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MFH Treatment Experts in Pune
We treat malignant fibrous histiocytoma at Sancheti Hospital’s Ortho Onco Department with specialized surgical care.
Overview
Malignant Fibrous Histiocytoma (MFH) has undergone significant changes in how doctors understand and classify it. What was once thought to be the most common soft tissue sarcoma in adults is now mainly called Undifferentiated Pleomorphic Sarcoma (UPS). This name change happened because newer diagnostic tools helped doctors realize that many tumors previously labeled as MFH were actually different types of sarcomas or other cancers.
UPS is a high-grade, aggressive sarcoma, meaning it’s a malignant tumor that grows rapidly and spreads easily. While doctors initially thought it came from immune cells called histiocytes, research now suggests it likely develops from primitive stem cells.
This rare cancer can affect different parts of the body, including:
- Soft tissues like muscles, tendons, and fat
- Bones
- Internal organs such as the heart, lungs, and liver
- The retroperitoneum (area behind the abdomen)
Because UPS is rare and challenging to diagnose, patients need specialized care from a team of different medical experts.
Symptoms
MFH or UPS can be tricky to spot early because tumors often grow for a long time without causing obvious symptoms. The first sign is usually a painless lump or swelling in the affected area.
As the tumor gets bigger, it may press on nearby structures and cause:
- Pain that can be constant or come and go
- Numbness or tingling if nerves are affected
- Swelling that feels firm or soft
- Limited movement when joints or muscles are involved
- Fractures if the sarcoma develops in bones
If UPS spreads to other parts of the body, it can cause general symptoms like:
- Continuous fever
- Weight loss and loss of appetite
- Fatigue and feeling unwell
- Difficulty breathing if it spreads to the lungs
Remember, these symptoms can also indicate other, less serious conditions. Any unusual or persistent lumps should be checked by a doctor.
Causes
The exact reasons why malignant fibrous histiocytoma or undifferentiated pleomorphic sarcoma develops aren’t fully understood. However, research suggests it’s likely caused by a combination of genetic changes and environmental factors.
At the cellular level, MFH/UPS tumors often have abnormal chromosomes, which can lead to uncontrolled cell growth. Scientists have identified specific genetic mutations in some cases, including changes in tumor suppressor genes and certain signaling pathways.
Environmental factors that may contribute include:
- Exposure to radiation therapy
- Previous bone trauma or conditions like Paget’s disease
- Contact with industrial chemicals like asbestos and vinyl chloride
Risk Factors
While the exact causes aren’t known, certain factors increase the risk of developing MFH or UPS:
- Age: Most common in adults between 50-70 years old
- Gender: Slightly more common in males
- Prior radiation exposure: People who had radiation therapy for previous cancers have higher risk
- Bone diseases: Conditions like Paget’s disease increase risk
- Previous injury: Sometimes develops in areas of prior trauma
- Environmental factors: Exposure to chemicals like asbestos and vinyl chloride
- Genetic factors: Certain mutations and chromosomal abnormalities
- Other conditions: Rare associations with hip replacement surgery and certain blood cancers
Diagnosis
Diagnosing malignant fibrous histiocytoma or undifferentiated pleomorphic sarcoma requires several steps because it can look similar to other conditions.
The diagnostic process typically includes:
Imaging Studies:
- X-rays for initial bone and tissue examination
- Ultrasound for soft tissue visualization
- CT scans for detailed 3D images
- MRI for highly detailed soft tissue images
- PET scans to distinguish between benign and malignant tumors and detect metastasis
- Bone scans to check for bone involvement
Biopsy: This is the most important step for definitive diagnosis. A tissue sample is removed and examined under a microscope. Different biopsy techniques may be used depending on the tumor’s location and size.
Pathological Analysis: The pathologist examines the tissue for characteristics unique to UPS. Because UPS cells can vary greatly, comprehensive testing is needed to exclude other cancers that might look similar.
Types
The classification of Malignant Fibrous Histiocytoma has changed significantly over time. Previously, it was divided into five subtypes, but modern understanding shows that MFH isn’t a single disease but rather a category for various poorly differentiated tumors.
Today, Undifferentiated Pleomorphic Sarcoma is diagnosed only when thorough testing can’t identify any specific type of sarcoma or other cancer.
Many tumors once called MFH are now classified as:
- Myxofibrosarcoma (formerly myxoid MFH)
- Angiomatoid Fibrous Histiocytoma (no longer considered MFH)
- Other specific sarcoma types like rhabdomyosarcoma or fibrosarcoma
- Sometimes even carcinomas, melanomas, or lymphomas
Stages
Undifferentiated Pleomorphic Sarcoma staging uses the TNM system plus histologic grade (G):
- T (Tumor): Size and extent of the primary tumor
- N (Nodes): Whether cancer has spread to nearby lymph nodes
- M (Metastasis): Whether cancer has spread to distant body parts
- G (Grade): Based on how abnormal the cells look and how fast they divide
These factors help classify UPS into stages I through IV. Better prognosis factors include early detection, smaller size, and location in arms or legs. Worse prognosis is associated with larger size, trunk location, and metastasis.
Treatment
Treating Malignant Fibrous Histiocytoma or Undifferentiated Pleomorphic Sarcoma requires a team approach with surgeons, radiation oncologists, and medical oncologists. Treatment plans are customized based on the tumor’s characteristics and location.
Main Treatment Options:
Surgical Excision: This is the primary treatment, aiming to remove the entire tumor with clear margins. Surgeons try to preserve limb function whenever possible, but amputation may be necessary in rare cases.
Radiation Therapy: Often used alongside surgery to improve local control and reduce local recurrence risk. It can be given:
- Before surgery to shrink large tumors
- After surgery to eliminate remaining cancer cells
- Through brachytherapy (radioactive sources placed directly in the tumor area)
- During surgery (IORT) for a single high dose
Chemotherapy: Uses powerful anti-cancer drugs to:
- Shrink tumors before surgery
- Kill remaining cancer cells after surgery
- Treat advanced or widespread disease
Emerging Treatments:
- Targeted therapy and immunotherapy are being studied for advanced cases
- Palliative care focuses on symptom management and quality of life
Rehabilitation
Rehabilitation is crucial for patients recovering from MFH/UPS treatment. The aggressive nature of the cancer and intensive treatments can cause various physical challenges.
Key Rehabilitation Components:
- Physical therapy to restore strength, flexibility, and mobility
- Occupational therapy to help with daily activities and work
- Pain management for post-surgical and treatment-related pain
- Lymphedema management if radiation therapy affects lymphatic drainage
- Scar tissue management to improve flexibility
- Prosthetic training for patients who need assistive devices
Rehabilitation is tailored to each patient’s needs and significantly impacts recovery and long-term function.
Complications
Malignant Fibrous Histiocytoma or Undifferentiated Pleomorphic Sarcoma can lead to several serious complications:
Disease-Related Complications:
- Local recurrence in the original tumor site
- Metastasis, especially to the lungs (40-55% of cases)
- Spread to bones, lymph nodes, liver, or brain
Treatment-Related Side Effects:
- Surgery: Pain, infection, bleeding, mobility issues
- Chemotherapy: Fatigue, hair loss, infection risk, nausea
- Radiation therapy: Skin changes, fibrosis, lymphedema, nerve damage
- Secondary cancers from radiation therapy
Emotional Impact:
- Anxiety, depression, and emotional distress from cancer diagnosis and treatment uncertainty
Prevention
Since the exact causes of MFH/UPS aren’t fully known, complete prevention isn’t possible. However, some strategies may help reduce risk:
- Healthy lifestyle: Balanced diet, regular exercise, avoiding tobacco
- Minimize radiation exposure: Discuss risks and benefits with healthcare providers
- Avoid harmful chemicals: Use proper safety measures around asbestos and vinyl chloride
- Manage existing conditions: Proper care of bone diseases like Paget’s disease
- Seek medical attention: Get significant injuries properly treated
Living With Rhabdomyosarcoma
Living with MFH/UPS presents both physical and emotional challenges. The rarity and aggressive nature of this sarcoma can create significant uncertainty.
Important Aspects of Living with the Condition:
Emotional Support:
- Seek counseling or therapy for anxiety, fear, and depression
- Join support groups to connect with others facing similar challenges
- Build a strong network of family and friends
Medical Care:
- Follow all scheduled follow-up appointments
- Monitor for local recurrence or metastasis through regular imaging
- Manage symptoms proactively with appropriate medications and therapies
Active Participation:
- Learn about your condition and treatment options
- Ask questions and seek second opinions when needed
- Consider clinical trials for access to new treatments
- Engage in prescribed rehabilitation programs
Living with UPS requires resilience and a proactive approach to managing both medical and emotional aspects of the disease.
Key Takeaways
- Malignant Fibrous Histiocytoma is now primarily known as Undifferentiated Pleomorphic Sarcoma, reflecting better understanding of its nature
- UPS is a rare, aggressive soft tissue sarcoma with high potential for metastasis, especially to the lungs
- Early symptoms typically begin as a painless lump, progressing to pain and functional limitations
- While exact causes are unclear, previous radiation therapy exposure is a significant risk factor
- Diagnosis requires comprehensive imaging and definitive biopsy with detailed pathological analysis
- Treatment involves multidisciplinary care with surgical excision as the primary approach, often combined with chemotherapy and radiation therapy
- Local recurrence and distant metastasis are common complications, along with various treatment-related side effects
- Prevention is challenging, but healthy lifestyle choices and minimizing known risk exposures are recommended
- Living with UPS requires strong medical and emotional support, adherence to follow-up care, and active participation in treatment decisions
At Sancheti Hospital, we understand the complex challenges that patients with Malignant Fibrous Histiocytoma and Undifferentiated Pleomorphic Sarcoma face. Our multidisciplinary team of orthopedic oncologists, medical oncologists, radiation oncologists, and rehabilitation specialists work together to provide comprehensive care tailored to each patient’s unique needs. We offer advanced diagnostic capabilities including state-of-the-art imaging technologies and expert pathological services to ensure accurate diagnosis. Our treatment approach combines the latest surgical techniques with cutting-edge radiation therapy and chemotherapy protocols. Beyond medical treatment, we provide comprehensive rehabilitation services and emotional support to help patients navigate their journey with UPS. At Sancheti Hospital, we’re committed to delivering world-class care while maintaining the compassionate, patient-centered approach that has been our hallmark for decades.
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
How long can someone live with UPS, and what affects survival rates?
Survival varies greatly depending on the tumor’s size, location, and whether it has spread. Early-stage tumors in arms or legs have better outcomes than those in the trunk or with metastasis. Overall 5-year survival rates range from 40-75%, with smaller tumors under 5cm having the best prognosis.
Can UPS be detected through regular blood tests or routine checkups?
No, UPS cannot be detected through routine blood tests. There are no specific blood markers for this cancer. It’s usually discovered when patients notice a growing lump or during imaging for other reasons. Regular physical exams may help detect lumps early.
How quickly does UPS grow and spread?
UPS is a fast-growing cancer that can double in size within weeks to months. It can spread to the lungs within 6-12 months of initial diagnosis if left untreated. This is why immediate treatment is crucial once diagnosed.
Will I need to have my limb amputated if I have UPS?
Amputation is rarely needed today. About 90-95% of patients can keep their limbs through limb-sparing surgery combined with radiation therapy. Amputation is only considered when the tumor involves major blood vessels or nerves that cannot be preserved.
What are the chances of UPS coming back after treatment?
Local recurrence happens in about 10-20% of cases when treated properly with surgery and radiation therapy. The risk is higher if surgical margins weren’t clear or if the tumor was large. Regular follow-up scans every 3-6 months help detect any recurrence early.
