Soft tissue sarcoma

• Liposarcoma (from fat): well-differentiated grows slowly; myxoid responds to chemotherapy and radiotherapy.
• Leiomyosarcoma (from smooth muscle), including in the uterus and blood vessels.
• Undifferentiated pleomorphic sarcoma.
• Synovial sarcoma (young adults, responds to chemotherapy).

Mainly in children and young adults. Very sensitive to chemotherapy, which is the main treatment along with surgery or radiotherapy.

• Angiosarcoma (from blood vessels), Kaposi sarcoma, and many rare types.
• Desmoid tumour: not cancer and does not spread, but can grow locally; often watched first.
• GIST is a sarcoma of the bowel wall and has its own map: choose GIST above.

Done before any biopsy or surgery. Shows the size, depth and relation to nerves and vessels.

A needle biopsy, planned so that the needle track can be removed later with the tumour. The lump should not be simply cut out before a scan and biopsy, as this can make proper surgery harder.

Some types are confirmed by specific mutations (for example SS18 in synovial sarcoma, MDM2 in liposarcoma).

NGS (next-generation sequencing) reads many genes in the tumour at once. In sarcoma it helps in two ways: it can confirm the exact type, and it can find a mutation that a specific medicine can target.

When is it advised? When the type is uncertain under the microscope, and in sarcoma that has spread or come back, before choosing the next treatment.

What the doctor is looking for:

• Not every patient will have a treatable mutation. Most sarcomas are still treated with surgery, radiotherapy and chemotherapy; a normal result is also useful.
• Some findings (for example TP53 in a young patient) may point to an inherited condition such as Li-Fraumeni syndrome; then a blood test and genetic counselling are advised.
• Results take about 2 to 3 weeks. The test is expensive, so your doctor will advise it when the result can change your diagnosis or treatment.

CT chest checks the lungs. PET-CT or CT of the abdomen for some types.

Before starting chemotherapy, immunotherapy or targeted therapy, these tests check that you are fit for treatment, and give a starting point to compare with later.

Before immunotherapy, also:

Sometimes needed, depending on your treatment:

• Pregnancy test for women who could be pregnant.
• Calcium and magnesium: for example before cisplatin or bone-strengthening injections.
• Dental check-up before bone-strengthening injections (zoledronic acid or denosumab), as they can rarely affect the jaw bone.
• Hearing test before cisplatin, in some patients.
• Clotting tests (PT/INR) before a biopsy or chemoport.

Your doctor will choose the tests that apply to you. Some of them are repeated during treatment.

• The type, grade and MRI decide the surgery, and whether radiotherapy or chemotherapy is given before it.
• An unplanned operation can lead to a larger second operation or the loss of a limb; careful planning protects the limb.

Slow growing, limited to one area. Usually cured with surgery.

Faster growing, limited to one area. Cure is likely with surgery and radiotherapy, sometimes chemotherapy.

Spread to the lungs or elsewhere. Treatment aims to control it; some with a few lung deposits can still be cured with surgery.

1. Surgery: wide removal of the tumour with a margin of normal tissue. Most limbs can be saved; amputation is rarely needed.
2. Radiotherapy for most high-grade or deep sarcomas, often before surgery (about 5 weeks) to shrink the tumour and reduce later stiffness.
3. Chemotherapy (doxorubicin and ifosfamide) for selected large high-grade, chemotherapy-sensitive types.
4. Retroperitoneal sarcoma: careful surgery at a specialist centre is the main treatment.

Side effects: surgery · radiotherapy · chemotherapy

1. Doxorubicin, alone or with ifosfamide.
2. Other options depending on type: gemcitabine with docetaxel, trabectedin, eribulin, pazopanib.
3. Targeted tablets if NGS finds a target (for example NTRK or ALK fusion).
4. Surgery to remove a few lung deposits in selected patients.
5. Radiotherapy to relieve symptoms.

Side effects: chemotherapy · pazopanib

• Recovery depends on the site; physiotherapy is important to regain movement and strength.
• Wound healing can be slow, especially after radiotherapy before surgery.

• Skin redness and tiredness during treatment.
• Later: stiffness, swelling of the limb, and a small risk of bone fracture in the treated area.

• Nausea and vomiting: well controlled with medicines given before and after chemotherapy.
• Low blood counts and infection risk: fever of 38°C or more needs urgent care.
• Tiredness, mouth sores, loose stools or constipation.
• Hair loss.
• Doxorubicin can affect the heart (echo is checked).
• Ifosfamide: can irritate the bladder (mesna is given to protect it), affect the kidneys, and rarely cause confusion or drowsiness.

More about chemotherapy · Side effects of your regimen

• High blood pressure, loose stools, liver changes (blood tests are checked), hair colour change and tiredness.

Fever of 38°C or more during chemotherapy, new breathlessness or cough, a red or discharging wound, or blood in the urine (with ifosfamide) need urgent care.

When to seek help urgently