Solitary Fibrous Tumor (SFT)

A solitary fibrous tumor (SFT) is a lump that grows from supporting (connective) tissue cells. These cells make fibers such as collagen, so the tumor often feels firm and looks fibrous under the microscope. SFT used to be thought of mainly as a tumor of the lining of the lung (the pleura), but doctors now know it can appear almost anywhere in the body, including the chest, abdomen, pelvis, limbs, head and neck, and even the coverings of the brain and spinal cord (the meninges). Most SFTs grow slowly and stay in one place, but some behave aggressively, coming back after surgery or spreading to other body parts. A key modern insight is that many SFTs share the same driver change in their DNA, called the NAB2–STAT6 fusion, which “turns on” growth signals in the tumor. In the lab, SFT cells almost always show nuclear STAT6 staining on immunohistochemistry, which strongly supports the diagnosis. NCBIPMC+1

Doctors also learned that the old term hemangiopericytoma overlaps with SFT. In the brain and spinal cord coverings, these two labels were combined into one family in recent World Health Organization (WHO) classifications, with grading based on how fast the cells divide and a few other features. This update helps doctors predict risk and choose follow-up plans more clearly. PMCThe Journal of Neuroscience

How it looks and behaves

Under the microscope, SFT often has a “patternless pattern”—a mix of spindle-shaped cells and thick collagen bands with branching, “staghorn-like” blood vessels. Most tumors are well-circumscribed, but some can invade nearby tissues. When doctors image these tumors with CT or MRI, they often see a well-defined, highly vascular mass that enhances clearly after contrast because the tumor has many blood vessels. Areas of dense collagen can look darker on some MRI sequences, while softer, more cellular areas look brighter; this mixed picture reflects the mix of tissue inside. PMC+1AJR American Journal of Roentgenology

A special but uncommon problem with SFT is severe low blood sugar caused by the tumor making excessive IGF-2 (“big IGF-2”). This is called Doege–Potter syndrome. People can feel shaky, sweaty, confused, or even pass out when sugar drops, and the low sugar often improves when the tumor is removed. Oxford Academicautopsyandcasereports.org

Types of solitary fibrous tumor

Doctors describe SFT in several helpful ways. These “types” guide risk discussion and follow-up. Think of them as patterns rather than totally separate diseases.

1. Classic SFT (low risk).
   This tumor grows slowly, has many collagen fibers, and shows very few dividing cells. It is usually removed completely with surgery and has a low chance of coming back or spreading.

2. Cellular SFT.
   This tumor has more tumor cells and less collagen. It can grow faster than the classic form and may need closer follow-up.

3. Malignant SFT.
   This tumor shows warning signs such as many dividing cells, larger and darker nuclei, areas of dead tissue (necrosis), and invasive edges. These features raise the chance of recurrence or spread, so the care team plans more careful monitoring.

4. Dedifferentiated SFT.
   In this rare pattern, part of the tumor suddenly looks very aggressive and different from the rest. This switch is linked to a higher risk of spread, and doctors manage it more like a high-grade sarcoma.

5. Pleural (intrathoracic) SFT.
   This tumor grows from the lining of the lung or chest wall. It may be found on a scan done for another reason or may cause cough, chest discomfort, or shortness of breath if large. Imaging often shows a well-circumscribed, enhancing mass that can be attached by a stalk to the pleura. AJR American Journal of Roentgenology

6. Extrapleural SFT.
   This is the broad group for all other body sites: abdomen, retroperitoneum, pelvis, liver, kidneys, limbs, head and neck, and more. Symptoms are usually due to pressure on nearby structures rather than pain.

7. Meningeal SFT (formerly hemangiopericytoma family).
   This tumor grows from the coverings of the brain/spinal cord. The WHO now groups it within the SFT family and grades it based on mitotic count and related features, because higher-grade tumors behave more aggressively. PMCThe Journal of Neuroscience

8. Myxoid SFT.
   This variant has more jelly-like (myxoid) areas. On MRI it can look brighter in those parts, echoing its soft internal texture. PMC

9. Fat-forming (lipomatous) SFT.
   This uncommon pattern contains both SFT cells and mature fat cells. It is still an SFT and shows STAT6 in the cell nuclei.

10. Giant SFT.
    Some tumors grow very large before being found, especially in the abdomen or retroperitoneum where there is room to expand. Size alone does not define behavior, but very large tumors need careful surgical planning.

11. Paraneoplastic SFT (with Doege–Potter syndrome).
    These are SFTs that make excess IGF-2 and cause repeated low blood sugar episodes. Treating the tumor is the key step to fix the sugar problem. Oxford Academic

12. Risk-stratified SFT (by models such as Demicco).
    Some teams estimate risk of spread by combining factors like age, tumor size, mitotic count, and necrosis. This does not change the diagnosis but helps set how closely to follow the patient after surgery. (Concept summarized from modern risk discussions in the SFT literature.) PMC

Causes

Important plain statement: For most people with SFT, there is no lifestyle cause and no inherited cause identified. The core driver is a random DNA change inside the tumor cells called NAB2–STAT6 fusion. This change happens after birth (somatic), and it is not passed down in families. Everything else below should be read as contributors, associations, or research ideas, not proven day-to-day causes. PMC

1. NAB2–STAT6 fusion (the real, proven driver inside the tumor).
   This DNA rearrangement fuses two genes and makes a protein that pushes the cell to keep growing. It is the main molecular hallmark of SFT. PMC

2. Random DNA copying errors over time.
   Cells divide and copy DNA. Rarely, errors slip through and, by chance, create the fusion that drives SFT. Age increases the number of opportunities for such errors to occur.

3. Natural wear-and-tear (oxidative stress).
   Every cell faces small chemical stresses. Over years, these can damage DNA, and a few damaged cells may pick up growth-driving changes.

4. Background body growth signals.
   Connective tissue cells respond to growth cues to repair and remodel tissues. Very rarely, a cell with a new fusion may over-respond and begin a tumor.

5. Prior radiation exposure (uncommon, suggested link).
   Strong radiation can break DNA. There are scattered reports of sarcomas, including SFT-like tumors, after prior radiation, but this is rare and not a typical history for SFT.

6. Chronic inflammation near the tumor site (uncertain).
   Inflammation brings cell turnover and reactive oxygen species. This could, in theory, raise the chance of a random DNA error, but it is not a proven cause of SFT.

7. Large body size or tall stature (theoretical, unproven).
   More cells and more lifetime cell divisions could mean more chances for random errors, but this is speculation, not established for SFT.

8. Environmental mutagens (general concept, not SFT-specific).
   Some chemicals can damage DNA, but no specific chemical has been proven to cause SFT in people.

9. Hormonal influences (uncertain).
   Hormones affect many tissues, but there is no clear hormonal cause for SFT.

10. Immune system suppression (broad, not specific).
    Certain cancers are more common with weaker immune surveillance. For SFT, this link is not proven.

11. Pre-existing scar tissue (speculative).
    Scarred areas remodel collagen and fibroblasts. While this might set the stage for more cell division, a direct causal link to SFT is not shown.

12. Chance alone.
    For many patients, the best explanation is simple chance—a rare random DNA event in a single connective tissue cell.

13. Site-specific microenvironment.
    Some tissues may be friendlier to SFT growth (for example, very vascular beds), but this is more about where the tumor can thrive than why it starts.

14. Aging.
    Older tissues have had more time for random mutations to accumulate, so SFT is more often found in adults than in children.

15. Background genetic makeup (no clear inherited syndrome).
    There is no widely accepted inherited SFT syndrome. Most SFTs are not caused by germline mutations.

16. Prior injury (not a cause).
    People often notice a lump after a bump or strain, but the injury usually draws attention to an already-present mass; it does not cause SFT.

17. Viruses (not supported).
    Some cancers are linked to viruses. SFT is not.

18. Diet (not a cause).
    Food choices do not cause SFT, and no diet prevents or triggers it.

19. Smoking or alcohol (not established causes).
    These are harmful for many reasons, but no clear link exists for SFT.

20. Occupational exposures (no proven tie).
    Unlike certain sarcomas tied to specific chemicals, no job exposure has been proven to cause SFT.

Take-home message: apart from the NAB2–STAT6 fusion inside the tumor, there is no single behavior, food, habit, or inherited pattern known to cause SFT. PMC

Symptoms

Symptoms depend on where the tumor grows and how big it is. Many SFTs are found by accident on a scan for another reason.

1. A painless lump.
   You may feel or see a firm mass under the skin or deep in a limb or body area. It may grow slowly.

2. Pressure or fullness.
   A deeper SFT in the abdomen or pelvis can press on nearby organs and cause a heavy or full feeling.

3. Pain or ache.
   Pain can occur if the tumor stretches tissues, presses on nerves, or outgrows its blood supply.

4. Cough or shortness of breath.
   A chest or pleural SFT can irritate airways or reduce lung expansion. AJR American Journal of Roentgenology

5. Change in bathroom habits.
   A pelvic or abdominal SFT can press on the bowel or bladder and change stool or urine patterns.

6. Swelling of a limb.
   A large tumor can block veins or lymph channels and cause swelling.

7. Numbness or tingling.
   If the tumor presses on a nerve, you can feel pins and needles or lose some sensation.

8. Weakness.
   Nerve compression can also make muscles weaker nearby.

9. Headache or seizures.
   If the tumor is in the meninges, it can cause headaches, seizures, or other neurologic symptoms. The Journal of Neuroscience

10. Weight loss or tiredness.
    Large or aggressive tumors can cause general body strain, leading to fatigue and weight loss.

11. Low blood sugar episodes (Doege–Potter syndrome).
    You may feel shaky, sweaty, confused, or faint; blood sugar tests show very low sugar unrelated to diabetes medication. Oxford Academic

12. Night sweats or fevers (uncommon).
    Some people notice systemic symptoms, but these are not specific.

13. Limited range of motion.
    A tumor near a joint can make movement stiff or painful.

14. Visible blood vessels or skin changes over a mass.
    Highly vascular tumors may make the overlying skin look stretched or show prominent veins.

15. Symptoms from spread (advanced cases).
    If the tumor spreads, symptoms depend on the new site, such as bone pain with bone spread.

Diagnostic tests

Goal of testing: find the mass, define its size and location, plan safe treatment, and confirm the diagnosis under the microscope. No single blood test makes the diagnosis. Doctors usually combine imaging, a core needle biopsy, and special lab stains to reach a firm answer. PMC

A) Physical exam

1. Inspection (looking).
   The clinician looks for a visible lump, swelling, skin stretching, or new veins over the area. They note size, shape, and whether the mass seems fixed or movable.

2. Palpation (feeling).
   The clinician gently presses around the lump to assess firmness, tenderness, warmth, and whether the edges are smooth or irregular. A firm, well-defined, deep mass points to a soft-tissue tumor such as SFT.

3. Range-of-motion check.
   If the mass is near a joint, the clinician moves the joint to see if the lump blocks movement or causes pain, which helps in surgical planning.

4. Basic neurologic screening.
   Light touch, pinprick, and strength are tested nearby; changes can suggest nerve compression from the mass.

B) Manual (bedside) maneuvers

5. Compression-release test over the mass.
   Gentle compression of the mass and release can help gauge tenderness, mobility, and relation to deeper planes, which guides imaging and biopsy approach.

6. Tinel-like tapping if a nerve is near.
   Light tapping over a suspected nerve pathway can reproduce tingling, supporting nerve irritation from the tumor.

7. Postural or Valsalva maneuvers (site-dependent).
   In the chest, breathing or postural changes may slightly change symptoms; in the abdomen, straining may accent fullness. This is not diagnostic by itself but adds context.

8. Bedside glucose check during symptoms.
   If the person is sweaty, shaky, or confused, a finger-stick glucose can reveal hypoglycemia, which raises suspicion for Doege–Potter syndrome when a mass is present. Oxford Academic

C) Laboratory and pathological tests

9. Basic blood panel (CBC, CMP).
   This checks overall health. It does not diagnose SFT, but it can reveal anemia, liver or kidney issues, and a safe baseline before procedures.

10. Fasting glucose and insulin levels.
    In suspected Doege–Potter syndrome, glucose is low, insulin is low or inappropriately normal, and C-peptide is low—pointing away from insulin overproduction and toward IGF-2 effects. Oxford Academic

11. IGF-2 and IGF-1 (and IGF-2:IGF-1 ratio).
    In “big IGF-2” secretion, IGF-2 is high relative to IGF-1. The ratio can strongly suggest paraneoplastic hypoglycemia from SFT when a mass is known. PubMed

12. Core needle biopsy (image-guided).
    A radiologist uses ultrasound or CT to guide a needle into the mass to take small cores. This is usually the safest, most informative first tissue test for deep soft-tissue tumors. The sample goes to pathology for diagnosis.

13. Histology (microscope exam).
    The pathologist looks for the patternless pattern, spindle cells, collagen bands, and branching staghorn-type vessels—features that fit SFT when combined with the right stains. PMC

14. Immunohistochemistry (STAT6).
    Nuclear STAT6 staining is a strong marker for SFT and reflects the NAB2–STAT6 fusion activity. Pathologists often also check CD34, CD99, and BCL-2, which are commonly positive but less specific. PMCBioMed Central

15. Molecular testing for NAB2–STAT6 fusion.
    If needed, a lab test using RT-PCR or next-generation sequencing can demonstrate the fusion directly, which supports or confirms the diagnosis, especially in challenging cases. PMC

16. Ki-67 proliferation index and mitotic count.
    These measurements estimate how fast the cells are dividing. Higher values suggest higher risk behavior and can influence follow-up intensity. (Part of routine sarcoma pathology reporting.) PMC

D) Electrodiagnostic tests (— used when nerves are affected)

17. Nerve conduction studies (NCS).
    If the mass compresses a nerve, NCS can detect slowed signal along that pathway, helping to document nerve involvement and guide surgery near critical nerves.

18. Electromyography (EMG).
    If weakness is present, EMG can show whether muscles near the mass are losing nerve input. This is not a test for SFT itself, but it helps assess the effect of the tumor on nerve function.

E) Imaging tests

19. Ultrasound (US).
    For superficial or accessible deep masses, US can show a solid, vascular lesion and guide a safe core biopsy. Color Doppler shows blood flow, which is often rich in SFT.

20. Contrast-enhanced CT scan.
    CT shows the size, shape, and relation to organs or bones, especially in the chest, abdomen, or pelvis. SFTs are often well-defined and avidly enhance due to many vessels; CT is also excellent for surgical planning. AJR American Journal of Roentgenology

21. MRI with contrast.
    MRI gives the best soft-tissue detail. SFTs typically show intermediate T1 signal and heterogeneous T2 signal with dark bands from dense collagen, plus strong enhancement. MRI helps judge invasion, map nerves and vessels, and, in the brain, define the dural base. PMC

22. Chest imaging for staging (CT chest).
    Because sarcomas can spread to the lungs, many teams image the chest even when the primary tumor is elsewhere, especially for higher-risk cases.

23. PET-CT (selected cases).
    Some SFTs show FDG uptake on PET-CT, but uptake can be mild or variable. PET-CT can help search for hidden spread or guide biopsy to the most active part of a large tumor. AJR American Journal of Roentgenology

24. Angiography (rare, pre-operative planning).
    Highly vascular SFTs may be mapped by angiography. In select cases, interventional radiology can embolize feeding vessels before surgery to reduce bleeding risk.

25. Brain and spine MRI (for meningeal SFT).
    If the tumor is in the meninges, MRI shows the dural attachment and any pressure on brain tissue. WHO grading for CNS SFT helps predict behavior and follow-up needs. PMC

Non-pharmacological treatments (therapies & others)

(each with description, purpose, and mechanism)

1. Multidisciplinary sarcoma board review
   Description: A team of surgeons, medical oncologists, radiation oncologists, pathologists, radiologists, and nurses review your case together.
   Purpose: To choose the safest, most effective plan.
   Mechanism: Combines different expert views to reduce errors and improve outcomes. NCBI

2. Wide local surgical excision with negative margins
   Description: Surgery to remove the tumor with a rim of normal tissue.
   Purpose: Cure when possible and reduce local recurrence.
   Mechanism: Physically removes all visible disease; clear margins lower seeding risk. JNCCN

3. Thoracic surgery (VATS or open) for pleural SFT
   Description: Video-assisted thoracoscopic surgery (VATS) or open thoracotomy to remove chest tumors.
   Purpose: Definitive treatment for chest SFT.
   Mechanism: Direct removal; minimally invasive options speed recovery. NCBI

4. Neurosurgical resection for meningeal SFT/HPC
   Description: Precise brain/skull-base surgery with removal of involved dura and venous sinuses when safe.
   Purpose: Maximal safe removal to prevent recurrence or neurological problems.
   Mechanism: En bloc resection decreases local failure; pathology confirms extent. Oxford Academic

5. Adjuvant radiation therapy (post-op RT)
   Description: Focused external-beam radiation after surgery in selected higher-risk cases (large size, close/positive margins, malignant histology).
   Purpose: Lower local recurrence risk.
   Mechanism: Damages DNA of residual microscopic tumor cells to prevent regrowth. NCBI

6. Neoadjuvant radiation
   Description: Radiation before surgery in carefully chosen cases.
   Purpose: Shrink tumor to enable safer or more complete surgery.
   Mechanism: Pre-op tumor kill can reduce intraoperative spill; may allow smaller post-op fields. JNCCN

7. Stereotactic radiosurgery / stereotactic body RT (SRS/SBRT)
   Description: Highly focused, high-dose radiation in 1–5 sessions for small brain or body targets.
   Purpose: Local control when surgery is not feasible, or for small metastases.
   Mechanism: Sub-millimeter precision delivers ablative doses while sparing normal tissue. NCBI

8. Proton therapy (select centers)
   Description: Proton beams deliver dose that stops at a precise depth.
   Purpose: Reduce radiation to nearby organs (e.g., base of skull, spine, mediastinum).
   Mechanism: Bragg peak spares exit dose, potentially lowering late effects. NCBI

9. Pre-operative arterial embolization (selected hypervascular tumors)
   Description: Interventional radiology blocks feeding arteries before surgery.
   Purpose: Reduce bleeding and make surgery safer.
   Mechanism: Cuts off blood flow, shrinking tumor vascularity.

10. Image-guided ablation (RFA/MWA/cryotherapy) for limited disease
    Description: Needle-based heat, cold, or microwave energy destroys small lesions.
    Purpose: Local control when surgery is not possible.
    Mechanism: Thermal or cryo injury causes tumor cell death.

11. Structured surveillance program
    Description: Regular physical exams and CT/MRI based on risk level.
    Purpose: Catch recurrences or metastases early when easier to treat.
    Mechanism: Scheduled imaging (e.g., every 3–6 months initially) finds small, treatable disease. NCBI

12. Pathology second opinion with STAT6 testing
    Description: Expert review of slides with STAT6 immunohistochemistry, and molecular testing when needed.
    Purpose: Confirm diagnosis; SFT can look like other tumors.
    Mechanism: STAT6 nuclear staining and NAB2-STAT6 fusion strongly support SFT. NaturePMC

13. Prehabilitation (pre-op fitness & nutrition)
    Description: Exercise, breathing training, and diet optimization before surgery.
    Purpose: Faster recovery and fewer complications.
    Mechanism: Improves cardiorespiratory reserve and wound healing.

14. Post-operative physical therapy
    Description: Guided movement and strengthening after surgery.
    Purpose: Restore function, reduce stiffness and pain.
    Mechanism: Gradual loading improves range of motion and muscle strength.

15. Lymphedema therapy (if limb/axillary surgery)
    Description: Compression, manual lymph drainage, and exercise.
    Purpose: Control swelling, prevent infections.
    Mechanism: Enhances lymphatic return and fluid balance.

16. Respiratory therapy after chest surgery
    Description: Breathing exercises and incentive spirometry.
    Purpose: Prevent pneumonia and atelectasis.
    Mechanism: Expands lungs and clears secretions.

17. Pain management with non-drug strategies
    Description: Heat/cold, relaxation, mindfulness, CBT.
    Purpose: Reduce pain and anxiety, improve sleep.
    Mechanism: Modulates pain pathways and stress hormones.

18. Psycho-oncology counseling
    Description: Mental health support for patients and families.
    Purpose: Treat anxiety/depression; improve coping.
    Mechanism: Cognitive and behavioral tools, social support.

19. Palliative care (at any stage if symptoms are significant)
    Description: Symptom-focused care alongside tumor treatment.
    Purpose: Improve quality of life and goal-concordant decisions.
    Mechanism: Multimodal relief of pain, dyspnea, fatigue, and distress.

20. Return-to-work and daily-life adaptations
    Description: Occupational therapy and workplace adjustments.
    Purpose: Safe, gradual return to normal activity.
    Mechanism: Task modification and energy conservation strategies.

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Drug treatments

(class, typical dosage & schedule, when used, purpose, mechanism, key side effects)

Important: Doses below reflect common study or label regimens used by clinicians; they are not personal medical advice. Your oncology team adjusts choices and doses to you.

1. Pazopanib (VEGFR tyrosine kinase inhibitor)
   Dose: 800 mg by mouth once daily (continuous).
   When/Purpose: Unresectable or metastatic SFT to slow growth and control disease.
   Mechanism: Blocks VEGF-driven blood vessel growth feeding the tumor.
   Side effects: Fatigue, high blood pressure, diarrhea, liver enzyme rise, hand-foot skin reaction. Strong evidence supports anti-angiogenics in SFT. PMCThe Lancet

2. Sunitinib (VEGFR/PDGFR TKI)
   Dose: 37.5 mg daily continuously, or 50 mg daily for 4 weeks then 2 weeks off.
   When/Purpose: After intolerance or progression on other anti-angiogenics; off-label but reported active.
   Mechanism: Multi-target anti-angiogenic.
   Side effects: Fatigue, hypertension, mouth sores, low platelets.

3. Regorafenib (multikinase anti-angiogenic)
   Dose: 160 mg daily, 3 weeks on / 1 week off.
   When/Purpose: Advanced SFT, including after other TKIs.
   Mechanism: Inhibits multiple angiogenic and oncogenic kinases.
   Side effects: Hand-foot syndrome, fatigue, hypertension, diarrhea. A 2023 study supports its use in fit patients. ScienceDirect

4. Sorafenib (multikinase anti-angiogenic)
   Dose: 400 mg by mouth twice daily.
   When/Purpose: Another anti-angiogenic option when others are unsuitable.
   Mechanism: Blocks RAF, VEGFR, PDGFR signaling.
   Side effects: Hand-foot reaction, rash, diarrhea, fatigue.

5. Axitinib (VEGFR TKI)
   Dose: 5 mg by mouth twice daily (titrated).
   When/Purpose: Off-label anti-angiogenic for SFT when other agents aren’t tolerated.
   Mechanism: Potent VEGFR blockade.
   Side effects: Hypertension, diarrhea, fatigue.

6. Temozolomide (alkylating agent)
   Dose: Common SFT regimen pairs temozolomide with bevacizumab: temozolomide 150 mg/m² orally on days 1–7 and 15–21 of a 28-day cycle.
   When/Purpose: Unresectable/metastatic disease, especially with the bevacizumab combination, to shrink or stabilize tumors.
   Mechanism: Damages tumor DNA; bevacizumab starves tumor blood vessels.
   Side effects: Fatigue, low blood counts, nausea. This regimen has shown responses in SFT/HPC. PubMed+1ACS Journals

7. Bevacizumab (anti-VEGF antibody)
   Dose: 5 mg/kg IV on days 8 and 22 when used with temozolomide in the above 28-day cycle (other schedules exist).
   When/Purpose: With temozolomide or as part of anti-angiogenic strategy.
   Mechanism: Neutralizes VEGF to inhibit tumor angiogenesis.
   Side effects: Hypertension, bleeding risk, wound-healing delay, proteinuria. PubMedACS Journals

8. Doxorubicin (anthracycline chemotherapy)
   Dose: 60–75 mg/m² IV every 3 weeks (alone) or in combinations.
   When/Purpose: Traditional first-line chemo in soft-tissue sarcoma when anti-angiogenic therapy is unsuitable.
   Mechanism: DNA intercalation/topoisomerase II inhibition.
   Side effects: Low counts, hair loss, mouth sores, heart toxicity (lifetime dose limits). JNCCN

9. Ifosfamide (alkylating chemotherapy)
   Dose: 1.5–3 g/m²/day for 3–5 days every 3 weeks with mesna uroprotection (varies).
   When/Purpose: As a sarcoma regimen component when shrinking the tumor could enable surgery or relieve symptoms.
   Mechanism: Cross-links DNA.
   Side effects: Low counts, nausea, kidney injury, encephalopathy; needs hydration and monitoring. JNCCN

10. Trabectedin (DNA-binding antineoplastic)
    Dose: 1.5 mg/m² as a 24-hour IV infusion every 3 weeks.
    When/Purpose: Later-line option in advanced soft-tissue sarcoma; case-series experience in SFT.
    Mechanism: Binds to DNA minor groove; modulates tumor microenvironment.
    Side effects: Liver enzyme rise, fatigue, neutropenia; requires central line and steroid premedication. JNCCN

Why so many anti-angiogenic drugs? SFTs are often highly vascular, and modern reviews and rare-cancer guidance favor these agents over traditional chemotherapy for advanced disease, while surgery remains first-line when feasible. PMCesmorarecancers.org

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Dietary molecular supplements

Always discuss supplements with your oncology team. Some herbs and antioxidants interact with TKIs or chemotherapy (for example, CYP3A4 interactions). The items below aim to support strength, nutrition, and symptom control—not to treat SFT itself.

1. Vitamin D3 — 1,000–2,000 IU daily (adjust to blood level).
   Supports bone, muscle, and immune function; deficiency is common during cancer care. Mechanism: nuclear receptor signaling in many tissues.

2. Omega-3 fish oil (EPA/DHA) — 1–2 g combined EPA+DHA daily with meals.
   Helps maintain weight and may reduce inflammation. Mechanism: membrane lipid mediators.

3. Protein powder (whey/pea) — 20–30 g after meals or rehab sessions.
   Preserves lean mass after surgery or during therapy. Mechanism: essential amino acids for repair.

4. Probiotics (e.g., Lactobacillus/Bifidobacterium) — 10–20 billion CFU/day if not neutropenic.
   May support gut health and bowel regularity. Mechanism: microbiome modulation.

5. Magnesium glycinate — 200–400 mg at night.
   Helps cramps/constipation; supports sleep. Mechanism: cofactor for many enzymes.

6. Vitamin B12 (methylcobalamin) — 1,000 mcg daily if low or borderline.
   Supports nerves and red blood cells. Mechanism: coenzyme for DNA synthesis.

7. Iron (if iron-deficiency only) — per labs, e.g., ferrous sulfate 325 mg every other day.
   Corrects iron-deficiency anemia. Mechanism: hemoglobin synthesis. (Avoid if iron stores are normal.)

8. Melatonin — 3–5 mg at bedtime.
   Supports sleep; investigated as adjunct in cancer symptom control. Mechanism: circadian and antioxidant effects.

9. Curcumin (with piperine) — 500–1,000 mg/day if no TKI is used and your team approves.
   May help joint discomfort and inflammation. Mechanism: NF-κB and cytokine modulation. (CYP interactions possible—check with your doctor.)

10. Green tea extract (EGCG) — 200–400 mg/day if approved by your team.
    Antioxidant/anti-inflammatory effects. Mechanism: polyphenols. (Potential drug interactions—clear with oncology first.)

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Regenerative / stem-cell–related” medicines

(real-world use is supportive, not anti-tumor; use only when your oncology team prescribes them)

1. Filgrastim (G-CSF) — ~5 mcg/kg/day subcutaneously after chemo until counts recover.
   Function: Boosts white blood cells to lower infection risk.
   Mechanism: Stimulates bone-marrow granulocyte production.

2. Pegfilgrastim — single 6 mg shot per chemo cycle.
   Function/Mechanism: Same as above with longer action.

3. Epoetin alfa / Darbepoetin alfa — e.g., epoetin alfa 40,000 units weekly (for chemo-induced anemia when criteria are met).
   Function: Raises red blood cells to reduce transfusions.
   Mechanism: Erythropoietin receptor stimulation in marrow.

4. Eltrombopag / Romiplostim — dosing per platelet count.
   Function: Raises platelets in selected refractory thrombocytopenia.
   Mechanism: TPO-receptor agonists stimulate megakaryocytes.

5. IVIG (intravenous immunoglobulin) — e.g., 0.4 g/kg/day × 5 days in specific immune deficits.
   Function: Temporarily supports immune defense.
   Mechanism: Provides pooled antibodies.

6. Clinical-trial therapies targeting NAB2-STAT6 (research only)
   Function: Experimental RNA-targeting/antisense approaches against the fusion driver.
   Mechanism: Attempts to silence the NAB2-STAT6 fusion that powers SFT growth; not standard of care yet. Ask about trial availability. PMCScienceDirectMDPI

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Surgeries

1. Wide local excision with margin assessment
   Procedure: Remove the tumor and a cuff of normal tissue; send margins for pathology.
   Why: Best chance of cure and lowest local recurrence when margins are negative. JNCCN

2. En bloc resection of involved structures
   Procedure: Remove tumor plus any directly invaded tissues (e.g., diaphragm slip, pericardial patch, chest wall segment) with reconstruction as needed.
   Why: Ensures no tumor is left behind if it has grown into adjacent structures. NCBI

3. VATS or open thoracotomy for pleural SFT
   Procedure: Minimally invasive or open removal of chest tumors; sometimes lobectomy if hilar involvement.
   Why: Definitive treatment for pleural lesions with quicker recovery if VATS is feasible. NCBI

4. Cranial resection with dural/venous sinus management for meningeal SFT
   Procedure: Microsurgical removal; resect/repair dura; manage venous sinuses when safe; adjuvant RT commonly considered.
   Why: Maximal safe resection reduces recurrence and neurological complications. Oxford Academic

5. Metastasectomy (e.g., lung or liver)
   Procedure: Surgical removal of a limited number of metastases in carefully selected patients.
   Why: Can prolong disease control when all visible disease can be removed and the primary site is controlled. Medscape

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Preventions

There is no proven way to prevent SFT, because its exact cause is not known. But you can reduce complications and improve outcomes with these practical steps:

1. Choose centers with sarcoma expertise for diagnosis and treatment.

2. Do not biopsy or excise a suspected sarcoma in a non-specialist setting; poor planning can cause tumor spill.

3. Stop smoking and avoid second-hand smoke to protect lungs and wound healing.

4. Keep vaccinations (flu, pneumococcal, COVID-19) up to date as advised.

5. Maintain a healthy weight, protein-sufficient diet, and regular physical activity.

6. Manage blood pressure and blood sugar, especially if on TKIs.

7. Follow post-operative instructions carefully to protect the surgical site.

8. Attend all surveillance scans to catch recurrences early.

9. Discuss drug–supplement interactions with your team before starting anything new.

10. Seek genomic/pathology review if the diagnosis is uncertain; correct labeling prevents mis-treatment. NCBI

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When to see doctors

• Right away (emergency) if you have sudden chest pain, severe shortness of breath, coughing blood, confusion, new seizures, or uncontrolled bleeding.

• Promptly if you notice a new lump or a known lump that is growing, if pain, swelling, or numbness increases, if you have persistent headaches or vision changes, or if you develop treatment side effects like high blood pressure, severe fatigue, fevers, mouth sores, swelling in legs, or shortness of breath while on TKIs or chemotherapy.

• Routinely for scheduled follow-ups and scans; early detection of relapse makes a difference. NCBI

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What to eat” and “what to avoid

Eat more of:

1. Protein-rich foods (eggs, fish, poultry, tofu, dal) to heal and maintain muscle.

2. Colorful fruits and vegetables for vitamins, minerals, and fiber.

3. Whole grains (oats, brown rice) for steady energy and bowel health.

4. Healthy fats (olive oil, nuts, seeds) to meet calories without heavy sugars.

5. Fermented foods (yogurt, kefir) if you are not neutropenic and your doctor agrees.

Avoid or limit:

1. Alcohol—can worsen blood pressure and liver enzymes, especially with TKIs.
2. Highly processed meats and deep-fried foods—raise inflammation and heart risk.
3. Large herbal/antioxidant doses without approval—possible drug interactions with anti-angiogenics and chemo.
4. Grapefruit or Seville oranges if on TKIs—CYP3A interactions can raise drug levels.
5. Unpasteurized/raw foods when counts are low—higher infection risk.

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FAQs

1) Is SFT cancer?
SFT is a soft-tissue sarcoma. Many are low-grade and slow, but some behave aggressively. Behavior varies by size, mitoses, necrosis, and margins. NCBI

2) What causes SFT?
No lifestyle cause is proven. Most tumors have a NAB2-STAT6 fusion that acts like a stuck “on-switch” for growth. PMC

3) How is SFT diagnosed?
Biopsy and pathology review with STAT6 nuclear staining and, when needed, molecular testing. Imaging (CT/MRI) maps the tumor. Nature

4) What is the first treatment?
If operable, complete surgical removal with negative margins is standard. JNCCN

5) Do I need radiation?
Some higher-risk cases benefit from adjuvant radiation to lower local recurrence. Your team weighs size, grade, and margins. NCBI

6) What if the tumor cannot be removed or has spread?
Anti-angiogenic drugs (like pazopanib) and temozolomide + bevacizumab are commonly used; classic sarcoma chemo is another option when appropriate. PMCPubMed

7) Are immunotherapies helpful?
Evidence is limited so far; clinical trials continue. Ask if a study is available for you. PMC

8) Does SFT come back?
It can. Risk depends on tumor factors and margins. That’s why regular scans are important. NCBI

9) How often are scans needed?
Typically every 3–6 months at first, then less often, personalized to risk. NCBI

10) Can SFT spread?
Yes. Common sites include lungs, liver, bones, and others; risk varies. NCBI

11) What about meningeal SFT (older term “hemangiopericytoma”)?
Managed with maximal safe resection and often radiation; close follow-up is crucial. Oxford Academic

12) Are anti-angiogenic pills safe long-term?
They can work for months to years, but blood pressure, liver tests, thyroid, skin, and hand-foot symptoms need monitoring. PMC

13) Will diet cure SFT?
No diet cures SFT. Nutrition supports strength and recovery and helps you tolerate therapy.

14) Should I take supplements?
Only with your team’s approval to avoid drug interactions; focus first on food and protein.

15) Where can I find trustworthy guidance?
Major sarcoma centers and national guidelines (e.g., NCCN/ESMO) offer up-to-date frameworks that your doctors use. NCCNJNCCNESMO

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: August 24, 2025.