Soft Tissue Sarcoma

Sarcomas are rare cancers of the body's connective tissues. Soft tissue sarcomas arise in fat, muscle, nerves, blood vessels or deep skin tissue, most often in the thigh, the arm, the trunk or the back of the abdomen. There are more than 70 subtypes, including liposarcoma, leiomyosarcoma, synovial sarcoma and, in children and young adults, rhabdomyosarcoma.

Because they are rare and resemble harmless lumps, they are often diagnosed late or removed without planning. Treatment is built around carefully planned surgery, frequently combined with radiotherapy, and it should be directed by a sarcoma team. Outlook depends on subtype, grade, size and spread and differs greatly between individuals.

Symptoms

• A lump that is growing
• A lump larger than about 5 cm, the size of a golf ball
• A lump lying deep in the muscle and not just under the skin
• A lump that has returned after removal
• Pain or tenderness, though many are painless
• Abdominal fullness, pain or early satiety with tumours behind the abdomen
• In children, a swelling around the eye, nose, bladder or testicle

Causes and risk factors

Most sarcomas occur without any identifiable reason. Known risk factors include previous radiotherapy to the area, long-standing lymphoedema, some chemical exposures, and inherited conditions such as Li-Fraumeni syndrome, neurofibromatosis type 1 and hereditary retinoblastoma. Many subtypes are defined by a specific gene fusion, which pathologists now test for in order to confirm the exact type. Injury does not cause sarcoma, although a knock often draws attention to a lump that was already there.

How it is diagnosed

• MRI of the lump: Done before any biopsy or surgery. It shows the size, depth and relation to nerves and vessels.
• Core needle biopsy: Taken by or in agreement with the sarcoma surgeon, so that the needle track can be removed at the operation. Unplanned removal makes later treatment harder.
• Expert pathology with molecular tests: Review by a sarcoma pathologist changes the diagnosis in a notable share of cases.
• CT of the chest: The lungs are the most common site of spread. CT of the abdomen or PET-CT is added for some subtypes.
• Grading and staging: Combine the tumour's microscopic appearance, its size, depth and spread.

Treatment options

• Wide surgical excision: Removal of the tumour with a cuff of healthy tissue. Limb-sparing surgery is possible in the large majority, sometimes with plastic surgical reconstruction.
• Radiotherapy: Given before or after surgery for large, deep or high-grade tumours to reduce local recurrence.
• Chemotherapy: Central for rhabdomyosarcoma and Ewing sarcoma. Used selectively in adult subtypes and for metastatic disease, typically doxorubicin and ifosfamide.
• Targeted therapy: Examples are imatinib for gastrointestinal stromal tumour, pazopanib, and drugs for tumours with NTRK fusions.
• Surgery or ablation of lung metastases: Considered when the deposits are few.
• Follow-up: Regular examination and chest imaging for at least 10 years.

When it is urgent

A lump that suddenly enlarges and becomes very painful, bleeding from a tumour, new breathlessness or coughing blood, or, in a child on chemotherapy, any fever needs urgent care at the nearest hospital.

Travelling to Türkiye for treatment

Sarcoma is one of the cancers where seeing a specialist team really matters, so travelling for a pathology review, a second opinion or a planned operation is reasonable, ideally before anything has been cut out. Surgery with reconstruction may need 2 to 4 weeks. Radiotherapy runs for about 5 to 6 weeks, and chemotherapy protocols for children last many months, which means a long stay or careful sharing of care with your home oncologist. Send the MRI on disc and the pathology blocks, not only the reports.

Send your reports, scans and a short history and a Clinic-Y coordinator replies within 24 hours with suitable teams and written, all-inclusive proposals side by side. Reviewing your case is free.

Frequently Asked Questions