1️⃣Bone Sarcomas (Osteosarcoma & Ewing Sarcoma)
Overview
Bone sarcomas are rare malignant tumours that arise from the bone-forming or
supportive tissue itself rather than from marrow or metastases.
They represent < 1 % of all cancers but are among the most common solid tumours
in teenagers and young adults.
Two principal types:
● Osteosarcoma – arises from osteoblasts that form immature bone.
● Ewing Sarcoma – derives from primitive neuro-ectodermal cells of bone or
soft tissue.
At Coimbatore Cancer Clinic, every patient is assessed by a sarcoma
multidisciplinary team that includes medical, surgical, and radiation oncologists,
orthopaedic oncologists, radiologists, and rehabilitation specialists to ensure
limb-preserving, function-oriented care.
Causes & Risk Factors
● Rapid bone growth during adolescence (explains teenage peak).
● Prior radiotherapy to bone.
● Genetic syndromes: Li-Fraumeni (TP53), RB1, Rothmund-Thomson.
● Chronic bone disease (Paget’s, fibrous dysplasia).
Symptoms
● Persistent localised bone pain, often night pain.
● Swelling or mass near joint.
● Limp or restricted motion.
● Occasionally pathologic fracture.
Diagnosis
1. X-ray & MRI of local region – defines intra-/extra-osseous extent.
2. CT chest – lung metastases evaluation.
3. PET-CT / Bone scan – whole-body staging.
4. Image-guided core biopsy – mandatory before surgery.
5. Histology & IHC confirm subtype (osteoid formation = osteosarcoma; CD99+,
EWSR1 fusion = Ewing).
Stage & Grade
Treatment Approach
1️⃣ Osteosarcoma
● Neoadjuvant Chemotherapy (10 weeks): Cisplatin + Doxorubicin ± High-Dose Methotrexate ± Ifosfamide.
● Surgery: Limb-salvage resection preferred; amputation rarely needed.
● Adjuvant Chemotherapy: same drugs for 18–24 weeks post-surgery.
● Response Assessment: tumour necrosis > 90% = excellent prognosis.
● 5-yr Survival: ≈ 70% (localized), 30% (metastatic).
2️⃣ Ewing Sarcoma
● Induction Chemotherapy: VDC/IE (Vincristine, Doxorubicin, Cyclophosphamide / Ifosfamide, Etoposide).
● Local Control: Surgery ± Radiotherapy 45–55 Gy.
● Adjuvant / Continuation Therapy: 14 cycles total over 9–10 months.
● Targeted/Immunotherapy:
o IGF-1R inhibitors (Figitumumab) – trials ongoing.
o PARP inhibitors + Temozolomide – for EWSR1-fusion tumours.
o GD2 CAR-T cells – early promising data (India/US 2024 trials).
Recurrent / Resistant Disease
● Gemcitabine + Docetaxel, Cyclophosphamide + Topotecan, or High-Dose Ifosfamide.
● Immunotherapy (Nivolumab ± Ipilimumab) experimental.
Supportive Care
● G-CSF for marrow protection.
● Bisphosphonates / Denosumab for bone pain.
● Intensive physiotherapy for post-operative limb recovery.
● Psychological counselling for adolescents and parents.
Recent Advances (2024–25)
● Liposomal Doxorubicin → less cardiac toxicity.
● Radioguided limb-sparing navigation surgery improves precision.
● Liquid biopsy cfDNA for early relapse detection.
● Osteosarcoma CAR-T targeting HER2 and GD2 under clinical evaluation.
Our Expertise
● Dedicated orthopaedic oncology collaboration for limb-salvage.
● Access to national protocols (ICON, EURAMOS, COG-inspired).
● 3D-printing for custom implants and prostheses.
● Comprehensive rehabilitation and physiotherapy.
2️⃣Soft-Tissue Sarcomas (STS)
Overview
Soft-tissue sarcomas are cancers of the body’s connective tissues—muscle, fat,
nerves, blood vessels, or fibrous tissue.
Over 50 histologic subtypes exist, each with unique behaviour and molecular
signatures.
While they can arise anywhere, the limbs and retroperitoneum are common sites.
Common Subtypes
Diagnosis
● MRI – defines local extent.
● Core biopsy with tract planned along surgical incision.
● Histopathology + IHC + NGS to subtype and identify actionable fusions.
● PET-CT – staging for metastasis.
Stage & Grade
● Stage I (Low grade) → often curable by surgery alone.
● Stage II–III (High grade localized) → Surgery + RT ± Chemo.
● Stage IV (Metastatic) → Systemic or palliative approach.
Treatment
Localized STS
1. Surgery – Wide excision with > 1 cm margin; limb-sparing preferred.
2. Radiotherapy
o Pre-operative 50 Gy → shrinks tumour, preserves function.
o Post-operative 60–66 Gy for close/positive margins.
3. Chemotherapy
o Doxorubicin ± Ifosfamide for high-risk disease.
o Pemetrexed or Gemcitabine/Docetaxel for second line.
4. Targeted / Novel Therapy
o Pazopanib (TKI) for non-adipocytic STS.
o Trabectedin for myxoid / round-cell sarcoma.
o Regorafenib for refractory disease.
o Immune checkpoint inhibitors (Pembrolizumab) in PD-L1+ tumours.
Metastatic / Recurrent
● Metastasectomy (lung) if solitary and controlled primary.
● Palliative systemic therapy as above.
● Enrolment in trials for combination IO + TKI therapy.
Rhabdomyosarcoma (RMS) – Paediatric Subtype
● Types: Embryonal, Alveolar, Pleomorphic.
● Therapy: VAC (Vincristine, Actinomycin-D, Cyclophosphamide) ± RT.
● Targeted Approaches: IGF-1R inhibitors, PI3K blockers, anti-PD-1 therapy.
● 5-yr Survival: 70–80 % (localised), 30 % (metastatic).
Emerging Options
● Tazemetostat (EZH2 inhibitor) for epithelioid sarcoma.
● Anlotinib – multi-target TKI improving PFS in soft-tissue sarcoma.
● CAR-T cells targeting NY-ESO-1 and MAGE-A4 antigens in synovial sarcoma
(phase II trials 2024–25).
● Adoptive T-cell therapy showing durable responses.
Supportive Care & Rehabilitation
● Prehabilitation for strength before surgery.
● Post-op physiotherapy, prosthetic training.
● Lymphedema prevention for limb sarcoma RT.
● Pain and nutritional management.
Our Expertise @ Coimbatore Cancer Clinic
● Onco-surgical partnership for complex limb or retroperitoneal resections.
● Availability of IMRT, IGRT for precise RT.
● Full systemic options including Doxorubicin, Ifosfamide, Trabectedin,
Pazopanib.
● Collaboration with centres offering CAR-T and TCR therapy trials.
● Comprehensive rehab, psychological support, and prosthesis services.
3️⃣Special Variants
Dermatofibrosarcoma Protuberans (DFSP)
● Low-grade skin sarcoma; COL1A1-PDGFB fusion.
● Wide local excision (> 2 cm margin) or Mohs surgery.
● Imatinib for unresectable or metastatic disease.
Kaposi Sarcoma
● Vascular sarcoma linked to HHV-8; seen in immunosuppressed patients.
● Local therapy: RT or intralesional Vinblastine.
● Systemic therapy: Pegylated liposomal Doxorubicin or Paclitaxel; HAART in HIV-associated cases.
Recent Breakthroughs (2024–25)
● TCR-engineered NY-ESO-1 therapy producing 40 % responses in synovial
sarcoma.
● NTRK inhibitors (Larotrectinib, Entrectinib) achieving 75 % response in
fusion-positive sarcoma.
● mRNA vaccine trials for fusion-driven sarcomas initiated globally.
● Circulating tumour DNA being used for early relapse detection.
Follow-Up & Prognosis
Surveillance: Clinical exam & chest CT every 3 months × 2 years, then 6-monthly to
5 years.
When to Seek Evaluation
Any persistent painful lump, increasing swelling, or mass > 5 cm in limb should be
evaluated by MRI and biopsy before any surgery.
Early diagnosis enables limb salvage and cure.
FAQs
Are all sarcomas aggressive?
No—some (like DFSP or low-grade leiomyosarcoma) are slow growing and curable by surgery alone.
Can limb amputation be avoided?
In > 90% cases, yes – limb-salvage surgery with reconstruction is feasible.
Is chemotherapy always necessary?
Mainly for high-grade or Ewing/Osteosarcoma types.
Are CAR-T or targeted therapies available in India?
Yes – through collaborating trial centres our clinic can facilitate access to these advanced options.
Disclaimer
This information is for educational purposes only. Treatment should be customised
by qualified oncologists based on tumour type, stage, and patient factors.
