoma varies significantly based on several important factors. Understanding these factors helps families prepare for the journey ahead and maintain realistic expectations.
Factors affecting prognosis include:
- Disease extent: Whether the tumor is localized or has spread
- Tumor size and location: Affects treatment complexity and functional outcomes
- Age at diagnosis: Younger children, especially infants, generally have better outcomes
- Treatment response: How well the tumor responds to chemotherapy and other treatments
- Overall health: The child’s ability to tolerate treatment protocols
For infantile fibrosarcoma, the outlook is generally excellent, with most children achieving long-term survival. Studies show that 80% of patients with this form are alive at long-term follow-up, with many requiring only surgery for treatment.
Children who survive fibrosarcoma may experience some long-term effects from treatment, such as limb length differences or bone changes. However, comprehensive survivorship programs help families navigate these challenges and maintain the best possible quality of life.
Key Takeaways
- Childhood fibrosarcoma is a rare cancer affecting connective tissue, primarily in arms and legs
- Two main types exist: infantile form (better prognosis) and adult form (more aggressive)
- Early symptoms include painless lumps, pain, or movement difficulties
- Genetic factors, particularly the ETV6-NTRK3 fusion, play important roles in development
- Diagnosis involves imaging, blood tests, and biopsy, followed by staging
- Treatment combines surgery, chemotherapy, and sometimes radiation therapy
- Infantile fibrosarcoma has excellent survival rates, especially when diagnosed early
- Rehabilitation and long-term follow-up care are essential for optimal outcomes
At Sancheti Hospital, we understand the challenges families face when dealing with childhood fibrosarcoma. Our multidisciplinary team of pediatric oncologists, orthopedic surgeons, and supportive care specialists works together to provide comprehensive, compassionate care for children with this rare condition.
We utilize the latest diagnostic technologies and treatment approaches, including advanced surgical techniques and modern chemotherapy protocols, to achieve the best possible outcomes while preserving function and quality of life.
Our rehabilitation services and long-term survivorship programs ensure that children and families receive ongoing support throughout their journey, helping them navigate both the immediate challenges of treatment and the long-term aspects of recovery and wellness.
Meet Our Ortho Onco Specialist
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
What is the difference between infantile fibrosarcoma and adult form fibrosarcoma in children?
Infantile fibrosarcoma affects babies under one year old, grows slowly, and is less aggressive with excellent survival rates. The adult form occurs in older children (10-15 years), is more aggressive, and requires more intensive treatment approaches.
How is childhood fibrosarcoma diagnosed?
Diagnosis involves physical examination, medical history, and various tests including imaging studies (X-rays, MRI, CT scans), blood tests, and most importantly, a biopsy to confirm the diagnosis through tissue examination.
What are the main treatments for childhood fibrosarcoma?
Primary treatment is surgery to remove the tumor, often using limb-saving techniques. Chemotherapy is almost always used either before surgery to shrink the tumor or after surgery to eliminate remaining cancer cells. Radiation therapy may be used in specific situations.
Is childhood fibrosarcoma curable?
For localized fibrosarcoma, cure is often achieved through combined treatments. Infantile fibrosarcoma has particularly excellent outcomes with high long-term survival rates, especially when diagnosed and treated early.
What is the significance of the ETV6-NTRK3 gene fusion in infantile fibrosarcoma?
The ETV6-NTRK3 gene fusion is found in about 70% of infantile fibrosarcoma cases and serves as an important diagnostic tool, helping doctors confirm the diagnosis and distinguish this tumor from other similar conditions in children.
